Aortitis

Complex MONDO:0006656 Pathograph 6 Vascular disorder Vasculitis Inflammatory disorder

Aortitis is inflammation of the aortic wall, spanning noninfectious large-vessel vasculitis, clinically isolated surgical-pathology aortitis, infectious aortitis, and drug-induced forms such as G-CSF-associated aortitis. The shared disease axis is aortic wall inflammation with risk for aneurysm, dissection, stenotic or occlusive disease, ischemic complications, and aortic valve involvement, but the required management differs sharply by etiology.

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5
Pathophys.
2
Histopath.
7
Phenotypes
6
Pathograph
5
Treatments
5
Subtypes
71
References
2
Deep Research
◆

Subtypes

5
Clinically isolated aortitis
Histology-confirmed aortitis without known systemic inflammatory or infectious disease; many cases are discovered incidentally during aortic surgery.
Show evidence (1 reference)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"The prevalence of aortitis was 10.6% (n = 57), of which 75% were clinically isolated."
This surgical cohort identifies clinically isolated aortitis as the majority subtype among histology-confirmed aortitis cases.
Large-vessel vasculitis-associated aortitis
Aortitis associated with giant cell arteritis, Takayasu arteritis, or other large- and variable-vessel vasculitides.
Show evidence (1 reference)
DOI:10.3390/jcm13216364 SUPPORT Human Clinical
"Large vessel vasculitis (LVV), particularly giant cell arteritis (GCA) and Takayasu arteritis (TAK), has garnered attention due to its significant morbidity and mortality."
This review anchors GCA and Takayasu arteritis as major large-vessel vasculitis contexts relevant to aortitis.
Infectious aortitis
Aortitis caused by microbial infection of the aortic wall, often presenting with aneurysm or structural aortic abnormalities and requiring urgent antimicrobial and vascular-surgery evaluation.
Show evidence (1 reference)
DOI:10.1093/cid/ciac560 SUPPORT Human Clinical
"One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each"
This multicenter aortitis cohort identifies infectious aortitis cases and their causative organisms.
G-CSF-associated drug-induced aortitis
A rare adverse event after granulocyte-colony stimulating factor exposure, most often reported in oncology supportive-care settings and occasionally in healthy stem-cell donors.
Show evidence (1 reference)
DOI:10.3389/fphar.2024.1487501 SUPPORT Human Clinical
"Recombinant human granulocyte-colony stimulating factors (G-CSF)-induced aortitis is a rare but particularly serious adverse event, commonly seen in cancer patients undergoing chemotherapy."
This literature review supports G-CSF exposure as a drug-induced aortitis subtype, especially in chemotherapy care.
IgG4-related aortitis/periaortitis
Aortitis or periaortitis occurring in the context of IgG4-related disease, characterized by IgG4-bearing plasma-cell infiltration and fibrotic inflammation that predominantly affects the abdominal aorta and iliac arteries.
Show evidence (2 references)
DOI:10.3389/fimmu.2025.1625456 SUPPORT Human Clinical
"IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs."
The review defines IgG4-related disease by IgG4-bearing plasma-cell infiltration and fibrotic inflammation.
DOI:10.3389/fimmu.2025.1625456 SUPPORT Human Clinical
"IgG4-related aortitis/periaortitis and periarteritis (IgG4-related PAO/PA) predominantly affect the abdominal aorta and iliac arteries, with a higher prevalence in elderly males."
This directly supports IgG4-related aortitis/periaortitis as a distinct vascular manifestation.
⚙

Pathophysiology

5
Immune-mediated aortic wall inflammation in large-vessel vasculitis
Large-vessel vasculitis-associated aortitis involves immune-mediated inflammation of the vascular wall, including inflammatory cell accumulation in aortic tissue in Takayasu arteritis.
T cell link macrophage link granulocyte link endothelial cell link smooth muscle cell link
inflammatory response link ↑ INCREASED adaptive immune response link ⚠ ABNORMAL
aorta link
Downstream
Aortic wall remodeling and structural complications Persistent vascular inflammation contributes to later remodeling and structural complications such as aneurysm, dissection, or ischemia.
Show evidence (3 references)
DOI:10.3390/jcm13216364 SUPPORT Human Clinical
"Both conditions involve immune-mediated inflammation of the vascular wall, despite differing in epidemiology and presentation."
Supports immune-mediated vascular-wall inflammation as a shared mechanism in the GCA/TAK large-vessel vasculitis context.
DOI:10.1177/000331970005100705 SUPPORT Human Clinical
"CD68 (macrophages), CD15 (granulocytes), von Willebrand factor (endothelial cells), and alpha-smooth muscle actin (smooth muscle cells)."
Directly supports macrophage and granulocyte involvement in affected aortic wall tissue in Takayasu arteritis.
DOI:10.1177/000331970005100705 SUPPORT Human Clinical
"(endothelial cells), and alpha-smooth muscle actin (smooth muscle cells)."
Supports endothelial-cell and smooth-muscle-cell assessment in affected aortic wall tissue.
Infectious aortic wall infection and aneurysmal injury
Infectious aortitis is initiated by microbial infection of native aortic tissue, producing wall thickening or aneurysmal distortion and a higher mortality-risk phenotype than noninfectious aortitis.
inflammatory response link ↑ INCREASED
aorta link
Downstream
Aortic aneurysm Infectious wall injury commonly presents with aneurysmal aortic disease.
Show evidence (2 references)
DOI:10.1093/cid/ciac560 SUPPORT Human Clinical
"One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each"
Identifies causative organisms in infectious aortitis and supports microbial infection as the upstream etiology.
DOI:10.1093/cid/ciac560 SUPPORT Human Clinical
"IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001)."
Links infectious aortitis to aneurysmal aortic injury more strongly than noninfectious aortitis in the comparison cohort.
G-CSF-associated aortic inflammation
Exogenous G-CSF exposure can trigger aortitis as a serious adverse event, most often detected by CT in the aortic arch and branch vessels.
cytokine-mediated signaling pathway link ⚠ ABNORMAL inflammatory response link ↑ INCREASED
aorta link
Show evidence (2 references)
DOI:10.3389/fphar.2024.1487501 SUPPORT Human Clinical
"The G-CSF type with the highest frequency of occurrence of aortitis is pegfilgrastim."
Identifies G-CSF exposure, particularly pegfilgrastim, as the most common trigger in the case-review dataset.
PMID:38521841 PARTIAL Human Clinical
"switching from one short-acting G-CSF to another does not prevent recurrence of G-CSF-associated aortitis."
The case supports recurrence after switching short-acting G-CSF preparations and helps explain why re-exposure should be treated cautiously.
Aortic wall remodeling and structural complications
Inflammatory injury can weaken and remodel the aortic wall, producing aneurysm or dissection and driving the need for long-term aortic follow-up.
smooth muscle cell link fibroblast link
extracellular matrix organization link ⚠ ABNORMAL
aorta link
Downstream
Aortic aneurysm Aortic wall remodeling can present as aneurysmal dilation.
Aortic dissection Weakened and inflamed aortic wall can dissect.
Show evidence (1 reference)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
Directly links aortic inflammation to the principal structural complications of aneurysm and dissection.
✶

Histopathology

2
Aortic wall inflammatory infiltrates
Aortitis can show inflammatory infiltrates in the aortic wall, with lymphocytes, dendritic cells, macrophages, granulocytes, endothelial cells, and smooth muscle cells characterized in Takayasu aortic tissue.
Show evidence (1 reference)
DOI:10.1177/000331970005100705 SUPPORT Human Clinical
"All specimens showed distinctive histologic features of Takayasu's arteritis and contained inflammatory infiltrates"
Histologic inflammatory infiltrates in aortic wall tissue support this microscopic aortitis feature.
Histology-confirmed clinically isolated aortitis
Clinically isolated aortitis is often diagnosed through intra-operative aortic sampling rather than through preoperative clinical symptoms.
Show evidence (1 reference)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Intra-operative sampling is essential for diagnosis, with many cases presenting asymptomatically as clinically isolated aortitis."
Supports intra-operative histologic sampling as a key diagnostic route for clinically isolated aortitis.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Aortitis Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
●

Phenotypes

7
Cardiovascular 3
Aortic aneurysm Aortic aneurysm (HP:0004942)
Show evidence (2 references)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
The abstract directly names aneurysm as a complication of aortitis.
DOI:10.1093/cid/ciac560 SUPPORT Human Clinical
"IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001)."
The infectious/noninfectious comparison shows a strong association between infectious aortitis and aortic aneurysm.
Aortic dissection Aortic dissection (HP:0002647)
Show evidence (1 reference)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
The abstract directly names dissection as a complication of aortitis.
Aortic regurgitation Aortic regurgitation (HP:0001659)
Show evidence (1 reference)
DOI:10.62186/001c.146456 SUPPORT Human Clinical
"Due to severe aortic regurgitation limiting her activity and aortic root aneurysm"
This case report links giant cell aortitis with severe aortic regurgitation and aortic root aneurysm.
Metabolism 1
Fever Fever (HP:0001945)
Show evidence (2 references)
PMID:40272133 SUPPORT Human Clinical
"The diagnosis of G-CSF-induced aortitis should be considered in patients with fever after G-CSF treatment, particularly if adequate antibiotic treatment does not lead to improvement."
The case report interpretation explicitly links fever after G-CSF exposure to consideration of G-CSF-induced aortitis.
PMID:34448091 SUPPORT Human Clinical
"If a patient develops a persistent high fever of unknown origin after pegfilgrastim administration, drug-induced aortitis should be considered during investigations for causes of fever."
The case-report conclusion supports persistent high fever as a key clinical clue for pegfilgrastim-induced aortitis.
Other 3
Aortitis Aortitis (HP:6001461)
Show evidence (1 reference)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
The abstract defines aortitis as inflammation of the aorta.
Elevated C-reactive protein Elevated circulating C-reactive protein concentration (HP:0011227)
Show evidence (2 references)
PMID:40272133 SUPPORT Human Clinical
"After four days, C-reactive protein (CRP) had increased from 104 to 331, but the patient's condition was largely unchanged."
The case report documents marked CRP increase during G-CSF-associated aortitis evaluation.
PMID:34448091 SUPPORT Human Clinical
"Therefore, C-reactive protein (CRP) is currently used to assess disease activity in clinical practice."
The full-text case report notes CRP use for assessing G-CSF-associated aortitis activity.
Elevated erythrocyte sedimentation rate Elevated erythrocyte sedimentation rate (HP:0003565)
Show evidence (1 reference)
PMID:36843728 SUPPORT Human Clinical
"The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated, and he also had inflammatory anemia with a hemoglobin of 11.7 g/L."
This giant-cell-aortitis case report documents elevated ESR and CRP in the inflammatory presentation.
💊

Treatments

5
Glucocorticoid therapy for noninfectious aortitis
Action: Pharmacotherapy NCIT:C15986
Agent: glucocorticoid ↗ prednisolone ↗
Glucocorticoids are a first-line immunosuppressive treatment backbone for noninfectious large-vessel aortitis contexts, including giant cell arteritis and IgG4-related aortitis/periaortitis.
Show evidence (3 references)
DOI:10.1186/s13063-024-07905-4 SUPPORT Human Clinical
"Glucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates."
Giant cell arteritis is a major large-vessel vasculitis context for aortitis, and this trial protocol identifies glucocorticoids as standard treatment.
PMID:36843728 SUPPORT Human Clinical
"Treatment with high-dose steroids should be initiated as soon as possible to rapidly control the inflammatory symptoms and prevent ischemic complications"
This giant-cell-arteritis/aortitis case report supports high-dose steroid treatment for inflammatory large-vessel disease.
DOI:10.3389/fimmu.2025.1625456 SUPPORT Human Clinical
"Glucocorticoids (GCs) are the mainstay of treatment, often combined with immunosuppressants (IMs), while B- and T-cell-targeted therapies are under investigation."
The IgG4-related aortitis review directly supports glucocorticoids as a mainstay treatment for IgG4-related aortitis/periaortitis.
Empiric antimicrobial therapy for infectious aortitis
Action: Pharmacotherapy NCIT:C15986
Infectious aortitis requires early antimicrobial therapy, initially empiric when microbiologic documentation is pending and then adapted to organism and susceptibility data.
Show evidence (1 reference)
DOI:10.1093/cid/ciac560 SUPPORT Human Clinical
"Effective empiric antimicrobial therapy, initiated before any microbial documentation, was associated with a decreased mortality"
Supports early empiric antimicrobial therapy as a mortality-associated protective intervention in infectious aortitis.
Tocilizumab for LVV-associated aortitis contexts
Action: Pharmacotherapy NCIT:C15986
Tocilizumab may be used for large-vessel vasculitis phenotypes associated with aortitis, with clinical remission and glucocorticoid-sparing effects reported in LV-GCA and Takayasu arteritis cohorts.
Show evidence (2 references)
DOI:10.3390/sci7010012 SUPPORT Human Clinical
"Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK)."
Supports tocilizumab as a treatment option in the LVV contexts that can include aortitis.
DOI:10.3390/sci7010012 PARTIAL Human Clinical
"complete imaging resolution was observed in only 18.9% of LV-GCA patients and 21.1% of TAK patients."
The clinical benefit is tempered by incomplete imaging resolution, supporting a partial rather than complete effect on vascular inflammation.
Upadacitinib for giant-cell-arteritis contexts
Action: Pharmacotherapy NCIT:C15986
Upadacitinib is a JAK inhibitor with phase III evidence for giant cell arteritis, a major large-vessel vasculitis context that can include aortitis. Its long-term effect on aortic remodeling remains uncertain.
Show evidence (1 reference)
PMID:40174237 PARTIAL Human Clinical
"In patients with giant-cell arteritis, upadacitinib at a dose of 15 mg - but not 7.5 mg - with a 26-week glucocorticoid taper showed efficacy superior to that of placebo with a 52-week glucocorticoid taper."
The SELECT-GCA phase III trial supports upadacitinib efficacy in GCA, but not specifically aortic remodeling or aortitis endpoints.
Specialist multidisciplinary follow-up and aortic surveillance
Action: clinical assessment MAXO:0000487
Patients with aortitis require specialist follow-up because of increased re-intervention risk and late aortic complications.
Show evidence (2 references)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Due to the increased re-intervention in aortitis, specialist multi-disciplinary follow-up and aortitis centres should be formed."
Supports specialist multidisciplinary follow-up for aortitis after major aortic surgery.
PMID:40099651 SUPPORT Human Clinical
"Surveillance of patients with IA with repeated clinical assessments and imaging is recommended."
This review supports repeated clinical and imaging surveillance for isolated aortitis.
🌍

Environmental Factors

2
Granulocyte-colony stimulating factor exposure
G-CSF exposure, particularly pegfilgrastim in reported cases, can trigger drug-induced aortitis.
Show evidence (1 reference)
DOI:10.3389/fphar.2024.1487501 SUPPORT Human Clinical
"The G-CSF type with the highest frequency of occurrence of aortitis is pegfilgrastim."
Identifies pegfilgrastim as the most frequently reported G-CSF exposure in G-CSF-associated aortitis.
Current tobacco smoking
exposure to tobacco smoking link
Current smoking was associated with histology-confirmed aortitis in a major aortic surgery cohort.
Show evidence (1 reference)
DOI:10.3390/jcdd11120405 SUPPORT Human Clinical
"Multivariate logistic regression identified increased age, female sex, current smoking, and other inflammatory diseases as significantly associated with aortitis"
Supports current smoking as a clinical risk association in the surgical aortitis cohort.
{ }

Source YAML

click to show 
name: Aortitis
creation_date: "2026-05-06T03:09:08Z"
updated_date: "2026-05-06T22:51:11Z"
description: >-
  Aortitis is inflammation of the aortic wall, spanning noninfectious
  large-vessel vasculitis, clinically isolated surgical-pathology aortitis,
  infectious aortitis, and drug-induced forms such as G-CSF-associated
  aortitis. The shared disease axis is aortic wall inflammation with risk for
  aneurysm, dissection, stenotic or occlusive disease, ischemic complications,
  and aortic valve involvement, but the required management differs sharply by
  etiology.
category: Complex
disease_term:
  preferred_term: aortitis
  term:
    id: MONDO:0006656
    label: aortitis
parents:
- Vascular disorder
- Vasculitis
- Inflammatory disorder
synonyms:
- Aorta inflammation
- Inflammation of aorta
- Aortic wall inflammation
has_subtypes:
- name: Clinically isolated aortitis
  description: >-
    Histology-confirmed aortitis without known systemic inflammatory or
    infectious disease; many cases are discovered incidentally during aortic
    surgery.
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The prevalence of aortitis was 10.6% (n = 57), of which 75% were clinically isolated."
    explanation: >-
      This surgical cohort identifies clinically isolated aortitis as the
      majority subtype among histology-confirmed aortitis cases.
- name: Large-vessel vasculitis-associated aortitis
  description: >-
    Aortitis associated with giant cell arteritis, Takayasu arteritis, or other
    large- and variable-vessel vasculitides.
  evidence:
  - reference: DOI:10.3390/jcm13216364
    reference_title: "Imaging in Large Vessel Vasculitis-A Narrative Review"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Large vessel vasculitis (LVV), particularly giant cell arteritis (GCA) and Takayasu arteritis (TAK), has garnered attention due to its significant morbidity and mortality."
    explanation: >-
      This review anchors GCA and Takayasu arteritis as major large-vessel
      vasculitis contexts relevant to aortitis.
- name: Infectious aortitis
  description: >-
    Aortitis caused by microbial infection of the aortic wall, often presenting
    with aneurysm or structural aortic abnormalities and requiring urgent
    antimicrobial and vascular-surgery evaluation.
  evidence:
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each"
    explanation: >-
      This multicenter aortitis cohort identifies infectious aortitis cases and
      their causative organisms.
- name: G-CSF-associated drug-induced aortitis
  description: >-
    A rare adverse event after granulocyte-colony stimulating factor exposure,
    most often reported in oncology supportive-care settings and occasionally in
    healthy stem-cell donors.
  evidence:
  - reference: DOI:10.3389/fphar.2024.1487501
    reference_title: "Literature review analysis of aortitis induced by granulocyte-colony stimulating factor"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Recombinant human granulocyte-colony stimulating factors (G-CSF)-induced aortitis is a rare but particularly serious adverse event, commonly seen in cancer patients undergoing chemotherapy."
    explanation: >-
      This literature review supports G-CSF exposure as a drug-induced aortitis
      subtype, especially in chemotherapy care.
- name: IgG4-related aortitis/periaortitis
  description: >-
    Aortitis or periaortitis occurring in the context of IgG4-related disease,
    characterized by IgG4-bearing plasma-cell infiltration and fibrotic
    inflammation that predominantly affects the abdominal aorta and iliac
    arteries.
  evidence:
  - reference: DOI:10.3389/fimmu.2025.1625456
    reference_title: "Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs."
    explanation: >-
      The review defines IgG4-related disease by IgG4-bearing plasma-cell
      infiltration and fibrotic inflammation.
  - reference: DOI:10.3389/fimmu.2025.1625456
    reference_title: "Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "IgG4-related aortitis/periaortitis and periarteritis (IgG4-related PAO/PA) predominantly affect the abdominal aorta and iliac arteries, with a higher prevalence in elderly males."
    explanation: >-
      This directly supports IgG4-related aortitis/periaortitis as a distinct
      vascular manifestation.
prevalence:
- population: Single-center major aortic surgery cohort with high histology sampling
  percentage: 10.6%
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The prevalence of aortitis was 10.6% (n = 57), of which 75% were clinically isolated."
    explanation: >-
      Provides the observed prevalence of histology-confirmed aortitis in a
      high-sampling surgical cohort.
epidemiology:
- name: Infectious versus noninfectious aortitis distribution
  description: >-
    In a French multicenter comparison, infectious aortitis accounted for about
    one-third of included aortitis cases and had distinct microbiologic and
    aneurysm patterns.
  factors:
  - microbial etiology
  - abdominal aortic involvement
  - aneurysm at presentation
  evidence:
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each"
    explanation: >-
      Quantifies infectious aortitis frequency and key organisms in a mixed
      infectious/noninfectious cohort.
- name: G-CSF-induced aortitis case-review profile
  description: >-
    The published case-review dataset is enriched for older women and oncology
    patients, with pegfilgrastim the most frequently implicated G-CSF agent.
    Case literature also suggests recurrence can occur after switching between
    short-acting G-CSF products in a genetically susceptible patient.
  factors:
  - G-CSF exposure
  - cancer chemotherapy
  - pegfilgrastim exposure
  - HLA-B52 susceptibility signal
  evidence:
  - reference: DOI:10.3389/fphar.2024.1487501
    reference_title: "Literature review analysis of aortitis induced by granulocyte-colony stimulating factor"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "A total of 72 patients were enrolled, including 14 males and 58 females"
    explanation: >-
      Summarizes the demographic profile of reported G-CSF-associated aortitis
      cases.
  - reference: PMID:38521841
    reference_title: Aortitis after switching short-acting granulocyte colony-stimulating factors in a lymphoma patient with HLA-B52.
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      The patient possessed human leukocyte antigen B52, which has been implicated
      in Takayasu arteritis.
    explanation: This case report supports an HLA-B52 susceptibility signal in G-CSF-associated aortitis, but it is single-patient evidence.
pathophysiology:
- name: Immune-mediated aortic wall inflammation in large-vessel vasculitis
  description: >-
    Large-vessel vasculitis-associated aortitis involves immune-mediated
    inflammation of the vascular wall, including inflammatory cell accumulation
    in aortic tissue in Takayasu arteritis.
  cell_types:
  - preferred_term: T cell
    term:
      id: CL:0000084
      label: T cell
  - preferred_term: macrophage
    term:
      id: CL:0000235
      label: macrophage
  - preferred_term: granulocyte
    term:
      id: CL:0000094
      label: granulocyte
  - preferred_term: endothelial cell
    term:
      id: CL:0000115
      label: endothelial cell
  - preferred_term: smooth muscle cell
    term:
      id: CL:0000192
      label: smooth muscle cell
  biological_processes:
  - preferred_term: inflammatory response
    modifier: INCREASED
    term:
      id: GO:0006954
      label: inflammatory response
  - preferred_term: adaptive immune response
    modifier: ABNORMAL
    term:
      id: GO:0002250
      label: adaptive immune response
  locations:
  - preferred_term: aorta
    term:
      id: UBERON:0000947
      label: aorta
  evidence:
  - reference: DOI:10.3390/jcm13216364
    reference_title: "Imaging in Large Vessel Vasculitis-A Narrative Review"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Both conditions involve immune-mediated inflammation of the vascular wall, despite differing in epidemiology and presentation."
    explanation: >-
      Supports immune-mediated vascular-wall inflammation as a shared mechanism
      in the GCA/TAK large-vessel vasculitis context.
  - reference: DOI:10.1177/000331970005100705
    reference_title: "Accumulation of Lymphocytes, Dendritic Cells, and Granulocytes in the Aortic Wall Affected by Takayasu's Disease"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "CD68 (macrophages), CD15 (granulocytes), von Willebrand factor (endothelial cells), and alpha-smooth muscle actin (smooth muscle cells)."
    explanation: >-
      Directly supports macrophage and granulocyte involvement in affected
      aortic wall tissue in Takayasu arteritis.
  - reference: DOI:10.1177/000331970005100705
    reference_title: "Accumulation of Lymphocytes, Dendritic Cells, and Granulocytes in the Aortic Wall Affected by Takayasu's Disease"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "(endothelial cells), and alpha-smooth muscle actin (smooth muscle cells)."
    explanation: >-
      Supports endothelial-cell and smooth-muscle-cell assessment in affected
      aortic wall tissue.
  downstream:
  - target: Aortic wall remodeling and structural complications
    description: >-
      Persistent vascular inflammation contributes to later remodeling and
      structural complications such as aneurysm, dissection, or ischemia.
- name: Infectious aortic wall infection and aneurysmal injury
  description: >-
    Infectious aortitis is initiated by microbial infection of native aortic
    tissue, producing wall thickening or aneurysmal distortion and a higher
    mortality-risk phenotype than noninfectious aortitis.
  biological_processes:
  - preferred_term: inflammatory response
    modifier: INCREASED
    term:
      id: GO:0006954
      label: inflammatory response
  locations:
  - preferred_term: aorta
    term:
      id: UBERON:0000947
      label: aorta
  evidence:
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "One hundred eighty-three patients were included. Of these, 66 had IA (36.1%); the causative organism was Enterobacterales and streptococci in 18.2% each"
    explanation: >-
      Identifies causative organisms in infectious aortitis and supports
      microbial infection as the upstream etiology.
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001)."
    explanation: >-
      Links infectious aortitis to aneurysmal aortic injury more strongly than
      noninfectious aortitis in the comparison cohort.
  downstream:
  - target: Aortic aneurysm
    description: Infectious wall injury commonly presents with aneurysmal aortic disease.
    evidence:
    - reference: DOI:10.1093/cid/ciac560
      reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: "IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001)."
      explanation: >-
        Provides cohort-level evidence that infectious aortitis is strongly
        associated with aneurysm.
- name: G-CSF-associated aortic inflammation
  description: >-
    Exogenous G-CSF exposure can trigger aortitis as a serious adverse event,
    most often detected by CT in the aortic arch and branch vessels.
  biological_processes:
  - preferred_term: cytokine-mediated signaling pathway
    modifier: ABNORMAL
    term:
      id: GO:0019221
      label: cytokine-mediated signaling pathway
  - preferred_term: inflammatory response
    modifier: INCREASED
    term:
      id: GO:0006954
      label: inflammatory response
  locations:
  - preferred_term: aorta
    term:
      id: UBERON:0000947
      label: aorta
  triggers:
  - preferred_term: granulocyte-colony stimulating factor exposure
  evidence:
  - reference: DOI:10.3389/fphar.2024.1487501
    reference_title: "Literature review analysis of aortitis induced by granulocyte-colony stimulating factor"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The G-CSF type with the highest frequency of occurrence of aortitis is pegfilgrastim."
    explanation: >-
      Identifies G-CSF exposure, particularly pegfilgrastim, as the most common
      trigger in the case-review dataset.
  - reference: PMID:38521841
    reference_title: Aortitis after switching short-acting granulocyte colony-stimulating factors in a lymphoma patient with HLA-B52.
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      switching from one short-acting G-CSF to another does not prevent recurrence
      of G-CSF-associated aortitis.
    explanation: The case supports recurrence after switching short-acting G-CSF preparations and helps explain why re-exposure should be treated cautiously.
- name: IgG4-related plasma-cell fibrotic vascular inflammation
  description: >-
    IgG4-related aortitis/periaortitis involves chronic fibrotic inflammation
    with IgG4-bearing plasma-cell infiltration in large-vessel tissue.
  cell_types:
  - preferred_term: plasma cell
    term:
      id: CL:0000786
      label: plasma cell
  biological_processes:
  - preferred_term: inflammatory response
    modifier: INCREASED
    term:
      id: GO:0006954
      label: inflammatory response
  - preferred_term: extracellular matrix organization
    modifier: ABNORMAL
    term:
      id: GO:0030198
      label: extracellular matrix organization
  locations:
  - preferred_term: aorta
    term:
      id: UBERON:0000947
      label: aorta
  evidence:
  - reference: DOI:10.3389/fimmu.2025.1625456
    reference_title: "Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs."
    explanation: >-
      Supports a fibrotic inflammatory and IgG4-bearing plasma-cell mechanism
      for IgG4-related large-vessel disease.
  downstream:
  - target: Aortic wall remodeling and structural complications
    description: Fibrotic inflammatory large-vessel disease can contribute to aneurysm, dissection, or rupture.
- name: Aortic wall remodeling and structural complications
  description: >-
    Inflammatory injury can weaken and remodel the aortic wall, producing
    aneurysm or dissection and driving the need for long-term aortic follow-up.
  cell_types:
  - preferred_term: smooth muscle cell
    term:
      id: CL:0000192
      label: smooth muscle cell
  - preferred_term: fibroblast
    term:
      id: CL:0000057
      label: fibroblast
  biological_processes:
  - preferred_term: extracellular matrix organization
    modifier: ABNORMAL
    term:
      id: GO:0030198
      label: extracellular matrix organization
  locations:
  - preferred_term: aorta
    term:
      id: UBERON:0000947
      label: aorta
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
    explanation: >-
      Directly links aortic inflammation to the principal structural
      complications of aneurysm and dissection.
  downstream:
  - target: Aortic aneurysm
    description: Aortic wall remodeling can present as aneurysmal dilation.
  - target: Aortic dissection
    description: Weakened and inflamed aortic wall can dissect.
phenotypes:
- category: Cardiovascular
  name: Aortitis
  diagnostic: true
  description: Inflammation of the aortic wall is the defining feature of aortitis.
  phenotype_term:
    preferred_term: Aortitis
    term:
      id: HP:6001461
      label: Aortitis
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
    explanation: The abstract defines aortitis as inflammation of the aorta.
- category: Cardiovascular
  name: Aortic aneurysm
  description: Aortic wall inflammation can weaken the vessel wall and contribute to aneurysmal dilation.
  phenotype_term:
    preferred_term: Aortic aneurysm
    term:
      id: HP:0004942
      label: Aortic aneurysm
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
    explanation: The abstract directly names aneurysm as a complication of aortitis.
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001)."
    explanation: >-
      The infectious/noninfectious comparison shows a strong association between
      infectious aortitis and aortic aneurysm.
- category: Cardiovascular
  name: Aortic dissection
  description: Severe inflammatory weakening of the aorta can predispose to dissection.
  phenotype_term:
    preferred_term: Aortic dissection
    term:
      id: HP:0002647
      label: Aortic dissection
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
    explanation: The abstract directly names dissection as a complication of aortitis.
- category: Cardiovascular
  name: Aortic regurgitation
  description: Aortic root or ascending aortic inflammation can impair aortic valve competence.
  phenotype_term:
    preferred_term: Aortic regurgitation
    term:
      id: HP:0001659
      label: Aortic regurgitation
  evidence:
  - reference: DOI:10.62186/001c.146456
    reference_title: "Stroke, Aortic Regurgitation and Aortic Aneurysm in Younger Female: Case of Giant Cell Aortitis and Discussion"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Due to severe aortic regurgitation limiting her activity and aortic root aneurysm"
    explanation: >-
      This case report links giant cell aortitis with severe aortic
      regurgitation and aortic root aneurysm.
- category: Constitutional
  name: Fever
  description: >-
    Fever is a common presentation in symptomatic G-CSF-associated aortitis and
    can trigger evaluation for drug-induced aortic inflammation.
  subtype: G-CSF-associated drug-induced aortitis
  phenotype_term:
    preferred_term: Fever
    term:
      id: HP:0001945
      label: Fever
  evidence:
  - reference: PMID:40272133
    reference_title: Aortitis triggered by granulocyte-colony stimulating factor.
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The diagnosis of G-CSF-induced aortitis should be considered in patients with fever after G-CSF treatment, particularly if adequate antibiotic treatment does not lead to improvement."
    explanation: >-
      The case report interpretation explicitly links fever after G-CSF
      exposure to consideration of G-CSF-induced aortitis.
  - reference: PMID:34448091
    reference_title: "Drug-induced aortitis of the subclavian artery caused by pegfilgrastim: a case report."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "If a patient develops a persistent high fever of unknown origin after pegfilgrastim administration, drug-induced aortitis should be considered during investigations for causes of fever."
    explanation: >-
      The case-report conclusion supports persistent high fever as a key
      clinical clue for pegfilgrastim-induced aortitis.
- category: Laboratory
  name: Elevated C-reactive protein
  description: >-
    C-reactive protein may be markedly elevated in drug-induced aortitis and is
    used clinically as an inflammatory activity marker.
  subtype: G-CSF-associated drug-induced aortitis
  phenotype_term:
    preferred_term: Elevated circulating C-reactive protein concentration
    term:
      id: HP:0011227
      label: Elevated circulating C-reactive protein concentration
  evidence:
  - reference: PMID:40272133
    reference_title: Aortitis triggered by granulocyte-colony stimulating factor.
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "After four days, C-reactive protein (CRP) had increased from 104 to 331, but the patient's condition was largely unchanged."
    explanation: >-
      The case report documents marked CRP increase during G-CSF-associated
      aortitis evaluation.
  - reference: PMID:34448091
    reference_title: "Drug-induced aortitis of the subclavian artery caused by pegfilgrastim: a case report."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Therefore, C-reactive protein (CRP) is currently used to assess disease activity in clinical practice."
    explanation: >-
      The full-text case report notes CRP use for assessing G-CSF-associated
      aortitis activity.
- category: Laboratory
  name: Elevated erythrocyte sedimentation rate
  description: >-
    Elevated erythrocyte sedimentation rate can accompany large-vessel
    vasculitis-associated aortitis presentations and supports inflammatory
    activity assessment.
  subtype: Large-vessel vasculitis-associated aortitis
  phenotype_term:
    preferred_term: Elevated erythrocyte sedimentation rate
    term:
      id: HP:0003565
      label: Elevated erythrocyte sedimentation rate
  evidence:
  - reference: PMID:36843728
    reference_title: "From Temporal Cell Arteritis to Giant Cell Aortitis Presenting as a Constitutional Syndrome: A Case Report."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated, and he also had inflammatory anemia with a hemoglobin of 11.7 g/L."
    explanation: >-
      This giant-cell-aortitis case report documents elevated ESR and CRP in the
      inflammatory presentation.
histopathology:
- name: Aortic wall inflammatory infiltrates
  description: >-
    Aortitis can show inflammatory infiltrates in the aortic wall, with
    lymphocytes, dendritic cells, macrophages, granulocytes, endothelial cells,
    and smooth muscle cells characterized in Takayasu aortic tissue.
  diagnostic: true
  context: Large-vessel vasculitis-associated aortitis
  evidence:
  - reference: DOI:10.1177/000331970005100705
    reference_title: "Accumulation of Lymphocytes, Dendritic Cells, and Granulocytes in the Aortic Wall Affected by Takayasu's Disease"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "All specimens showed distinctive histologic features of Takayasu's arteritis and contained inflammatory infiltrates"
    explanation: >-
      Histologic inflammatory infiltrates in aortic wall tissue support this
      microscopic aortitis feature.
- name: Histology-confirmed clinically isolated aortitis
  description: >-
    Clinically isolated aortitis is often diagnosed through intra-operative
    aortic sampling rather than through preoperative clinical symptoms.
  diagnostic: true
  context: Major aortic surgery
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Intra-operative sampling is essential for diagnosis, with many cases presenting asymptomatically as clinically isolated aortitis."
    explanation: >-
      Supports intra-operative histologic sampling as a key diagnostic route for
      clinically isolated aortitis.
environmental:
- name: Granulocyte-colony stimulating factor exposure
  presence: Positive
  description: >-
    G-CSF exposure, particularly pegfilgrastim in reported cases, can trigger
    drug-induced aortitis.
  evidence:
  - reference: DOI:10.3389/fphar.2024.1487501
    reference_title: "Literature review analysis of aortitis induced by granulocyte-colony stimulating factor"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The G-CSF type with the highest frequency of occurrence of aortitis is pegfilgrastim."
    explanation: >-
      Identifies pegfilgrastim as the most frequently reported G-CSF exposure in
      G-CSF-associated aortitis.
- name: Current tobacco smoking
  presence: Positive
  description: >-
    Current smoking was associated with histology-confirmed aortitis in a major
    aortic surgery cohort.
  exposure_term:
    preferred_term: exposure to tobacco smoking
    term:
      id: ECTO:6000029
      label: exposure to tobacco smoking
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Multivariate logistic regression identified increased age, female sex, current smoking, and other inflammatory diseases as significantly associated with aortitis"
    explanation: >-
      Supports current smoking as a clinical risk association in the surgical
      aortitis cohort.
diagnosis:
- name: Histologic aortic wall assessment
  diagnosis_term:
    preferred_term: diagnostic procedure
    term:
      id: MAXO:0000003
      label: diagnostic procedure
  description: >-
    Intra-operative aortic sampling can diagnose clinically isolated aortitis
    that may be missed clinically.
  results: Aortic wall inflammation on histology supports the diagnosis.
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Intra-operative sampling is essential for diagnosis, with many cases presenting asymptomatically as clinically isolated aortitis."
    explanation: >-
      Supports histologic assessment as a diagnostic approach in surgical
      aortitis and clinically isolated aortitis.
- name: Large-vessel vascular imaging
  diagnosis_term:
    preferred_term: diagnostic procedure
    term:
      id: MAXO:0000003
      label: diagnostic procedure
  description: >-
    CTA, ultrasound, MRI, and FDG-PET are used to detect large-vessel vasculitis
    activity, wall thickening, and aortic or branch-vessel involvement.
  results: Vessel-wall thickening or FDG uptake supports active vascular inflammation.
  evidence:
  - reference: DOI:10.3390/jcm13216364
    reference_title: "Imaging in Large Vessel Vasculitis-A Narrative Review"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Vascular imaging, including computed tomography angiography (CTA), ultrasound (US), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT), is key in diagnosing vasculitis, revealing vessel wall thickening and other suggestive features."
    explanation: >-
      Supports vascular imaging as a diagnostic method for large-vessel
      vasculitis-associated aortitis.
  - reference: DOI:10.1136/rmdopen-2023-003379
    reference_title: "Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Ultrasound, MRI and FDG-PET revealed a good performance for the diagnosis of GCA."
    explanation: >-
      Supports ultrasound, MRI, and FDG-PET as evidence-backed imaging tools in
      giant-cell-arteritis large-vessel disease.
- name: Infectious versus noninfectious etiology assessment
  diagnosis_term:
    preferred_term: diagnostic procedure
    term:
      id: MAXO:0000003
      label: diagnostic procedure
  description: >-
    Evaluation should distinguish infectious aortitis from noninfectious
    aortitis because treatment and mortality risk differ.
  results: Microbiologic evidence and aortic imaging pattern help classify etiology.
  evidence:
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Determining the etiology of aortitis is often challenging, in particular to distinguish infectious aortitis (IA) and noninfectious aortitis (NIA)."
    explanation: >-
      Supports explicit infectious/noninfectious etiologic assessment during
      aortitis diagnosis.
  - reference: DOI:10.3390/jcm13216364
    reference_title: "Imaging in Large Vessel Vasculitis-A Narrative Review"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Vascular imaging, including computed tomography angiography (CTA), ultrasound (US), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT), is key in diagnosing vasculitis, revealing vessel wall thickening and other suggestive features."
    explanation: >-
      Supports vascular imaging as a central diagnostic tool when evaluating
      aortitis and large-vessel vasculitis.
treatments:
- name: Glucocorticoid therapy for noninfectious aortitis
  description: >-
    Glucocorticoids are a first-line immunosuppressive treatment backbone for
    noninfectious large-vessel aortitis contexts, including giant cell arteritis
    and IgG4-related aortitis/periaortitis.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: glucocorticoid
      term:
        id: CHEBI:24261
        label: glucocorticoid
    - preferred_term: prednisolone
      term:
        id: CHEBI:8378
        label: prednisolone
  evidence:
  - reference: DOI:10.1186/s13063-024-07905-4
    reference_title: "The Meteoritics Trial: efficacy of methotrexate after remission-induction with tocilizumab and glucocorticoids in giant cell arteritis—study protocol for a randomized, double-blind, placebo-controlled, parallel-group phase II study"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Glucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates."
    explanation: >-
      Giant cell arteritis is a major large-vessel vasculitis context for
      aortitis, and this trial protocol identifies glucocorticoids as standard
      treatment.
  - reference: PMID:36843728
    reference_title: "From Temporal Cell Arteritis to Giant Cell Aortitis Presenting as a Constitutional Syndrome: A Case Report."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Treatment with high-dose steroids should be initiated as soon as possible to rapidly control the inflammatory symptoms and prevent ischemic complications"
    explanation: >-
      This giant-cell-arteritis/aortitis case report supports high-dose steroid
      treatment for inflammatory large-vessel disease.
  - reference: DOI:10.3389/fimmu.2025.1625456
    reference_title: "Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Glucocorticoids (GCs) are the mainstay of treatment, often combined with immunosuppressants (IMs), while B- and T-cell-targeted therapies are under investigation."
    explanation: >-
      The IgG4-related aortitis review directly supports glucocorticoids as a
      mainstay treatment for IgG4-related aortitis/periaortitis.
- name: Empiric antimicrobial therapy for infectious aortitis
  description: >-
    Infectious aortitis requires early antimicrobial therapy, initially empiric
    when microbiologic documentation is pending and then adapted to organism and
    susceptibility data.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
  evidence:
  - reference: DOI:10.1093/cid/ciac560
    reference_title: "Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Effective empiric antimicrobial therapy, initiated before any microbial documentation, was associated with a decreased mortality"
    explanation: >-
      Supports early empiric antimicrobial therapy as a mortality-associated
      protective intervention in infectious aortitis.
- name: Tocilizumab for LVV-associated aortitis contexts
  description: >-
    Tocilizumab may be used for large-vessel vasculitis phenotypes associated
    with aortitis, with clinical remission and glucocorticoid-sparing effects
    reported in LV-GCA and Takayasu arteritis cohorts.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
  evidence:
  - reference: DOI:10.3390/sci7010012
    reference_title: "Tocilizumab in Extracranial Giant-Cell Arteritis and Takayasu Arteritis: A Multicentric Observational Comparative Study"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK)."
    explanation: >-
      Supports tocilizumab as a treatment option in the LVV contexts that can
      include aortitis.
  - reference: DOI:10.3390/sci7010012
    reference_title: "Tocilizumab in Extracranial Giant-Cell Arteritis and Takayasu Arteritis: A Multicentric Observational Comparative Study"
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: "complete imaging resolution was observed in only 18.9% of LV-GCA patients and 21.1% of TAK patients."
    explanation: >-
      The clinical benefit is tempered by incomplete imaging resolution,
      supporting a partial rather than complete effect on vascular inflammation.
- name: Upadacitinib for giant-cell-arteritis contexts
  description: >-
    Upadacitinib is a JAK inhibitor with phase III evidence for giant cell
    arteritis, a major large-vessel vasculitis context that can include
    aortitis. Its long-term effect on aortic remodeling remains uncertain.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
  evidence:
  - reference: PMID:40174237
    reference_title: Upadacitinib for Giant-Cell Arteritis.
    supports: PARTIAL
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      In patients with giant-cell arteritis, upadacitinib at a dose of 15 mg - but
      not 7.5 mg - with a 26-week glucocorticoid taper showed efficacy superior
      to that of placebo with a 52-week glucocorticoid taper.
    explanation: The SELECT-GCA phase III trial supports upadacitinib efficacy in GCA, but not specifically aortic remodeling or aortitis endpoints.
- name: Specialist multidisciplinary follow-up and aortic surveillance
  description: >-
    Patients with aortitis require specialist follow-up because of increased
    re-intervention risk and late aortic complications.
  treatment_term:
    preferred_term: clinical assessment
    term:
      id: MAXO:0000487
      label: clinical assessment
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Due to the increased re-intervention in aortitis, specialist multi-disciplinary follow-up and aortitis centres should be formed."
    explanation: >-
      Supports specialist multidisciplinary follow-up for aortitis after major
      aortic surgery.
  - reference: PMID:40099651
    reference_title: Isolated aortitis - an approach to diagnosis and management.
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: >-
      Surveillance of patients with IA with repeated clinical assessments and imaging
      is recommended.
    explanation: This review supports repeated clinical and imaging surveillance for isolated aortitis.
progression:
- phase: Asymptomatic discovery during aortic surgery
  subtype: Clinically isolated aortitis
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Intra-operative sampling is essential for diagnosis, with many cases presenting asymptomatically as clinically isolated aortitis."
    explanation: >-
      Supports asymptomatic surgical discovery as an important presentation mode
      for clinically isolated aortitis.
- phase: Re-intervention after aortitis-associated aortic surgery
  notes: Re-operation risk is higher in aortitis than in non-aortitis surgical comparators.
  evidence:
  - reference: DOI:10.3390/jcdd11120405
    reference_title: "Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery"
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The re-operation rate in aortitis was twice that of non-aortitis patients (17.5% vs. 9.4%, p = 0.054)."
    explanation: >-
      Quantifies higher post-surgical re-operation rates in the aortitis cohort.
references:
- reference: DOI:10.3390/jcdd11120405
  title: Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections.
    supporting_text: "Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
- reference: DOI:10.3389/fphar.2024.1487501
  title: Literature review analysis of aortitis induced by granulocyte-colony stimulating factor
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Recombinant human granulocyte-colony stimulating factors (G-CSF)-induced aortitis is a rare but particularly serious adverse event, commonly seen in cancer patients undergoing chemotherapy.
    supporting_text: "Recombinant human granulocyte-colony stimulating factors (G-CSF)-induced aortitis is a rare but particularly serious adverse event, commonly seen in cancer patients undergoing chemotherapy."
- reference: DOI:10.3389/fimmu.2025.1625456
  title: "Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice"
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs.
    supporting_text: "IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs."
- reference: DOI:10.1186/s13063-024-07905-4
  title: "The Meteoritics Trial: efficacy of methotrexate after remission-induction with tocilizumab and glucocorticoids in giant cell arteritis—study protocol for a randomized, double-blind, placebo-controlled, parallel-group phase II study"
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Glucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates.
    supporting_text: "Glucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates."
- reference: DOI:10.1093/cid/ciac560
  title: Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Infectious aortitis had higher aneurysm association and lower survival than noninfectious aortitis.
    supporting_text: "IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P < .001), especially located in the abdominal aorta (69.7% vs 23.1%, P < .001)."
- reference: DOI:10.1136/rmdopen-2023-003379
  title: "Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations"
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV.
    supporting_text: "To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV."
- reference: DOI:10.3390/jcm13216364
  title: Imaging in Large Vessel Vasculitis—A Narrative Review
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems.
    supporting_text: "Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems."
- reference: DOI:10.3390/sci7010012
  title: "Tocilizumab in Extracranial Giant-Cell Arteritis and Takayasu Arteritis: A Multicentric Observational Comparative Study"
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK).
    supporting_text: "Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK)."
- reference: DOI:10.62186/001c.146456
  title: "Stroke, Aortic Regurgitation and Aortic Aneurysm in Younger Female: Case of Giant Cell Aortitis and Discussion"
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: The author presents the case of a middle-aged woman who had a left parietal lobe infarct.
    supporting_text: "The author presents the case of a middle-aged woman who had a left parietal lobe infarct."
- reference: DOI:10.1177/000331970005100705
  title: "Accumulation of Lymphocytes, Dendritic Cells, and Granulocytes in the Aortic Wall Affected by Takayasu's Disease"
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Aortic wall tissue in Takayasu arteritis contains inflammatory infiltrates and multiple immune cell populations.
    supporting_text: "All specimens showed distinctive histologic features of Takayasu's arteritis and contained inflammatory infiltrates"
- reference: PMID:40272133
  title: Aortitis triggered by granulocyte-colony stimulating factor.
  findings:
  - statement: G-CSF-associated aortitis should be considered in patients with fever after G-CSF treatment.
    supporting_text: "The diagnosis of G-CSF-induced aortitis should be considered in patients with fever after G-CSF treatment, particularly if adequate antibiotic treatment does not lead to improvement."
- reference: PMID:34448091
  title: "Drug-induced aortitis of the subclavian artery caused by pegfilgrastim: a case report."
  findings:
  - statement: Persistent high fever and CRP can support evaluation for pegfilgrastim-induced aortitis.
    supporting_text: "She was hospitalized on day 15 when CRP increased to 21.5 mg/dL and the high fever continued."
- reference: PMID:36843728
  title: "From Temporal Cell Arteritis to Giant Cell Aortitis Presenting as a Constitutional Syndrome: A Case Report."
  findings:
  - statement: Giant cell aortitis can present with constitutional symptoms and elevated ESR/CRP.
    supporting_text: "The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated, and he also had inflammatory anemia with a hemoglobin of 11.7 g/L."
- reference: DOI:10.1016/j.berh.2023.101856
  title: 'Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow'
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: 'Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow'
    supporting_text: 'Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow'
- reference: DOI:10.1055/a-2765-8610
  title: 'Thoracic Aortic Aneurysm and Giant Cell Arteritis: Clarifying the Link'
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: We aim to better define the association between thoracic aortic aneurysm (TAA) and giant cell arteritis (GCA), thereby enhancing cross-diagnosis, monitoring, and therapy.
    supporting_text: We aim to better define the association between thoracic aortic aneurysm (TAA) and giant cell arteritis (GCA), thereby enhancing cross-diagnosis, monitoring, and therapy.
    evidence:
    - reference: DOI:10.1055/a-2765-8610
      reference_title: 'Thoracic Aortic Aneurysm and Giant Cell Arteritis: Clarifying the Link'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: We aim to better define the association between thoracic aortic aneurysm (TAA) and giant cell arteritis (GCA), thereby enhancing cross-diagnosis, monitoring, and therapy.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: DOI:10.1161/circgen.118.002296
  title: Single-Nucleotide Polymorphism of the <i>MLX</i> Gene Is Associated With Takayasu Arteritis
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Takayasu arteritis (TAK) is an autoimmune systemic arteritis of unknown pathogenesis.
    supporting_text: Takayasu arteritis (TAK) is an autoimmune systemic arteritis of unknown pathogenesis.
    evidence:
    - reference: DOI:10.1161/circgen.118.002296
      reference_title: Single-Nucleotide Polymorphism of the <i>MLX</i> Gene Is Associated With Takayasu Arteritis
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Takayasu arteritis (TAK) is an autoimmune systemic arteritis of unknown pathogenesis.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: DOI:10.31138/mjr.220125.era
  title: 'The Great Vasculitis Pretenders: Mycotic Pseudoaneurysm, Aortitis with Occlusive Iliac Thrombus, and Paraneoplastic Aortitis. A Case-Based Review'
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: 'The Great Vasculitis Pretenders: Mycotic Pseudoaneurysm, Aortitis with Occlusive Iliac Thrombus, and Paraneoplastic Aortitis. A Case-Based Review'
    supporting_text: 'The Great Vasculitis Pretenders: Mycotic Pseudoaneurysm, Aortitis with Occlusive Iliac Thrombus, and Paraneoplastic Aortitis. A Case-Based Review'
- reference: DOI:10.3389/fmed.2023.1103752
  title: 18-Fluorodeoxyglucose positron emission tomography/computed tomography for large vessel vasculitis in clinical practice
  found_in:
  - Aortitis-deep-research-falcon.md
  findings:
  - statement: Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging.
    supporting_text: Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging.
    evidence:
    - reference: DOI:10.3389/fmed.2023.1103752
      reference_title: 18-Fluorodeoxyglucose positron emission tomography/computed tomography for large vessel vasculitis in clinical practice
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:15675134
  title: Predictors of left ventricular dysfunction in patients with Takayasu's or giant cell aortitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: LVSD in patients with TA/GCA has been described in case reports and attributed variously to hemodynamic and immunologic factors.
    supporting_text: LVSD in patients with TA/GCA has been described in case reports and attributed variously to hemodynamic and immunologic factors.
    evidence:
    - reference: PMID:15675134
      reference_title: Predictors of left ventricular dysfunction in patients with Takayasu's or giant cell aortitis.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: LVSD in patients with TA/GCA has been described in case reports and attributed variously to hemodynamic and immunologic factors.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:19150884
  title: Toll-like receptors 4 and 5 induce distinct types of vasculitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2009 Feb 27;104(4):488-95. doi: 10.1161/CIRCRESAHA.108.185777.'
    supporting_text: '2009 Feb 27;104(4):488-95. doi: 10.1161/CIRCRESAHA.108.185777.'
    evidence:
    - reference: PMID:19150884
      reference_title: Toll-like receptors 4 and 5 induce distinct types of vasculitis.
      supports: SUPPORT
      evidence_source: MODEL_ORGANISM
      snippet: '2009 Feb 27;104(4):488-95. doi: 10.1161/CIRCRESAHA.108.185777.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:21124083
  title: IgG4-related inflammatory abdominal aortic aneurysm.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2011 Jan;23(1):18-23. doi: 10.1097/BOR.0b013e32833ee95f.'
    supporting_text: '2011 Jan;23(1):18-23. doi: 10.1097/BOR.0b013e32833ee95f.'
    evidence:
    - reference: PMID:21124083
      reference_title: IgG4-related inflammatory abdominal aortic aneurysm.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2011 Jan;23(1):18-23. doi: 10.1097/BOR.0b013e32833ee95f.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:21220737
  title: Blocking the NOTCH pathway inhibits vascular inflammation in large-vessel vasculitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Giant cell arteritis is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke, and aortic aneurysm.
    supporting_text: Giant cell arteritis is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke, and aortic aneurysm.
    evidence:
    - reference: PMID:21220737
      reference_title: Blocking the NOTCH pathway inhibits vascular inflammation in large-vessel vasculitis.
      supports: SUPPORT
      evidence_source: MODEL_ORGANISM
      snippet: Giant cell arteritis is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke, and aortic aneurysm.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:23843109
  title: Genetic component of giant cell arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2014 Jan;53(1):6-18. doi: 10.1093/rheumatology/ket231.'
    supporting_text: '2014 Jan;53(1):6-18. doi: 10.1093/rheumatology/ket231.'
    evidence:
    - reference: PMID:23843109
      reference_title: Genetic component of giant cell arteritis.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2014 Jan;53(1):6-18. doi: 10.1093/rheumatology/ket231.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:25658487
  title: Serpin treatment suppresses inflammatory vascular lesions in temporal artery implants (TAI) from patients with giant cell arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2015 Feb 6;10(2):e0115482. doi: 10.1371/journal.pone.0115482. eCollection 2015.'
    supporting_text: '2015 Feb 6;10(2):e0115482. doi: 10.1371/journal.pone.0115482. eCollection 2015.'
    evidence:
    - reference: PMID:25658487
      reference_title: Serpin treatment suppresses inflammatory vascular lesions in temporal artery implants (TAI) from patients with giant cell arteritis.
      supports: SUPPORT
      evidence_source: MODEL_ORGANISM
      snippet: '2015 Feb 6;10(2):e0115482. doi: 10.1371/journal.pone.0115482. eCollection 2015.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:25931203
  title: 'Takayasu arteritis and ulcerative colitis: high rate of co-occurrence and genetic overlap.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2015 May;67(8):2226-32. doi: 10.1002/art.39157.'
    supporting_text: '2015 May;67(8):2226-32. doi: 10.1002/art.39157.'
    evidence:
    - reference: PMID:25931203
      reference_title: 'Takayasu arteritis and ulcerative colitis: high rate of co-occurrence and genetic overlap.'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2015 May;67(8):2226-32. doi: 10.1002/art.39157.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:26051917
  title: 'Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2015 Sep-Oct;24(5):267-78. doi: 10.1016/j.carpath.2015.05.001.'
    supporting_text: '2015 Sep-Oct;24(5):267-78. doi: 10.1016/j.carpath.2015.05.001.'
    evidence:
    - reference: PMID:26051917
      reference_title: 'Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2015 Sep-Oct;24(5):267-78. doi: 10.1016/j.carpath.2015.05.001.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:26093659
  title: 'Epigenetics and Vasculitis: a Comprehensive Review.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2016 Jun;50(3):357-66. doi: 10.1007/s12016-015-8495-6.'
    supporting_text: '2016 Jun;50(3):357-66. doi: 10.1007/s12016-015-8495-6.'
    evidence:
    - reference: PMID:26093659
      reference_title: 'Epigenetics and Vasculitis: a Comprehensive Review.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2016 Jun;50(3):357-66. doi: 10.1007/s12016-015-8495-6.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:26775836
  title: '[Infectious aortitis caused by Streptococcus pneumoniae].'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2016 Feb;41(1):36-41. doi: 10.1016/j.jmv.2015.12.003.'
    supporting_text: '2016 Feb;41(1):36-41. doi: 10.1016/j.jmv.2015.12.003.'
    evidence:
    - reference: PMID:26775836
      reference_title: '[Infectious aortitis caused by Streptococcus pneumoniae].'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2016 Feb;41(1):36-41. doi: 10.1016/j.jmv.2015.12.003.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:27193038
  title: The Severity of Takayasu Arteritis Is Associated with the HLA-B52 Allele in Japanese Patients.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2016 May;239(1):67-72. doi: 10.1620/tjem.239.67.'
    supporting_text: '2016 May;239(1):67-72. doi: 10.1620/tjem.239.67.'
    evidence:
    - reference: PMID:27193038
      reference_title: The Severity of Takayasu Arteritis Is Associated with the HLA-B52 Allele in Japanese Patients.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2016 May;239(1):67-72. doi: 10.1620/tjem.239.67.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:27224742
  title: Varicella zoster virus triggers the immunopathology of giant cell arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2016 Jul;28(4):376-82. doi: 10.1097/BOR.0000000000000292.'
    supporting_text: '2016 Jul;28(4):376-82. doi: 10.1097/BOR.0000000000000292.'
    evidence:
    - reference: PMID:27224742
      reference_title: Varicella zoster virus triggers the immunopathology of giant cell arteritis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2016 Jul;28(4):376-82. doi: 10.1097/BOR.0000000000000292.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:27815653
  title: 'Relationship of HLA-B*51 and HLA-B*52 alleles and TNF-α-308A/G polymorphism with susceptibility to Takayasu arteritis: a meta-analysis.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2017 Jan;36(1):173-181. doi: 10.1007/s10067-016-3445-0.'
    supporting_text: '2017 Jan;36(1):173-181. doi: 10.1007/s10067-016-3445-0.'
    evidence:
    - reference: PMID:27815653
      reference_title: 'Relationship of HLA-B*51 and HLA-B*52 alleles and TNF-α-308A/G polymorphism with susceptibility to Takayasu arteritis: a meta-analysis.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2017 Jan;36(1):173-181. doi: 10.1007/s10067-016-3445-0.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:28277489
  title: Analysis of the common genetic component of large-vessel vasculitides through a meta-Immunochip strategy.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2017 Mar 9;7:43953. doi: 10.1038/srep43953.'
    supporting_text: '2017 Mar 9;7:43953. doi: 10.1038/srep43953.'
    evidence:
    - reference: PMID:28277489
      reference_title: Analysis of the common genetic component of large-vessel vasculitides through a meta-Immunochip strategy.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2017 Mar 9;7:43953. doi: 10.1038/srep43953.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:28457683
  title: '[Epidemiology and natural history of giant cell arteritis].'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2017 Oct;38(10):663-669. doi: 10.1016/j.revmed.2017.03.007.'
    supporting_text: '2017 Oct;38(10):663-669. doi: 10.1016/j.revmed.2017.03.007.'
    evidence:
    - reference: PMID:28457683
      reference_title: '[Epidemiology and natural history of giant cell arteritis].'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2017 Oct;38(10):663-669. doi: 10.1016/j.revmed.2017.03.007.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:28756072
  title: Epidemiology of Takayasu arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2017 Jul-Aug;46(7-8 Pt 2):e197-e203. doi: 10.1016/j.lpm.2017.05.034.'
    supporting_text: '2017 Jul-Aug;46(7-8 Pt 2):e197-e203. doi: 10.1016/j.lpm.2017.05.034.'
    evidence:
    - reference: PMID:28756072
      reference_title: Epidemiology of Takayasu arteritis.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2017 Jul-Aug;46(7-8 Pt 2):e197-e203. doi: 10.1016/j.lpm.2017.05.034.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:28957963
  title: An update on the role of epigenetics in systemic vasculitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2018 Jan;30(1):4-15. doi: 10.1097/BOR.0000000000000451.'
    supporting_text: '2018 Jan;30(1):4-15. doi: 10.1097/BOR.0000000000000451.'
    evidence:
    - reference: PMID:28957963
      reference_title: An update on the role of epigenetics in systemic vasculitis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2018 Jan;30(1):4-15. doi: 10.1097/BOR.0000000000000451.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:29105322
  title: Clinical characteristics and outcomes of 61 patients with chronic periaortitis including IgG4-related and non-IgG4-related cases.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194.'
    supporting_text: '2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194.'
    evidence:
    - reference: PMID:29105322
      reference_title: Clinical characteristics and outcomes of 61 patients with chronic periaortitis including IgG4-related and non-IgG4-related cases.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:29254929
  title: Inhibition of JAK-STAT Signaling Suppresses Pathogenic Immune Responses in Medium and Large Vessel Vasculitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Giant cell arteritis, a chronic autoimmune disease of the aorta and its large branches, is complicated by aneurysm formation, dissection, and arterial occlusions.
    supporting_text: Giant cell arteritis, a chronic autoimmune disease of the aorta and its large branches, is complicated by aneurysm formation, dissection, and arterial occlusions.
    evidence:
    - reference: PMID:29254929
      reference_title: Inhibition of JAK-STAT Signaling Suppresses Pathogenic Immune Responses in Medium and Large Vessel Vasculitis.
      supports: SUPPORT
      evidence_source: MODEL_ORGANISM
      snippet: Giant cell arteritis, a chronic autoimmune disease of the aorta and its large branches, is complicated by aneurysm formation, dissection, and arterial occlusions.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:30498034
  title: Genetic determinants and an epistasis of LILRA3 and HLA-B*52 in Takayasu arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2018 Dec 18;115(51):13045-13050. doi: 10.1073/pnas.1808850115.'
    supporting_text: '2018 Dec 18;115(51):13045-13050. doi: 10.1073/pnas.1808850115.'
    evidence:
    - reference: PMID:30498034
      reference_title: Genetic determinants and an epistasis of LILRA3 and HLA-B*52 in Takayasu arteritis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2018 Dec 18;115(51):13045-13050. doi: 10.1073/pnas.1808850115.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:30805622
  title: Markers of angiogenesis and macrophage products for predicting disease course and monitoring vascular inflammation in giant cell arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2019 Feb 25;58(8):1383-92. doi: 10.1093/rheumatology/kez034.'
    supporting_text: '2019 Feb 25;58(8):1383-92. doi: 10.1093/rheumatology/kez034.'
    evidence:
    - reference: PMID:30805622
      reference_title: Markers of angiogenesis and macrophage products for predicting disease course and monitoring vascular inflammation in giant cell arteritis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2019 Feb 25;58(8):1383-92. doi: 10.1093/rheumatology/kez034.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:30993253
  title: Microbiomes of Inflammatory Thoracic Aortic Aneurysms Due to Giant Cell Arteritis and Clinically Isolated Aortitis Differ From Those of Non-Inflammatory Aneurysms.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2019 Mar 15;4(1):105-123. doi: 10.20411/pai.v4i1.269. eCollection 2019.'
    supporting_text: '2019 Mar 15;4(1):105-123. doi: 10.20411/pai.v4i1.269. eCollection 2019.'
    evidence:
    - reference: PMID:30993253
      reference_title: Microbiomes of Inflammatory Thoracic Aortic Aneurysms Due to Giant Cell Arteritis and Clinically Isolated Aortitis Differ From Those of Non-Inflammatory Aneurysms.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2019 Mar 15;4(1):105-123. doi: 10.20411/pai.v4i1.269. eCollection 2019.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:31270110
  title: 2018 Update of the EULAR recommendations for the management of large vessel vasculitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: 2018 Update of the EULAR recommendations for the management of large vessel vasculitis
    supporting_text: Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations.
    evidence:
    - reference: PMID:31270110
      reference_title: 2018 Update of the EULAR recommendations for the management of large vessel vasculitis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:32234379
  title: 'Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018.'
    supporting_text: '2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018.'
    evidence:
    - reference: PMID:32234379
      reference_title: 'Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases.'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:33128155
  title: 'Clinically isolated aortitis: imaging features and clinical outcomes: comparison with giant cell arteritis and giant cell aortitis.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2021 Apr;37(4):1433-1443. doi: 10.1007/s10554-020-02087-x.'
    supporting_text: '2021 Apr;37(4):1433-1443. doi: 10.1007/s10554-020-02087-x.'
    evidence:
    - reference: PMID:33128155
      reference_title: 'Clinically isolated aortitis: imaging features and clinical outcomes: comparison with giant cell arteritis and giant cell aortitis.'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2021 Apr;37(4):1433-1443. doi: 10.1007/s10554-020-02087-x.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:33593995
  title: 'Aortitis: recent advances, current concepts and future possibilities.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2021 Oct;107(20):1620-1629. doi: 10.1136/heartjnl-2020-318085.'
    supporting_text: '2021 Oct;107(20):1620-1629. doi: 10.1136/heartjnl-2020-318085.'
    evidence:
    - reference: PMID:33593995
      reference_title: 'Aortitis: recent advances, current concepts and future possibilities.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2021 Oct;107(20):1620-1629. doi: 10.1136/heartjnl-2020-318085.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:33734973
  title: The presence of both HLA-DRB1*04:01 and HLA-B*15:01 increases the susceptibility to cranial and extracranial giant cell arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2021 Mar-Apr;39 Suppl 129(2):21-26. doi: 10.55563/clinexprheumatol/nn15lt.'
    supporting_text: '2021 Mar-Apr;39 Suppl 129(2):21-26. doi: 10.55563/clinexprheumatol/nn15lt.'
    evidence:
    - reference: PMID:33734973
      reference_title: The presence of both HLA-DRB1*04:01 and HLA-B*15:01 increases the susceptibility to cranial and extracranial giant cell arteritis.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2021 Mar-Apr;39 Suppl 129(2):21-26. doi: 10.55563/clinexprheumatol/nn15lt.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:36277471
  title: A Case Report of Immune Checkpoint Inhibitor-Induced Aortitis Treated with Tocilizumab.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2022 Oct 12;2022:7971169. doi: 10.1155/2022/7971169. eCollection 2022.'
    supporting_text: '2022 Oct 12;2022:7971169. doi: 10.1155/2022/7971169. eCollection 2022.'
    evidence:
    - reference: PMID:36277471
      reference_title: A Case Report of Immune Checkpoint Inhibitor-Induced Aortitis Treated with Tocilizumab.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2022 Oct 12;2022:7971169. doi: 10.1155/2022/7971169. eCollection 2022.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:36912345
  title: Cranial and extracranial giant cell arteritis do not exhibit differences in the IL6 -174 G/C gene polymorphism.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2023 Apr;41(4):910-915. doi: 10.55563/clinexprheumatol/cbjnmo.'
    supporting_text: '2023 Apr;41(4):910-915. doi: 10.55563/clinexprheumatol/cbjnmo.'
    evidence:
    - reference: PMID:36912345
      reference_title: Cranial and extracranial giant cell arteritis do not exhibit differences in the IL6 -174 G/C gene polymorphism.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2023 Apr;41(4):910-915. doi: 10.55563/clinexprheumatol/cbjnmo.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:37256147
  title: Arterial wall fibrosis in Takayasu arteritis and its potential for therapeutic modulation.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2023 May 15;14:1174249. doi: 10.3389/fimmu.2023.1174249. eCollection 2023.'
    supporting_text: '2023 May 15;14:1174249. doi: 10.3389/fimmu.2023.1174249. eCollection 2023.'
    evidence:
    - reference: PMID:37256147
      reference_title: Arterial wall fibrosis in Takayasu arteritis and its potential for therapeutic modulation.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2023 May 15;14:1174249. doi: 10.3389/fimmu.2023.1174249. eCollection 2023.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:37279826
  title: 'An unusual presentation of invasive Fusarium aortitis in a patient who is immunocompromised: A case report.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2023 Sep;134:102-105. doi: 10.1016/j.ijid.2023.05.069.'
    supporting_text: '2023 Sep;134:102-105. doi: 10.1016/j.ijid.2023.05.069.'
    evidence:
    - reference: PMID:37279826
      reference_title: 'An unusual presentation of invasive Fusarium aortitis in a patient who is immunocompromised: A case report.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2023 Sep;134:102-105. doi: 10.1016/j.ijid.2023.05.069.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:37304255
  title: 'Clinical experience and safety of Janus kinase inhibitors in giant cell arteritis: a retrospective case series from Sweden.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2023 May 25;14:1187584. doi: 10.3389/fimmu.2023.1187584. eCollection 2023.'
    supporting_text: '2023 May 25;14:1187584. doi: 10.3389/fimmu.2023.1187584. eCollection 2023.'
    evidence:
    - reference: PMID:37304255
      reference_title: 'Clinical experience and safety of Janus kinase inhibitors in giant cell arteritis: a retrospective case series from Sweden.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2023 May 25;14:1187584. doi: 10.3389/fimmu.2023.1187584. eCollection 2023.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:37673506
  title: Long-Term Outcome and Prognosis of Noninfectious Thoracic Aortitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Aortitis is a group of disorders characterized by the inflammation of the aorta.
    supporting_text: Aortitis is a group of disorders characterized by the inflammation of the aorta.
    evidence:
    - reference: PMID:37673506
      reference_title: Long-Term Outcome and Prognosis of Noninfectious Thoracic Aortitis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Aortitis is a group of disorders characterized by the inflammation of the aorta.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:37944298
  title: 'Beyond the symptoms: Personalizing giant cell arteritis care through multidimensional patient reported outcome measure.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50.
    supporting_text: Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50.
    evidence:
    - reference: PMID:37944298
      reference_title: 'Beyond the symptoms: Personalizing giant cell arteritis care through multidimensional patient reported outcome measure.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:38521841
  title: Aortitis after switching short-acting granulocyte colony-stimulating factors in a lymphoma patient with HLA-B52.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2024 May;119(5):608-612. doi: 10.1007/s12185-024-03744-w.'
    supporting_text: '2024 May;119(5):608-612. doi: 10.1007/s12185-024-03744-w.'
    evidence:
    - reference: PMID:38521841
      reference_title: Aortitis after switching short-acting granulocyte colony-stimulating factors in a lymphoma patient with HLA-B52.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2024 May;119(5):608-612. doi: 10.1007/s12185-024-03744-w.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:38734017
  title: 'Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older.
    supporting_text: Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older.
    evidence:
    - reference: PMID:38734017
      reference_title: 'Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:39714261
  title: 'Immune checkpoint inhibitors-associated vasculitis: a heterogeneous condition with possible severe disease course.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2025 Jun 1;64(6):3685-3690. doi: 10.1093/rheumatology/keae711.'
    supporting_text: '2025 Jun 1;64(6):3685-3690. doi: 10.1093/rheumatology/keae711.'
    evidence:
    - reference: PMID:39714261
      reference_title: 'Immune checkpoint inhibitors-associated vasculitis: a heterogeneous condition with possible severe disease course.'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2025 Jun 1;64(6):3685-3690. doi: 10.1093/rheumatology/keae711.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:39826312
  title: Histological pattern of non-infectious thoracic aortitis impacts mortality.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Non-infectious aortitis encompasses various histological patterns, but their specific cardiovascular outcomes remain unclear.
    supporting_text: Non-infectious aortitis encompasses various histological patterns, but their specific cardiovascular outcomes remain unclear.
    evidence:
    - reference: PMID:39826312
      reference_title: Histological pattern of non-infectious thoracic aortitis impacts mortality.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Non-infectious aortitis encompasses various histological patterns, but their specific cardiovascular outcomes remain unclear.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:40021438
  title: Aortic disease in giant cell arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2025 Jun;72S:152677. doi: 10.1016/j.semarthrit.2025.152677.'
    supporting_text: '2025 Jun;72S:152677. doi: 10.1016/j.semarthrit.2025.152677.'
    evidence:
    - reference: PMID:40021438
      reference_title: Aortic disease in giant cell arteritis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2025 Jun;72S:152677. doi: 10.1016/j.semarthrit.2025.152677.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:40099651
  title: Isolated aortitis - is it truly isolated? An approach to diagnosis and management.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2025 May 1;37(3):185-191. doi: 10.1097/BOR.0000000000001084.'
    supporting_text: '2025 May 1;37(3):185-191. doi: 10.1097/BOR.0000000000001084.'
    evidence:
    - reference: PMID:40099651
      reference_title: Isolated aortitis - is it truly isolated? An approach to diagnosis and management.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2025 May 1;37(3):185-191. doi: 10.1097/BOR.0000000000001084.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:40174237
  title: A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Giant-cell arteritis is a systemic vasculitis with limited treatment options.
    supporting_text: Giant-cell arteritis is a systemic vasculitis with limited treatment options.
    evidence:
    - reference: PMID:40174237
      reference_title: A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis.
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Giant-cell arteritis is a systemic vasculitis with limited treatment options.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:40349694
  title: Potential Key Genes for Giant Cell Arteritis Revealed Based on Single-Cell Sequencing and Mendelian Randomization Analysis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026;187(2):199-212. doi: 10.1159/000546323.'
    supporting_text: '2026;187(2):199-212. doi: 10.1159/000546323.'
    evidence:
    - reference: PMID:40349694
      reference_title: Potential Key Genes for Giant Cell Arteritis Revealed Based on Single-Cell Sequencing and Mendelian Randomization Analysis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2026;187(2):199-212. doi: 10.1159/000546323.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:40706746
  title: Usefulness of 18F-FDG PET-CT in detecting subclinical arteritis and cancer associated with polymyalgia rheumatica.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026 Jan;93(1):105950. doi: 10.1016/j.jbspin.2025.105950.'
    supporting_text: '2026 Jan;93(1):105950. doi: 10.1016/j.jbspin.2025.105950.'
    evidence:
    - reference: PMID:40706746
      reference_title: Usefulness of 18F-FDG PET-CT in detecting subclinical arteritis and cancer associated with polymyalgia rheumatica.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2026 Jan;93(1):105950. doi: 10.1016/j.jbspin.2025.105950.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:40853447
  title: Cardiac disease in patients with vasculitis.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Cardiac involvement has been described in many forms of vasculitides and is associated with worse outcomes.
    supporting_text: Cardiac involvement has been described in many forms of vasculitides and is associated with worse outcomes.
    evidence:
    - reference: PMID:40853447
      reference_title: Cardiac disease in patients with vasculitis.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Cardiac involvement has been described in many forms of vasculitides and is associated with worse outcomes.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41005512
  title: Long-term results of the neo-aorto-iliac system procedure as a compelling choice for the treatment of aortic infections.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026 Feb;83(2):598-606. doi: 10.1016/j.jvs.2025.09.038.'
    supporting_text: '2026 Feb;83(2):598-606. doi: 10.1016/j.jvs.2025.09.038.'
    evidence:
    - reference: PMID:41005512
      reference_title: Long-term results of the neo-aorto-iliac system procedure as a compelling choice for the treatment of aortic infections.
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2026 Feb;83(2):598-606. doi: 10.1016/j.jvs.2025.09.038.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41198090
  title: 'Syphilitic Cardiac and Vascular Disease: A Comprehensive Review.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2025 Nov 3. doi: 10.1097/CRD.0000000000001115.'
    supporting_text: '2025 Nov 3. doi: 10.1097/CRD.0000000000001115.'
    evidence:
    - reference: PMID:41198090
      reference_title: 'Syphilitic Cardiac and Vascular Disease: A Comprehensive Review.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2025 Nov 3. doi: 10.1097/CRD.0000000000001115.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41218409
  title: 'Tocilizumab monotherapy versus combined in aortitis associated with giant cell arteritis: Factors associated with imaging remission in a multicenter open-label study of 196 patients.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Aortitis is a frequent and potentially serious complication of giant cell arteritis (GCA).
    supporting_text: Aortitis is a frequent and potentially serious complication of giant cell arteritis (GCA).
    evidence:
    - reference: PMID:41218409
      reference_title: 'Tocilizumab monotherapy versus combined in aortitis associated with giant cell arteritis: Factors associated with imaging remission in a multicenter open-label study of 196 patients.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Aortitis is a frequent and potentially serious complication of giant cell arteritis (GCA).
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41365838
  title: 'The influence of baseline aortitis on aortic dilation risk in GCA: a multicentre imaging study.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026 Mar 5;65(3):keaf656. doi: 10.1093/rheumatology/keaf656.'
    supporting_text: '2026 Mar 5;65(3):keaf656. doi: 10.1093/rheumatology/keaf656.'
    evidence:
    - reference: PMID:41365838
      reference_title: 'The influence of baseline aortitis on aortic dilation risk in GCA: a multicentre imaging study.'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: '2026 Mar 5;65(3):keaf656. doi: 10.1093/rheumatology/keaf656.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41655516
  title: "From Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events."
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization.
    supporting_text: Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization.
    evidence:
    - reference: PMID:41655516
      reference_title: "From Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events."
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization.
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41852766
  title: 'Aortic malignancy masquerading as infrarenal aortitis: A diagnostic challenge.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026 Feb 13;12(3):102186. doi: 10.1016/j.jvscit.2026.102186. eCollection 2026 Jun.'
    supporting_text: '2026 Feb 13;12(3):102186. doi: 10.1016/j.jvscit.2026.102186. eCollection 2026 Jun.'
    evidence:
    - reference: PMID:41852766
      reference_title: 'Aortic malignancy masquerading as infrarenal aortitis: A diagnostic challenge.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2026 Feb 13;12(3):102186. doi: 10.1016/j.jvscit.2026.102186. eCollection 2026 Jun.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41907597
  title: 'Case Report: From metabolic normalization to incidental type A aortic dissection in immune checkpoint inhibitor-associated aortitis.'
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026 Mar 13;16:1755873. doi: 10.3389/fonc.2026.1755873. eCollection 2026.'
    supporting_text: '2026 Mar 13;16:1755873. doi: 10.3389/fonc.2026.1755873. eCollection 2026.'
    evidence:
    - reference: PMID:41907597
      reference_title: 'Case Report: From metabolic normalization to incidental type A aortic dissection in immune checkpoint inhibitor-associated aortitis.'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2026 Mar 13;16:1755873. doi: 10.3389/fonc.2026.1755873. eCollection 2026.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:41939570
  title: "Human Immunodeficiency Virus-Associated Vasculitis: A Case Report of Sensorineural Hearing Loss, Bell's Palsy, and Psoriasis Guttata."
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '2026 Mar 5;18(3):e104705. doi: 10.7759/cureus.104705. eCollection 2026 Mar.'
    supporting_text: '2026 Mar 5;18(3):e104705. doi: 10.7759/cureus.104705. eCollection 2026 Mar.'
    evidence:
    - reference: PMID:41939570
      reference_title: "Human Immunodeficiency Virus-Associated Vasculitis: A Case Report of Sensorineural Hearing Loss, Bell's Palsy, and Psoriasis Guttata."
      supports: SUPPORT
      evidence_source: OTHER
      snippet: '2026 Mar 5;18(3):e104705. doi: 10.7759/cureus.104705. eCollection 2026 Mar.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
- reference: PMID:9185506
  title: Glucocorticoid-mediated repression of cytokine gene transcription in human arteritis-SCID chimeras.
  found_in:
  - Aortitis-deep-research-openscientist.md
  findings:
  - statement: '1997 Jun 15;99(12):2842-50. doi: 10.1172/JCI119477.'
    supporting_text: '1997 Jun 15;99(12):2842-50. doi: 10.1172/JCI119477.'
    evidence:
    - reference: PMID:9185506
      reference_title: Glucocorticoid-mediated repression of cytokine gene transcription in human arteritis-SCID chimeras.
      supports: SUPPORT
      evidence_source: MODEL_ORGANISM
      snippet: '1997 Jun 15;99(12):2842-50. doi: 10.1172/JCI119477.'
      explanation: Deep research cited this publication as relevant literature for Aortitis.
datasets: []
📚

References & Deep Research

References

71
DOI:10.3390/jcdd11120405
Aortitis Increases the Risk of Surgical Complications and Re-Operations After Major Aortic Surgery
1 finding
Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections.
"Aortitis, defined as inflammation of the aorta, can lead to aneurysms and dissections."
DOI:10.3389/fphar.2024.1487501
Literature review analysis of aortitis induced by granulocyte-colony stimulating factor
1 finding
Recombinant human granulocyte-colony stimulating factors (G-CSF)-induced aortitis is a rare but particularly serious adverse event, commonly seen in cancer patients undergoing chemotherapy.
"Recombinant human granulocyte-colony stimulating factors (G-CSF)-induced aortitis is a rare but particularly serious adverse event, commonly seen in cancer patients undergoing chemotherapy."
DOI:10.3389/fimmu.2025.1625456
Unraveling the complexity of IgG4-related aortitis and periarteritis: from pathogenesis to clinical practice
1 finding
IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs.
"IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs."
DOI:10.1186/s13063-024-07905-4
The Meteoritics Trial: efficacy of methotrexate after remission-induction with tocilizumab and glucocorticoids in giant cell arteritis—study protocol for a randomized, double-blind, placebo-controlled, parallel-group phase II study
1 finding
Glucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates.
"Glucocorticoids (GC) are the standard treatment for giant cell arteritis (GCA), even though they are associated with adverse side effects and high relapse rates."
DOI:10.1093/cid/ciac560
Retrospective Multicenter Study Comparing Infectious and Noninfectious Aortitis
1 finding
Infectious aortitis had higher aneurysm association and lower survival than noninfectious aortitis.
"IA was more frequently associated with aortic aneurysms compared with NIA (78.8% vs 17.6%, P &lt; .001), especially located in the abdominal aorta (69.7% vs 23.1%, P &lt; .001)."
DOI:10.1136/rmdopen-2023-003379
Imaging in diagnosis, monitoring and outcome prediction of large vessel vasculitis: a systematic literature review and meta-analysis informing the 2023 update of the EULAR recommendations
1 finding
To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV.
"To update the evidence on imaging for diagnosis, monitoring and outcome prediction in large vessel vasculitis (LVV) to inform the 2023 update of the European Alliance of Associations for Rheumatology recommendations on imaging in LVV."
DOI:10.3390/jcm13216364
Imaging in Large Vessel Vasculitis—A Narrative Review
1 finding
Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems.
"Vasculitis refers to a group of rare conditions characterized by the inflammation of blood vessels, affecting multiple systems."
DOI:10.3390/sci7010012
Tocilizumab in Extracranial Giant-Cell Arteritis and Takayasu Arteritis: A Multicentric Observational Comparative Study
1 finding
Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK).
"Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK)."
DOI:10.62186/001c.146456
Stroke, Aortic Regurgitation and Aortic Aneurysm in Younger Female: Case of Giant Cell Aortitis and Discussion
1 finding
The author presents the case of a middle-aged woman who had a left parietal lobe infarct.
"The author presents the case of a middle-aged woman who had a left parietal lobe infarct."
DOI:10.1177/000331970005100705
Accumulation of Lymphocytes, Dendritic Cells, and Granulocytes in the Aortic Wall Affected by Takayasu's Disease
1 finding
Aortic wall tissue in Takayasu arteritis contains inflammatory infiltrates and multiple immune cell populations.
"All specimens showed distinctive histologic features of Takayasu's arteritis and contained inflammatory infiltrates"
PMID:40272133
Aortitis triggered by granulocyte-colony stimulating factor.
1 finding
G-CSF-associated aortitis should be considered in patients with fever after G-CSF treatment.
"The diagnosis of G-CSF-induced aortitis should be considered in patients with fever after G-CSF treatment, particularly if adequate antibiotic treatment does not lead to improvement."
PMID:34448091
Drug-induced aortitis of the subclavian artery caused by pegfilgrastim: a case report.
1 finding
Persistent high fever and CRP can support evaluation for pegfilgrastim-induced aortitis.
"She was hospitalized on day 15 when CRP increased to 21.5 mg/dL and the high fever continued."
PMID:36843728
From Temporal Cell Arteritis to Giant Cell Aortitis Presenting as a Constitutional Syndrome: A Case Report.
1 finding
Giant cell aortitis can present with constitutional symptoms and elevated ESR/CRP.
"The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were elevated, and he also had inflammatory anemia with a hemoglobin of 11.7 g/L."
DOI:10.1016/j.berh.2023.101856
Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow
1 finding
Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow
"Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow"
DOI:10.1055/a-2765-8610
Thoracic Aortic Aneurysm and Giant Cell Arteritis: Clarifying the Link
1 finding
We aim to better define the association between thoracic aortic aneurysm (TAA) and giant cell arteritis (GCA), thereby enhancing cross-diagnosis, monitoring, and therapy.
"We aim to better define the association between thoracic aortic aneurysm (TAA) and giant cell arteritis (GCA), thereby enhancing cross-diagnosis, monitoring, and therapy."
Show evidence (1 reference)
DOI:10.1055/a-2765-8610 SUPPORT Human Clinical
"We aim to better define the association between thoracic aortic aneurysm (TAA) and giant cell arteritis (GCA), thereby enhancing cross-diagnosis, monitoring, and therapy."
Deep research cited this publication as relevant literature for Aortitis.
DOI:10.1161/circgen.118.002296
Single-Nucleotide Polymorphism of the <i>MLX</i> Gene Is Associated With Takayasu Arteritis
1 finding
Takayasu arteritis (TAK) is an autoimmune systemic arteritis of unknown pathogenesis.
"Takayasu arteritis (TAK) is an autoimmune systemic arteritis of unknown pathogenesis."
Show evidence (1 reference)
DOI:10.1161/circgen.118.002296 SUPPORT Human Clinical
"Takayasu arteritis (TAK) is an autoimmune systemic arteritis of unknown pathogenesis."
Deep research cited this publication as relevant literature for Aortitis.
DOI:10.31138/mjr.220125.era
The Great Vasculitis Pretenders: Mycotic Pseudoaneurysm, Aortitis with Occlusive Iliac Thrombus, and Paraneoplastic Aortitis. A Case-Based Review
1 finding
The Great Vasculitis Pretenders: Mycotic Pseudoaneurysm, Aortitis with Occlusive Iliac Thrombus, and Paraneoplastic Aortitis. A Case-Based Review
"The Great Vasculitis Pretenders: Mycotic Pseudoaneurysm, Aortitis with Occlusive Iliac Thrombus, and Paraneoplastic Aortitis. A Case-Based Review"
DOI:10.3389/fmed.2023.1103752
18-Fluorodeoxyglucose positron emission tomography/computed tomography for large vessel vasculitis in clinical practice
1 finding
Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging.
"Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging."
Show evidence (1 reference)
DOI:10.3389/fmed.2023.1103752 SUPPORT Human Clinical
"Diagnosis, prognostic assessment, and monitoring disease activity in patients with large vessel vasculitis (LVV) can be challenging."
Deep research cited this publication as relevant literature for Aortitis.
PMID:15675134
Predictors of left ventricular dysfunction in patients with Takayasu's or giant cell aortitis.
1 finding
LVSD in patients with TA/GCA has been described in case reports and attributed variously to hemodynamic and immunologic factors.
"LVSD in patients with TA/GCA has been described in case reports and attributed variously to hemodynamic and immunologic factors."
Show evidence (1 reference)
PMID:15675134 SUPPORT Human Clinical
"LVSD in patients with TA/GCA has been described in case reports and attributed variously to hemodynamic and immunologic factors."
Deep research cited this publication as relevant literature for Aortitis.
PMID:19150884
Toll-like receptors 4 and 5 induce distinct types of vasculitis.
1 finding
2009 Feb 27;104(4):488-95. doi: 10.1161/CIRCRESAHA.108.185777.
"2009 Feb 27;104(4):488-95. doi: 10.1161/CIRCRESAHA.108.185777."
Show evidence (1 reference)
PMID:19150884 SUPPORT Model Organism
"2009 Feb 27;104(4):488-95. doi: 10.1161/CIRCRESAHA.108.185777."
Deep research cited this publication as relevant literature for Aortitis.
PMID:21124083
IgG4-related inflammatory abdominal aortic aneurysm.
1 finding
2011 Jan;23(1):18-23. doi: 10.1097/BOR.0b013e32833ee95f.
"2011 Jan;23(1):18-23. doi: 10.1097/BOR.0b013e32833ee95f."
Show evidence (1 reference)
PMID:21124083 SUPPORT Human Clinical
"2011 Jan;23(1):18-23. doi: 10.1097/BOR.0b013e32833ee95f."
Deep research cited this publication as relevant literature for Aortitis.
PMID:21220737
Blocking the NOTCH pathway inhibits vascular inflammation in large-vessel vasculitis.
1 finding
Giant cell arteritis is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke, and aortic aneurysm.
"Giant cell arteritis is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke, and aortic aneurysm."
Show evidence (1 reference)
PMID:21220737 SUPPORT Model Organism
"Giant cell arteritis is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke, and aortic aneurysm."
Deep research cited this publication as relevant literature for Aortitis.
PMID:23843109
Genetic component of giant cell arteritis.
1 finding
2014 Jan;53(1):6-18. doi: 10.1093/rheumatology/ket231.
"2014 Jan;53(1):6-18. doi: 10.1093/rheumatology/ket231."
Show evidence (1 reference)
PMID:23843109 SUPPORT Human Clinical
"2014 Jan;53(1):6-18. doi: 10.1093/rheumatology/ket231."
Deep research cited this publication as relevant literature for Aortitis.
PMID:25658487
Serpin treatment suppresses inflammatory vascular lesions in temporal artery implants (TAI) from patients with giant cell arteritis.
1 finding
2015 Feb 6;10(2):e0115482. doi: 10.1371/journal.pone.0115482. eCollection 2015.
"2015 Feb 6;10(2):e0115482. doi: 10.1371/journal.pone.0115482. eCollection 2015."
Show evidence (1 reference)
PMID:25658487 SUPPORT Model Organism
"2015 Feb 6;10(2):e0115482. doi: 10.1371/journal.pone.0115482. eCollection 2015."
Deep research cited this publication as relevant literature for Aortitis.
PMID:25931203
Takayasu arteritis and ulcerative colitis: high rate of co-occurrence and genetic overlap.
1 finding
2015 May;67(8):2226-32. doi: 10.1002/art.39157.
"2015 May;67(8):2226-32. doi: 10.1002/art.39157."
Show evidence (1 reference)
PMID:25931203 SUPPORT Human Clinical
"2015 May;67(8):2226-32. doi: 10.1002/art.39157."
Deep research cited this publication as relevant literature for Aortitis.
PMID:26051917
Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases.
1 finding
2015 Sep-Oct;24(5):267-78. doi: 10.1016/j.carpath.2015.05.001.
"2015 Sep-Oct;24(5):267-78. doi: 10.1016/j.carpath.2015.05.001."
Show evidence (1 reference)
PMID:26051917 SUPPORT Other
"2015 Sep-Oct;24(5):267-78. doi: 10.1016/j.carpath.2015.05.001."
Deep research cited this publication as relevant literature for Aortitis.
PMID:26093659
Epigenetics and Vasculitis: a Comprehensive Review.
1 finding
2016 Jun;50(3):357-66. doi: 10.1007/s12016-015-8495-6.
"2016 Jun;50(3):357-66. doi: 10.1007/s12016-015-8495-6."
Show evidence (1 reference)
PMID:26093659 SUPPORT Other
"2016 Jun;50(3):357-66. doi: 10.1007/s12016-015-8495-6."
Deep research cited this publication as relevant literature for Aortitis.
PMID:26775836
[Infectious aortitis caused by Streptococcus pneumoniae].
1 finding
2016 Feb;41(1):36-41. doi: 10.1016/j.jmv.2015.12.003.
"2016 Feb;41(1):36-41. doi: 10.1016/j.jmv.2015.12.003."
Show evidence (1 reference)
PMID:26775836 SUPPORT Human Clinical
"2016 Feb;41(1):36-41. doi: 10.1016/j.jmv.2015.12.003."
Deep research cited this publication as relevant literature for Aortitis.
PMID:27193038
The Severity of Takayasu Arteritis Is Associated with the HLA-B52 Allele in Japanese Patients.
1 finding
2016 May;239(1):67-72. doi: 10.1620/tjem.239.67.
"2016 May;239(1):67-72. doi: 10.1620/tjem.239.67."
Show evidence (1 reference)
PMID:27193038 SUPPORT Human Clinical
"2016 May;239(1):67-72. doi: 10.1620/tjem.239.67."
Deep research cited this publication as relevant literature for Aortitis.
PMID:27224742
Varicella zoster virus triggers the immunopathology of giant cell arteritis.
1 finding
2016 Jul;28(4):376-82. doi: 10.1097/BOR.0000000000000292.
"2016 Jul;28(4):376-82. doi: 10.1097/BOR.0000000000000292."
Show evidence (1 reference)
PMID:27224742 SUPPORT Other
"2016 Jul;28(4):376-82. doi: 10.1097/BOR.0000000000000292."
Deep research cited this publication as relevant literature for Aortitis.
PMID:27815653
Relationship of HLA-B*51 and HLA-B*52 alleles and TNF-α-308A/G polymorphism with susceptibility to Takayasu arteritis: a meta-analysis.
1 finding
2017 Jan;36(1):173-181. doi: 10.1007/s10067-016-3445-0.
"2017 Jan;36(1):173-181. doi: 10.1007/s10067-016-3445-0."
Show evidence (1 reference)
PMID:27815653 SUPPORT Other
"2017 Jan;36(1):173-181. doi: 10.1007/s10067-016-3445-0."
Deep research cited this publication as relevant literature for Aortitis.
PMID:28277489
Analysis of the common genetic component of large-vessel vasculitides through a meta-Immunochip strategy.
1 finding
2017 Mar 9;7:43953. doi: 10.1038/srep43953.
"2017 Mar 9;7:43953. doi: 10.1038/srep43953."
Show evidence (1 reference)
PMID:28277489 SUPPORT Other
"2017 Mar 9;7:43953. doi: 10.1038/srep43953."
Deep research cited this publication as relevant literature for Aortitis.
PMID:28457683
[Epidemiology and natural history of giant cell arteritis].
1 finding
2017 Oct;38(10):663-669. doi: 10.1016/j.revmed.2017.03.007.
"2017 Oct;38(10):663-669. doi: 10.1016/j.revmed.2017.03.007."
Show evidence (1 reference)
PMID:28457683 SUPPORT Human Clinical
"2017 Oct;38(10):663-669. doi: 10.1016/j.revmed.2017.03.007."
Deep research cited this publication as relevant literature for Aortitis.
PMID:28756072
Epidemiology of Takayasu arteritis.
1 finding
2017 Jul-Aug;46(7-8 Pt 2):e197-e203. doi: 10.1016/j.lpm.2017.05.034.
"2017 Jul-Aug;46(7-8 Pt 2):e197-e203. doi: 10.1016/j.lpm.2017.05.034."
Show evidence (1 reference)
PMID:28756072 SUPPORT Human Clinical
"2017 Jul-Aug;46(7-8 Pt 2):e197-e203. doi: 10.1016/j.lpm.2017.05.034."
Deep research cited this publication as relevant literature for Aortitis.
PMID:28957963
An update on the role of epigenetics in systemic vasculitis.
1 finding
2018 Jan;30(1):4-15. doi: 10.1097/BOR.0000000000000451.
"2018 Jan;30(1):4-15. doi: 10.1097/BOR.0000000000000451."
Show evidence (1 reference)
PMID:28957963 SUPPORT Other
"2018 Jan;30(1):4-15. doi: 10.1097/BOR.0000000000000451."
Deep research cited this publication as relevant literature for Aortitis.
PMID:29105322
Clinical characteristics and outcomes of 61 patients with chronic periaortitis including IgG4-related and non-IgG4-related cases.
1 finding
2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194.
"2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194."
Show evidence (1 reference)
PMID:29105322 SUPPORT Human Clinical
"2017 Nov;20(11):1751-1762. doi: 10.1111/1756-185X.13194."
Deep research cited this publication as relevant literature for Aortitis.
PMID:29254929
Inhibition of JAK-STAT Signaling Suppresses Pathogenic Immune Responses in Medium and Large Vessel Vasculitis.
1 finding
Giant cell arteritis, a chronic autoimmune disease of the aorta and its large branches, is complicated by aneurysm formation, dissection, and arterial occlusions.
"Giant cell arteritis, a chronic autoimmune disease of the aorta and its large branches, is complicated by aneurysm formation, dissection, and arterial occlusions."
Show evidence (1 reference)
PMID:29254929 SUPPORT Model Organism
"Giant cell arteritis, a chronic autoimmune disease of the aorta and its large branches, is complicated by aneurysm formation, dissection, and arterial occlusions."
Deep research cited this publication as relevant literature for Aortitis.
PMID:30498034
Genetic determinants and an epistasis of LILRA3 and HLA-B*52 in Takayasu arteritis.
1 finding
2018 Dec 18;115(51):13045-13050. doi: 10.1073/pnas.1808850115.
"2018 Dec 18;115(51):13045-13050. doi: 10.1073/pnas.1808850115."
Show evidence (1 reference)
PMID:30498034 SUPPORT Other
"2018 Dec 18;115(51):13045-13050. doi: 10.1073/pnas.1808850115."
Deep research cited this publication as relevant literature for Aortitis.
PMID:30805622
Markers of angiogenesis and macrophage products for predicting disease course and monitoring vascular inflammation in giant cell arteritis.
1 finding
2019 Feb 25;58(8):1383-92. doi: 10.1093/rheumatology/kez034.
"2019 Feb 25;58(8):1383-92. doi: 10.1093/rheumatology/kez034."
Show evidence (1 reference)
PMID:30805622 SUPPORT Other
"2019 Feb 25;58(8):1383-92. doi: 10.1093/rheumatology/kez034."
Deep research cited this publication as relevant literature for Aortitis.
PMID:30993253
Microbiomes of Inflammatory Thoracic Aortic Aneurysms Due to Giant Cell Arteritis and Clinically Isolated Aortitis Differ From Those of Non-Inflammatory Aneurysms.
1 finding
2019 Mar 15;4(1):105-123. doi: 10.20411/pai.v4i1.269. eCollection 2019.
"2019 Mar 15;4(1):105-123. doi: 10.20411/pai.v4i1.269. eCollection 2019."
Show evidence (1 reference)
PMID:30993253 SUPPORT Other
"2019 Mar 15;4(1):105-123. doi: 10.20411/pai.v4i1.269. eCollection 2019."
Deep research cited this publication as relevant literature for Aortitis.
PMID:31270110
2018 Update of the EULAR recommendations for the management of large vessel vasculitis.
1 finding
2018 Update of the EULAR recommendations for the management of large vessel vasculitis
"Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore..."
Show evidence (1 reference)
PMID:31270110 SUPPORT Other
"Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore..."
Deep research cited this publication as relevant literature for Aortitis.
PMID:32234379
Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases.
1 finding
2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018.
"2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018."
Show evidence (1 reference)
PMID:32234379 SUPPORT Human Clinical
"2020 Apr;49(1):104018. doi: 10.1016/j.lpm.2020.104018."
Deep research cited this publication as relevant literature for Aortitis.
PMID:33128155
Clinically isolated aortitis: imaging features and clinical outcomes: comparison with giant cell arteritis and giant cell aortitis.
1 finding
2021 Apr;37(4):1433-1443. doi: 10.1007/s10554-020-02087-x.
"2021 Apr;37(4):1433-1443. doi: 10.1007/s10554-020-02087-x."
Show evidence (1 reference)
PMID:33128155 SUPPORT Human Clinical
"2021 Apr;37(4):1433-1443. doi: 10.1007/s10554-020-02087-x."
Deep research cited this publication as relevant literature for Aortitis.
PMID:33593995
Aortitis: recent advances, current concepts and future possibilities.
1 finding
2021 Oct;107(20):1620-1629. doi: 10.1136/heartjnl-2020-318085.
"2021 Oct;107(20):1620-1629. doi: 10.1136/heartjnl-2020-318085."
Show evidence (1 reference)
PMID:33593995 SUPPORT Other
"2021 Oct;107(20):1620-1629. doi: 10.1136/heartjnl-2020-318085."
Deep research cited this publication as relevant literature for Aortitis.
PMID:33734973
The presence of both HLA-DRB1*04:01 and HLA-B*15:01 increases the susceptibility to cranial and extracranial giant cell arteritis.
1 finding
2021 Mar-Apr;39 Suppl 129(2):21-26. doi: 10.55563/clinexprheumatol/nn15lt.
"2021 Mar-Apr;39 Suppl 129(2):21-26. doi: 10.55563/clinexprheumatol/nn15lt."
Show evidence (1 reference)
PMID:33734973 SUPPORT Human Clinical
"2021 Mar-Apr;39 Suppl 129(2):21-26. doi: 10.55563/clinexprheumatol/nn15lt."
Deep research cited this publication as relevant literature for Aortitis.
PMID:36277471
A Case Report of Immune Checkpoint Inhibitor-Induced Aortitis Treated with Tocilizumab.
1 finding
2022 Oct 12;2022:7971169. doi: 10.1155/2022/7971169. eCollection 2022.
"2022 Oct 12;2022:7971169. doi: 10.1155/2022/7971169. eCollection 2022."
Show evidence (1 reference)
PMID:36277471 SUPPORT Other
"2022 Oct 12;2022:7971169. doi: 10.1155/2022/7971169. eCollection 2022."
Deep research cited this publication as relevant literature for Aortitis.
PMID:36912345
Cranial and extracranial giant cell arteritis do not exhibit differences in the IL6 -174 G/C gene polymorphism.
1 finding
2023 Apr;41(4):910-915. doi: 10.55563/clinexprheumatol/cbjnmo.
"2023 Apr;41(4):910-915. doi: 10.55563/clinexprheumatol/cbjnmo."
Show evidence (1 reference)
PMID:36912345 SUPPORT Human Clinical
"2023 Apr;41(4):910-915. doi: 10.55563/clinexprheumatol/cbjnmo."
Deep research cited this publication as relevant literature for Aortitis.
PMID:37256147
Arterial wall fibrosis in Takayasu arteritis and its potential for therapeutic modulation.
1 finding
2023 May 15;14:1174249. doi: 10.3389/fimmu.2023.1174249. eCollection 2023.
"2023 May 15;14:1174249. doi: 10.3389/fimmu.2023.1174249. eCollection 2023."
Show evidence (1 reference)
PMID:37256147 SUPPORT Other
"2023 May 15;14:1174249. doi: 10.3389/fimmu.2023.1174249. eCollection 2023."
Deep research cited this publication as relevant literature for Aortitis.
PMID:37279826
An unusual presentation of invasive Fusarium aortitis in a patient who is immunocompromised: A case report.
1 finding
2023 Sep;134:102-105. doi: 10.1016/j.ijid.2023.05.069.
"2023 Sep;134:102-105. doi: 10.1016/j.ijid.2023.05.069."
Show evidence (1 reference)
PMID:37279826 SUPPORT Other
"2023 Sep;134:102-105. doi: 10.1016/j.ijid.2023.05.069."
Deep research cited this publication as relevant literature for Aortitis.
PMID:37304255
Clinical experience and safety of Janus kinase inhibitors in giant cell arteritis: a retrospective case series from Sweden.
1 finding
2023 May 25;14:1187584. doi: 10.3389/fimmu.2023.1187584. eCollection 2023.
"2023 May 25;14:1187584. doi: 10.3389/fimmu.2023.1187584. eCollection 2023."
Show evidence (1 reference)
PMID:37304255 SUPPORT Other
"2023 May 25;14:1187584. doi: 10.3389/fimmu.2023.1187584. eCollection 2023."
Deep research cited this publication as relevant literature for Aortitis.
PMID:37673506
Long-Term Outcome and Prognosis of Noninfectious Thoracic Aortitis.
1 finding
Aortitis is a group of disorders characterized by the inflammation of the aorta.
"Aortitis is a group of disorders characterized by the inflammation of the aorta."
Show evidence (1 reference)
PMID:37673506 SUPPORT Other
"Aortitis is a group of disorders characterized by the inflammation of the aorta."
Deep research cited this publication as relevant literature for Aortitis.
PMID:37944298
Beyond the symptoms: Personalizing giant cell arteritis care through multidimensional patient reported outcome measure.
1 finding
Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50.
"Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50."
Show evidence (1 reference)
PMID:37944298 SUPPORT Other
"Giant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50."
Deep research cited this publication as relevant literature for Aortitis.
PMID:38521841
Aortitis after switching short-acting granulocyte colony-stimulating factors in a lymphoma patient with HLA-B52.
1 finding
2024 May;119(5):608-612. doi: 10.1007/s12185-024-03744-w.
"2024 May;119(5):608-612. doi: 10.1007/s12185-024-03744-w."
Show evidence (1 reference)
PMID:38521841 SUPPORT Human Clinical
"2024 May;119(5):608-612. doi: 10.1007/s12185-024-03744-w."
Deep research cited this publication as relevant literature for Aortitis.
PMID:38734017
Risk loci involved in giant cell arteritis susceptibility: a genome-wide association study.
1 finding
Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older.
"Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older."
Show evidence (1 reference)
PMID:38734017 SUPPORT Other
"Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older."
Deep research cited this publication as relevant literature for Aortitis.
PMID:39714261
Immune checkpoint inhibitors-associated vasculitis: a heterogeneous condition with possible severe disease course.
1 finding
2025 Jun 1;64(6):3685-3690. doi: 10.1093/rheumatology/keae711.
"2025 Jun 1;64(6):3685-3690. doi: 10.1093/rheumatology/keae711."
Show evidence (1 reference)
PMID:39714261 SUPPORT Human Clinical
"2025 Jun 1;64(6):3685-3690. doi: 10.1093/rheumatology/keae711."
Deep research cited this publication as relevant literature for Aortitis.
PMID:39826312
Histological pattern of non-infectious thoracic aortitis impacts mortality.
1 finding
Non-infectious aortitis encompasses various histological patterns, but their specific cardiovascular outcomes remain unclear.
"Non-infectious aortitis encompasses various histological patterns, but their specific cardiovascular outcomes remain unclear."
Show evidence (1 reference)
PMID:39826312 SUPPORT Human Clinical
"Non-infectious aortitis encompasses various histological patterns, but their specific cardiovascular outcomes remain unclear."
Deep research cited this publication as relevant literature for Aortitis.
PMID:40021438
Aortic disease in giant cell arteritis.
1 finding
2025 Jun;72S:152677. doi: 10.1016/j.semarthrit.2025.152677.
"2025 Jun;72S:152677. doi: 10.1016/j.semarthrit.2025.152677."
Show evidence (1 reference)
PMID:40021438 SUPPORT Other
"2025 Jun;72S:152677. doi: 10.1016/j.semarthrit.2025.152677."
Deep research cited this publication as relevant literature for Aortitis.
PMID:40099651
Isolated aortitis - is it truly isolated? An approach to diagnosis and management.
1 finding
2025 May 1;37(3):185-191. doi: 10.1097/BOR.0000000000001084.
"2025 May 1;37(3):185-191. doi: 10.1097/BOR.0000000000001084."
Show evidence (1 reference)
PMID:40099651 SUPPORT Human Clinical
"2025 May 1;37(3):185-191. doi: 10.1097/BOR.0000000000001084."
Deep research cited this publication as relevant literature for Aortitis.
PMID:40174237
A Phase 3 Trial of Upadacitinib for Giant-Cell Arteritis.
1 finding
Giant-cell arteritis is a systemic vasculitis with limited treatment options.
"Giant-cell arteritis is a systemic vasculitis with limited treatment options."
Show evidence (1 reference)
PMID:40174237 SUPPORT Human Clinical
"Giant-cell arteritis is a systemic vasculitis with limited treatment options."
Deep research cited this publication as relevant literature for Aortitis.
PMID:40349694
Potential Key Genes for Giant Cell Arteritis Revealed Based on Single-Cell Sequencing and Mendelian Randomization Analysis.
1 finding
2026;187(2):199-212. doi: 10.1159/000546323.
"2026;187(2):199-212. doi: 10.1159/000546323."
Show evidence (1 reference)
PMID:40349694 SUPPORT Other
"2026;187(2):199-212. doi: 10.1159/000546323."
Deep research cited this publication as relevant literature for Aortitis.
PMID:40706746
Usefulness of 18F-FDG PET-CT in detecting subclinical arteritis and cancer associated with polymyalgia rheumatica.
1 finding
2026 Jan;93(1):105950. doi: 10.1016/j.jbspin.2025.105950.
"2026 Jan;93(1):105950. doi: 10.1016/j.jbspin.2025.105950."
Show evidence (1 reference)
PMID:40706746 SUPPORT Other
"2026 Jan;93(1):105950. doi: 10.1016/j.jbspin.2025.105950."
Deep research cited this publication as relevant literature for Aortitis.
PMID:40853447
Cardiac disease in patients with vasculitis.
1 finding
Cardiac involvement has been described in many forms of vasculitides and is associated with worse outcomes.
"Cardiac involvement has been described in many forms of vasculitides and is associated with worse outcomes."
Show evidence (1 reference)
PMID:40853447 SUPPORT Other
"Cardiac involvement has been described in many forms of vasculitides and is associated with worse outcomes."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41005512
Long-term results of the neo-aorto-iliac system procedure as a compelling choice for the treatment of aortic infections.
1 finding
2026 Feb;83(2):598-606. doi: 10.1016/j.jvs.2025.09.038.
"2026 Feb;83(2):598-606. doi: 10.1016/j.jvs.2025.09.038."
Show evidence (1 reference)
PMID:41005512 SUPPORT Other
"2026 Feb;83(2):598-606. doi: 10.1016/j.jvs.2025.09.038."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41198090
Syphilitic Cardiac and Vascular Disease: A Comprehensive Review.
1 finding
2025 Nov 3. doi: 10.1097/CRD.0000000000001115.
"2025 Nov 3. doi: 10.1097/CRD.0000000000001115."
Show evidence (1 reference)
PMID:41198090 SUPPORT Other
"2025 Nov 3. doi: 10.1097/CRD.0000000000001115."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41218409
Tocilizumab monotherapy versus combined in aortitis associated with giant cell arteritis: Factors associated with imaging remission in a multicenter open-label study of 196 patients.
1 finding
Aortitis is a frequent and potentially serious complication of giant cell arteritis (GCA).
"Aortitis is a frequent and potentially serious complication of giant cell arteritis (GCA)."
Show evidence (1 reference)
PMID:41218409 SUPPORT Other
"Aortitis is a frequent and potentially serious complication of giant cell arteritis (GCA)."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41365838
The influence of baseline aortitis on aortic dilation risk in GCA: a multicentre imaging study.
1 finding
2026 Mar 5;65(3):keaf656. doi: 10.1093/rheumatology/keaf656.
"2026 Mar 5;65(3):keaf656. doi: 10.1093/rheumatology/keaf656."
Show evidence (1 reference)
PMID:41365838 SUPPORT Human Clinical
"2026 Mar 5;65(3):keaf656. doi: 10.1093/rheumatology/keaf656."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41655516
From Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events.
1 finding
Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization.
"Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization."
Show evidence (1 reference)
PMID:41655516 SUPPORT Other
"Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41852766
Aortic malignancy masquerading as infrarenal aortitis: A diagnostic challenge.
1 finding
2026 Feb 13;12(3):102186. doi: 10.1016/j.jvscit.2026.102186. eCollection 2026 Jun.
"2026 Feb 13;12(3):102186. doi: 10.1016/j.jvscit.2026.102186. eCollection 2026 Jun."
Show evidence (1 reference)
PMID:41852766 SUPPORT Other
"2026 Feb 13;12(3):102186. doi: 10.1016/j.jvscit.2026.102186. eCollection 2026 Jun."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41907597
Case Report: From metabolic normalization to incidental type A aortic dissection in immune checkpoint inhibitor-associated aortitis.
1 finding
2026 Mar 13;16:1755873. doi: 10.3389/fonc.2026.1755873. eCollection 2026.
"2026 Mar 13;16:1755873. doi: 10.3389/fonc.2026.1755873. eCollection 2026."
Show evidence (1 reference)
PMID:41907597 SUPPORT Other
"2026 Mar 13;16:1755873. doi: 10.3389/fonc.2026.1755873. eCollection 2026."
Deep research cited this publication as relevant literature for Aortitis.
PMID:41939570
Human Immunodeficiency Virus-Associated Vasculitis: A Case Report of Sensorineural Hearing Loss, Bell's Palsy, and Psoriasis Guttata.
1 finding
2026 Mar 5;18(3):e104705. doi: 10.7759/cureus.104705. eCollection 2026 Mar.
"2026 Mar 5;18(3):e104705. doi: 10.7759/cureus.104705. eCollection 2026 Mar."
Show evidence (1 reference)
PMID:41939570 SUPPORT Other
"2026 Mar 5;18(3):e104705. doi: 10.7759/cureus.104705. eCollection 2026 Mar."
Deep research cited this publication as relevant literature for Aortitis.
PMID:9185506
Glucocorticoid-mediated repression of cytokine gene transcription in human arteritis-SCID chimeras.
1 finding
1997 Jun 15;99(12):2842-50. doi: 10.1172/JCI119477.
"1997 Jun 15;99(12):2842-50. doi: 10.1172/JCI119477."
Show evidence (1 reference)
PMID:9185506 SUPPORT Model Organism
"1997 Jun 15;99(12):2842-50. doi: 10.1172/JCI119477."
Deep research cited this publication as relevant literature for Aortitis.

Deep Research

2
Falcon ▸
1. Disease Information
Edison Scientific Literature 45 citations 2026-05-05T23:44:14.822781

1. Disease Information

1.1 Overview / definition

Aortitis is explicitly defined as “inflammation of the aorta”. (staniforth2024aortitisincreasesthe pages 1-2)

Clinically, it is best conceptualized as a syndrome with divergent causes and management imperatives (e.g., immunosuppression for inflammatory aortitis vs antimicrobials ± surgery for infectious aortitis). (arcilla2025thegreatvasculitis pages 7-9, staniforth2024aortitisincreasesthe pages 1-2)

1.2 Key identifiers (requested: OMIM/Orphanet/ICD/MeSH/MONDO)

  • Not found in retrieved texts: ICD-10/ICD-11 codes, MeSH headings/unique IDs, MONDO IDs, Orphanet IDs. (staniforth2024aortitisincreasesthe pages 9-11, staniforth2024aortitisincreasesthe pages 2-4)
  • Common literature labels/synonyms used in retrieved texts (non-exhaustive):
  • Aortitis; infectious aortitis; noninfectious aortitis; inflammatory aortitis; clinically isolated aortitis (CIA); “idiopathic aortitis”; “giant cell aortitis”. (staniforth2024aortitisincreasesthe pages 1-2, staniforth2024aortitisincreasesthe pages 9-11, staniforth2024aortitisincreasesthe pages 2-4)

1.3 Evidence sources

The information here is derived from aggregated disease-level resources (systematic reviews, narrative reviews, cohort studies) and case-based reviews; several sources are surgical-cohort based (histology from aortic resections) and thus reflect a selected population (patients undergoing major aortic surgery). (staniforth2024aortitisincreasesthe pages 1-2, bosch2023imagingindiagnosis pages 1-2, arcilla2025thegreatvasculitis pages 7-9)

2. Etiology

2.1 Major causal categories

  1. Noninfectious (immune-mediated) aortitis
  2. Large-vessel vasculitis: Giant cell arteritis (GCA), Takayasu arteritis (TAK), and variable-vessel vasculitis such as Behçet disease can involve the aorta/major branches. (popescu2024imaginginlarge pages 4-5)
  3. Clinically isolated aortitis (CIA): defined as aortitis without systemic vasculitis or other inflammatory conditions, often detected on histology after aortic surgery. (staniforth2024aortitisincreasesthe pages 1-2)

  4. IgG4-related periaortitis/aortitis is a distinct fibro-inflammatory entity (review evidence retrieved is 2025). (wang2025unravelingthecomplexity pages 6-7)

  5. Infectious aortitis

  6. Pathogens include Salmonella, Staphylococcus aureus, Streptococcus spp., Enterococcus, Haemophilus influenzae, Mycobacterium tuberculosis, Treponema pallidum, fungi, and others; infection can seed damaged aortic wall (e.g., atherosclerosis) with pseudoaneurysm formation and high rupture risk. (arcilla2025thegreatvasculitis pages 7-9, arcilla2025thegreatvasculitis pages 6-7)

  7. Drug-induced aortitis

  8. G-CSF–induced aortitis has been characterized through systematic case aggregation, particularly in chemotherapy settings. (zhao2024literaturereviewanalysis pages 1-2, zhao2024literaturereviewanalysis pages 5-7)

2.2 Risk factors

  • CIA/noninfectious surgical aortitis (surgical cohort): increased age, female sex, current smoking, and prior inflammatory disease were associated with histologic aortitis; bicuspid aortic valve was associated with reduced odds in that cohort. (staniforth2024aortitisincreasesthe pages 6-8)
  • Infectious aortitis: risk factors include trauma, atherosclerotic plaques, congenital malformations, immunosuppression, infections, and malignancy. (arcilla2025thegreatvasculitis pages 1-3)
  • G-CSF–induced: predominantly older women; common setting is breast cancer chemotherapy; geographic clustering in East Asia reported in the case-review dataset. (zhao2024literaturereviewanalysis pages 1-2, zhao2024literaturereviewanalysis pages 2-3)

2.3 Protective factors

A bicuspid aortic valve was associated with lower likelihood of histologic aortitis in a surgical cohort (association, not necessarily causal protection). (staniforth2024aortitisincreasesthe pages 6-8)

2.4 Gene–environment interactions

Direct gene–environment interaction studies specific to “aortitis” were not retrieved. However, TAK genetic susceptibility (e.g., MLX variant; HLA associations) plausibly interacts with inflammatory triggers; infectious mimics can also induce autoantibodies and confound immune phenotyping. (tamura2018singlenucleotidepolymorphismof pages 6-7, arcilla2025thegreatvasculitis pages 7-9)

3. Phenotypes (with HPO suggestions)

Aortitis phenotypes vary by etiology and vascular territory involved. A curated phenotype-to-HPO mapping table is provided below.

Phenotype (plain) HPO term(s) suggestion Evidence details (including onset/course, frequencies, quantitative lab values) Major etiologies/subtypes where seen Key citations (pqac ids)
Constitutional symptoms / malaise / fatigue / weight loss HP:0012378 Fatigue; HP:0004305 Malaise; HP:0001824 Weight loss Aortitis often presents nonspecifically with fatigue, feeling generally ill, and weight loss; GCA usually has gradual onset over weeks to months; TAK has a prodromal constitutional phase; IgG4-related PAO/PA also includes fatigue/weight loss/malaise; Behçet often relapsing-remitting General aortitis, GCA, TAK, IgG4-related aortitis, Behçet disease (staniforth2024aortitisincreasesthe pages 1-2, popescu2024imaginginlarge pages 4-5, wang2025unravelingthecomplexity pages 6-7)
Fever HP:0001945 Fever Fever is common in GCA and TAK constitutional phases; in G-CSF-induced aortitis, 68/72 (96%) were symptomatic, commonly with fever; mean temperatures around 38.6°C; infectious aortitis also commonly presents with systemic inflammatory illness GCA, TAK, G-CSF-induced aortitis, infectious aortitis (popescu2024imaginginlarge pages 4-5, zhao2024literaturereviewanalysis pages 5-7, arcilla2025thegreatvasculitis pages 7-9)
Pain (chest, back, abdominal, neck, sore throat) HP:0100749 Chest pain; HP:0002027 Abdominal pain; HP:0003418 Back pain; HP:0003202 Cervical pain G-CSF-induced aortitis commonly caused fever plus pain (chest, back, abdominal, neck, sore throat); IgG4-related PAO/PA commonly causes abdominal or back pain; thoracic involvement may cause chest pain and dyspnea G-CSF-induced aortitis, IgG4-related aortitis/periaortitis (zhao2024literaturereviewanalysis pages 5-7, wang2025unravelingthecomplexity pages 6-7)
Polymyalgia-type proximal stiffness HP:0002829 Arthralgia; HP:0003326 Myalgia Noninfectious aortitis may include polymyalgic symptoms with proximal shoulder/pelvic girdle stiffness; polymyalgia rheumatica co-occurs in 40–60% of GCA Noninfectious aortitis, especially GCA-associated aortitis (staniforth2024aortitisincreasesthe pages 1-2, popescu2024imaginginlarge pages 4-5)
Headache HP:0002315 Headache GCA cranial phenotype includes headache; onset usually gradual over weeks to months, though ~20% may be abrupt GCA-associated aortitis/large-vessel GCA (popescu2024imaginginlarge pages 4-5)
Jaw or tongue claudication HP:0200044 Jaw claudication Characteristic cranial ischemic symptom in GCA; occurs with chewing and supports cranial involvement in patients who may also have aortitis/large-vessel disease GCA-associated aortitis (popescu2024imaginginlarge pages 4-5)
Visual loss / ischemic optic neuropathy HP:0000572 Visual loss; HP:0000648 Optic atrophy (suggestive downstream term if chronic) In GCA, vascular occlusion can cause cranial ischemia and ischemic optic neuropathy; vision loss is typically painless and irreversible; ischemic optic neuropathy reported in ~14% in imaging review context GCA-associated aortitis/large-vessel GCA (popescu2024imaginginlarge pages 4-5)
Limb claudication / arm or leg claudication HP:0004936 Intermittent claudication Aortic or main-branch involvement in GCA can cause limb claudication; TAK vascular phase includes arm or leg claudication; IgG4 iliac/femoral disease can also cause claudication GCA, TAK, IgG4-related aortitis (popescu2024imaginginlarge pages 4-5, chan2025strokeaorticregurgitation pages 2-4, wang2025unravelingthecomplexity pages 6-7)
Pulseless vascular phase / pulse deficits HP:0031808 Abnormality of peripheral pulse TAK classically progresses from a constitutional “pre-pulseless” phase to a “pulseless” vascular phase with stenotic lesions in >90% TAK-associated aortitis (popescu2024imaginginlarge pages 4-5)
Hypertension HP:0000822 Hypertension Common in TAK (30–90%); IgG4-related renal artery involvement can also cause hypertension TAK, IgG4-related aortitis (popescu2024imaginginlarge pages 4-5, wang2025unravelingthecomplexity pages 6-7)
Stroke / transient ischemic attack HP:0001297 Stroke; HP:0002326 Transient ischemic attack TIAs/strokes occur in 2–7% of GCA and 10–20% of TAK; aortitis cohorts also report increased stroke risk during follow-up GCA, TAK, general aortitis complications (popescu2024imaginginlarge pages 4-5, staniforth2024aortitisincreasesthe pages 8-9)
Mucocutaneous ulcers HP:0000197 Oral ulcer; HP:0000135 Genital ulcer Behçet disease phenotype includes oral and genital ulcers with frequent relapses/remissions and potential large-vessel aortitis/aneurysm/thrombosis Behçet disease-associated aortitis (popescu2024imaginginlarge pages 4-5)
Uveitis / ocular inflammation HP:0000554 Uveitis Behçet disease includes uveitis as a key systemic phenotype accompanying possible large-vessel complications Behçet disease-associated aortitis (popescu2024imaginginlarge pages 4-5)
Thrombosis / occlusion HP:0004930 Venous thrombosis; HP:0012393 Arterial thrombosis; HP:0004417 Vascular occlusion Behçet large-vessel involvement may include thrombosis and occlusion; aortitis overall can cause stenosis/occlusion; infectious aortitis can present with pseudoaneurysm and occlusive thrombus Behçet disease, infectious aortitis, general aortitis (popescu2024imaginginlarge pages 4-5, staniforth2024aortitisincreasesthe pages 1-2, arcilla2025thegreatvasculitis pages 7-9)
Elevated CRP / ESR / inflammatory markers HP:0011227 Elevated C-reactive protein level; HP:0003565 Increased erythrocyte sedimentation rate Aortitis workup commonly shows high inflammatory markers; G-CSF-induced cases had CRP means ~26.06 ± 15.39 mg/dL (filgrastim), 24.81 ± 9.65 mg/dL (pegfilgrastim), 10.45 ± 9.91 mg/dL (lenograstim); giant cell aortitis case had CRP 82 mg/L and ESR 140 mm/h General aortitis, G-CSF-induced aortitis, giant cell aortitis case, IgG4-related aortitis (staniforth2024aortitisincreasesthe pages 1-2, zhao2024literaturereviewanalysis pages 5-7, chan2025strokeaorticregurgitation pages 2-4, wang2025unravelingthecomplexity pages 6-7)
Leukocytosis HP:0001974 Leukocytosis Reported as a common laboratory abnormality in infectious/inflammatory aortitis differential workup Infectious aortitis and mimics (arcilla2025thegreatvasculitis pages 7-9)
Thrombocytosis HP:0001873 Thrombocytosis Mild-to-moderate thrombocytosis reported in infectious/inflammatory aortitis differential workup Infectious aortitis and mimics (arcilla2025thegreatvasculitis pages 7-9)
Normocytic/normochromic anemia HP:0001877 Abnormality of erythrocytes; HP:0001892 Normocytic anemia Normochromic/normocytic anemia described among laboratory abnormalities in infectious/inflammatory aortitis evaluation Infectious aortitis and mimics (arcilla2025thegreatvasculitis pages 7-9)
Hypoalbuminemia HP:0003073 Hypoalbuminemia Reported among laboratory abnormalities in infectious/inflammatory aortitis differential workup Infectious aortitis and mimics (arcilla2025thegreatvasculitis pages 7-9)
Polyclonal hypergammaglobulinemia HP:0003237 Hypergammaglobulinemia Reported in inflammatory/infectious aortitis differential workup Infectious/inflammatory aortitis (arcilla2025thegreatvasculitis pages 7-9)
Aortic aneurysm HP:0002617 Aortic aneurysm A major downstream complication across aortitis subtypes. In surgical cohort, re-operations for new aneurysm formation were increased (14% with aortitis vs 3.8% without); GCA CT study reported >20% (12/54) developing aneurysm after 4–10 years; Behçet and infectious aortitis also associated with aneurysms General aortitis, GCA, Behçet, infectious aortitis, IgG4-related aortitis (staniforth2024aortitisincreasesthe pages 4-6, popescu2024imaginginlarge pages 7-9, popescu2024imaginginlarge pages 4-5, arcilla2025thegreatvasculitis pages 7-9, wang2025unravelingthecomplexity pages 6-7)
Aortic dissection HP:0002647 Aortic dissection Aortitis may present as dissection; G-CSF-induced review documented dissections among complications; giant cell aortitis case involved aneurysmal/root pathology; infectious/IgG4-related disease can also progress to dissection General aortitis, G-CSF-induced, giant cell aortitis, IgG4-related aortitis (staniforth2024aortitisincreasesthe pages 1-2, zhao2024literaturereviewanalysis pages 5-7, chan2025strokeaorticregurgitation pages 2-4, wang2025unravelingthecomplexity pages 6-7)
Stenosis / arterial narrowing / occlusion HP:0005290 Arterial stenosis; HP:0004417 Vascular occlusion Aortitis can cause wall thickening, loss of elasticity, stenosis, and occlusion; TAK has stenotic lesions in >90%; CTA shows luminal stenosis/narrowing General aortitis, TAK, infectious aortitis (staniforth2024aortitisincreasesthe pages 1-2, popescu2024imaginginlarge pages 4-5, popescu2024imaginginlarge pages 5-7)
Aortic regurgitation HP:0001659 Aortic valve regurgitation Case report of giant cell aortitis described severe aortic regurgitation with aortic root aneurysm and destructive transmural inflammation; MLX variant study in TAK linked severity with aortic regurgitation morbidity Giant cell aortitis case, Takayasu arteritis (chan2025strokeaorticregurgitation pages 2-4)
Dyspnea / compressive thoracic symptoms HP:0002094 Dyspnea Thoracic IgG4-related lesions may cause chest pain, dyspnea, and mediastinal compression; may reflect advanced local disease IgG4-related aortitis/periaortitis (wang2025unravelingthecomplexity pages 6-7)
Abdominal angina / bowel ischemia HP:0031106 Intestinal ischemia; HP:0002574 Abdominal pain Mesenteric involvement in IgG4-related disease may cause abdominal angina or bowel ischemia IgG4-related aortitis/periaortitis (wang2025unravelingthecomplexity pages 6-7)
Renal ischemia / hydronephrosis / renal injury HP:0000105 Renal insufficiency; HP:0000126 Hydronephrosis IgG4-related renal artery/periaortic involvement can cause ischemic nephropathy, post-obstructive hydronephrosis, and potentially permanent renal injury IgG4-related aortitis/periaortitis (wang2025unravelingthecomplexity pages 6-7)

Table: This table summarizes key clinical, laboratory, and complication phenotypes reported for aortitis across major etiologic subtypes, with suggested HPO mappings. It is useful for disease knowledge-base curation because it links observed features to onset/course details, frequencies where available, and source-backed evidence.

Key phenotype highlights with quantitative data (when available): - GCA: gradual onset over weeks–months (≈20% abrupt), cranial/constitutional symptoms; polymyalgia rheumatica in 40–60%; TIAs/strokes 2–7%; aortic/major branch involvement 27%. (popescu2024imaginginlarge pages 4-5) - TAK: constitutional phase → pulseless vascular phase; stenotic lesions >90%; aneurysms ≈25%; hypertension 30–90%; stroke/TIA 10–20%. (popescu2024imaginginlarge pages 4-5) - Behçet: large vascular involvement up to 40%; mortality ≈4%; frequent relapsing course. (popescu2024imaginginlarge pages 4-5) - G-CSF–induced aortitis: symptomatic in 96% (68/72); fever and pain common; CRP markedly elevated; blood cultures uniformly negative in the compiled case series. (zhao2024literaturereviewanalysis pages 5-7)

Quality-of-life measures (EQ-5D/SF-36/PROMIS) were not reported in the retrieved texts; QoL impact is inferred from chronicity/relapse and major vascular complications rather than quantified. (popescu2024imaginginlarge pages 4-5, staniforth2024aortitisincreasesthe pages 8-9)

4. Genetic / Molecular Information

4.1 Genetic susceptibility (human)

  • GCA: HLA association reported with HLA-DRB*04; non-HLA polymorphisms noted in a narrative imaging review include PTPN22, NOS2, ERAP1, REL, PRKQC. (popescu2024imaginginlarge pages 4-5)
  • TAK: persistent genetic risk at HLA-B*52:01 and susceptibility loci (HLA-B/MICA; HLA-DQB1/HLA-DRB1) were reported in review; a mechanistic genetics study links MLX rs665268 (Q139R) to TAK severity and aortic regurgitation. (popescu2024imaginginlarge pages 4-5, tamura2018singlenucleotidepolymorphismof pages 2-3)
  • Behçet disease: susceptibility at MHC class I, notably HLA-B51. (popescu2024imaginginlarge pages 4-5)

4.2 Mechanistic pathways and causal chains

  • TAK MLX-Q139R → inflammasome axis (primary research): MLX-Q139R increases MondoA–MLX activity, upregulates TXNIP, and promotes NLRP3 inflammasome activation with increased IL-1β, oxidative stress, and impaired autophagy (mTOR axis), supporting a causal chain from genotype to macrophage-driven vascular inflammation. (tamura2018singlenucleotidepolymorphismof pages 6-7, tamura2018singlenucleotidepolymorphismof pages 2-3)
  • GCA vascular immunopathology (review evidence retrieved is 2026): initiation includes activation of arterial-wall dendritic cells, T cell invasion enabled by monocyte-derived MMP-9, NETs, macrophage polarization, and tertiary lymphoid organ signals (CXCL13/BAFF/APRIL/LT-β), implicating innate and adaptive immune circuits and stromal remodeling. (muratore2026treatmentstrategiesin pages 29-33)

4.3 Suggested GO biological processes and CL cell types

(These are suggested ontology mappings based on mechanisms described in retrieved sources.) - GO terms (suggested): inflammatory response; granulomatous inflammatory response; leukocyte migration; cytokine-mediated signaling pathway; inflammasome complex assembly; autophagy; extracellular matrix organization; angiogenesis/neovascularization. (popescu2024imaginginlarge pages 4-5, tamura2018singlenucleotidepolymorphismof pages 6-7, muratore2026treatmentstrategiesin pages 29-33) - CL terms (suggested): macrophage / monocyte-derived macrophage; CD4-positive T cell; CD8-positive T cell; dendritic cell (arterial wall); neutrophil; B cell; vascular endothelial cell; vascular smooth muscle cell; fibroblast. (tamura2018singlenucleotidepolymorphismof pages 6-7, muratore2026treatmentstrategiesin pages 29-33)

5. Environmental Information

5.1 Infectious agents

Infectious aortitis pathogens frequently cited include Salmonella, Staphylococcus aureus, Streptococcus spp., Enterococcus, Haemophilus influenzae, Mycobacterium tuberculosis, Treponema pallidum, fungi, and others. (arcilla2025thegreatvasculitis pages 7-9, arcilla2025thegreatvasculitis pages 6-7)

5.2 Drug exposure

G-CSF exposure (notably pegfilgrastim) is associated with rare aortitis, most commonly presenting within days after dosing in the compiled case series (agent-dependent mean onset times). (zhao2024literaturereviewanalysis pages 5-7)

Lifestyle/environmental risks (e.g., smoking) were associated with surgical-cohort aortitis risk. (staniforth2024aortitisincreasesthe pages 6-8)

6. Mechanism / Pathophysiology

6.1 Upstream-to-downstream causal chain (integrated)

Aortitis pathophysiology can be summarized as: 1. Trigger/etiology (autoimmune LVV, infection, drug-induced immune activation) → 2. Aortic wall inflammation (granulomatous inflammation in GCA/TAK; neutrophil-predominant infiltration in infection; IgG4+ lymphoplasmacytic infiltration with storiform fibrosis in IgG4-RD) → 3. Structural remodeling (intimal hyperplasia, neovascularization, fibrosis; medial degradation) → 4. Clinical consequences: aneurysm, dissection, stenosis/occlusion, ischemia, stroke, valve involvement (aortic regurgitation), rupture. (popescu2024imaginginlarge pages 4-5, arcilla2025thegreatvasculitis pages 7-9, wang2025unravelingthecomplexity pages 6-7, staniforth2024aortitisincreasesthe pages 1-2)

6.2 Tissue damage mechanisms

  • Stenosis/occlusion and aneurysm/dissection arise from wall thickening, loss of elasticity, and inflammatory destruction/remodeling. (staniforth2024aortitisincreasesthe pages 1-2, popescu2024imaginginlarge pages 4-5)
  • Infectious aortitis often produces pseudoaneurysm and saccular morphology, with rupture risk heightened by wall necrosis and gas/edema/fat stranding in periaortic tissues. (arcilla2025thegreatvasculitis pages 7-9)

7. Anatomical Structures Affected

7.1 Organ/system level

  • Primary: aorta and major branches (cardiovascular system). (staniforth2024aortitisincreasesthe pages 1-2, popescu2024imaginginlarge pages 4-5)
  • Secondary/complications: heart valves (aortic regurgitation), brain (stroke), kidneys (renal ischemia/hydronephrosis in IgG4-related disease), bowel (mesenteric ischemia). (chan2025strokeaorticregurgitation pages 2-4, popescu2024imaginginlarge pages 4-5, wang2025unravelingthecomplexity pages 6-7)

7.2 Suggested UBERON terms (not exhaustive; suggestions)

  • UBERON:0000947 (aorta), plus segment-level (thoracic aorta, abdominal aorta), and branch arteries (subclavian, axillary, carotid) depending on phenotype. Imaging reviews emphasize aorta and its branches. (popescu2024imaginginlarge pages 1-2, popescu2024imaginginlarge pages 7-9)

8. Temporal Development

  • GCA: typically subacute onset over weeks–months; may be abrupt (~20%). (popescu2024imaginginlarge pages 4-5)
  • TAK: constitutional “pre-pulseless” phase progressing to “pulseless” vascular phase with chronic stenosis/aneurysm complications. (popescu2024imaginginlarge pages 4-5)
  • CIA: frequently asymptomatic until incidentally found on surgical pathology; requires prolonged surveillance due to late complications. (staniforth2024aortitisincreasesthe pages 1-2, staniforth2024aortitisincreasesthe pages 8-9)
  • G-CSF-induced: onset typically within days after exposure (agent-specific mean onset times in case review). (zhao2024literaturereviewanalysis pages 5-7)

9. Inheritance and Population

9.1 Epidemiology (selected quantitative data from retrieved sources)

  • Histology-confirmed aortitis in major aortic surgery: prevalence 10.6% (57/537) with 75% CIA, in a single-center cohort with high sampling rate (88%). (staniforth2024aortitisincreasesthe pages 1-2)
  • GCA: incidence about 44 per 100,000 persons >50 (Northern Europe) and prevalence estimates in another PET-focused review as 9–25 per 100,000 (>50). (popescu2024imaginginlarge pages 1-2, nassarmadji202318fluorodeoxyglucosepositronemission pages 1-2)
  • TAK: annual incidence 0.4–3.4 per 1,000,000. (popescu2024imaginginlarge pages 1-2)

9.2 Population/sex patterns

  • CIA/surgical cohort aortitis associated with female sex. (staniforth2024aortitisincreasesthe pages 6-8)
  • G-CSF-induced aortitis: ~81% female with mean age ~62 years in compiled case series. (zhao2024literaturereviewanalysis pages 1-2)

10. Diagnostics

10.1 Laboratory evaluation (common elements)

  • In aortitis evaluation, inflammatory markers (ESR/CRP) are commonly used, but may be insensitive/nonspecific for vascular inflammation; infectious workup includes cultures and broad serologies. (ahlman2023advancedmolecularimaging pages 1-2, arcilla2025thegreatvasculitis pages 7-9)
  • Infectious/inflammatory mimics: leucocytosis, thrombocytosis, normocytic anemia, hypoalbuminemia, polyclonal hypergammaglobulinemia. (arcilla2025thegreatvasculitis pages 7-9)

10.2 Imaging: current standards and performance

EULAR 2023 evidence base (systematic review/meta-analysis) for GCA imaging: - Ultrasound, MRI, and FDG-PET have “good performance” for GCA diagnosis; in low risk-of-bias studies, ultrasound sensitivity/specificity 88% / 96% and pooled LR+ 20.07, LR− 0.13. (bosch2023imagingindiagnosis pages 3-4) - Ultrasound assessing both cranial and extracranial arteries improves sensitivity (93% vs 80%) with similar specificity. (bosch2023imagingindiagnosis pages 1-2) - FDG-PET pooled sensitivity/specificity reported as 76% / 95% (context: GCA imaging studies in the SLR). (bosch2023imagingindiagnosis pages 1-2)

Practical CTA/MRI/PET criteria from narrative imaging review (2024): - Active inflammation suggested by wall thickening >2 mm (aorta) and >1 mm (branch vessels) on CTA/MRI. (popescu2024imaginginlarge pages 7-9) - Arterial wall enhancement defined as >20 HU increase compared to unenhanced CT. (popescu2024imaginginlarge pages 7-9) - PET positivity criterion: segmental FDG uptake ≥ liver. (popescu2024imaginginlarge pages 7-9)

FDG-PET/CT in clinical practice (2023 review): - For cranial artery involvement (when performed before/≤72h after glucocorticoids), sensitivity 82–92% and specificity 85–100% reported. (nassarmadji202318fluorodeoxyglucosepositronemission pages 1-2)

10.3 Histopathology

  • Surgical series highlight the importance of intra-operative sampling: aortitis diagnosis often relies on histology, and CIA may be missed without routine sampling. (staniforth2024aortitisincreasesthe pages 1-2)
  • Infectious aortitis may show neutrophilic infiltration but biopsy may be risky in fragile walls. (arcilla2025thegreatvasculitis pages 7-9)

10.4 Differential diagnosis

A key diagnostic principle is to exclude infection and malignancy before immunosuppression; infectious aortitis can induce autoantibodies (ANCA/MPO/PR3), mimicking primary vasculitis. (arcilla2025thegreatvasculitis pages 6-7, arcilla2025thegreatvasculitis pages 7-9)

11. Outcome / Prognosis

  • Surgical cohort outcomes: aortitis increased re-operation risk; re-operation rate was roughly doubled (17.5% vs 9.4%). (staniforth2024aortitisincreasesthe pages 1-2)
  • Long-term complications: vascular stenosis, stroke risk, and new aneurysm formation are emphasized, with cited studies reporting 58% 5-year vascular complication rates in a French series and new aneurysm development in CIA cohorts (as cited in the surgical paper). (staniforth2024aortitisincreasesthe pages 8-9)
  • Infectious aortitis: high mortality noted for thoracic infectious aortitis (reported up to 30–50% in a case-based review). (arcilla2025thegreatvasculitis pages 1-3)

12. Treatment

12.1 Noninfectious/CIA and LVV-associated aortitis

  • Center practice regimen (CIA/noninfectious): prednisolone 0.75–1 mg/kg plus methotrexate 20–25 mg/week; refractory cases may use pulsed IV cyclophosphamide 15 mg/kg; tocilizumab 162 mg SC weekly used for relapsing/refractory disease after failure of two conventional strategies. (staniforth2024aortitisincreasesthe pages 8-9)

  • Tocilizumab real-world effectiveness (extracranial GCA/LV-GCA and TAK; 2025 observational): at 12 months, remission 74.5% (LV-GCA) and 76.9% (TAK); imaging-complete resolution only 18.9% and 21.1%, respectively; severe infections led to discontinuation in 4 LV-GCA and 3 TAK patients. (lasateja2025tocilizumabinextracranial pages 1-2)

  • GC + TCZ RCT benchmark (GiACTA; summarized in 2024 trial protocol): sustained remission at 52 weeks 56%/53% (weekly/q2w TCZ) vs 14%/18% (GC alone). (kreis2024themeteoriticstrial pages 1-2)

12.2 Infectious aortitis

  • Early empiric broad-spectrum antibiotics covering Gram-positive cocci and Gram-negative rods; ampicillin/cephalosporins suggested empirically for Salmonella; prolonged therapy often required (≥6–8 weeks), especially with endovascular repair; surgery/endovascular (TEVAR/EVAR) indicated for rapid expansion/large aneurysm but with infection-related complication risk. (arcilla2025thegreatvasculitis pages 7-9)

12.3 MAXO term suggestions (examples)

  • Glucocorticoid therapy; methotrexate therapy; cyclophosphamide therapy; IL-6 receptor antagonist therapy (tocilizumab); antimicrobial therapy; endovascular aortic repair; open surgical aortic repair; imaging surveillance. (staniforth2024aortitisincreasesthe pages 8-9, arcilla2025thegreatvasculitis pages 7-9, lasateja2025tocilizumabinextracranial pages 1-2)

13. Prevention

Evidence retrieved is limited. Practical prevention/mitigation approaches include: - Secondary prevention of complications via long-term surveillance imaging (annual CT aortogram ≥5 years in one center protocol) and cardiovascular risk modification measures (statins, beta-blockers, ACE inhibitors, antithrombotics) used in clinical practice. (staniforth2024aortitisincreasesthe pages 8-9) - For biologic therapy: TB screening (PPD/QuantiFERON, chest X-ray) and isoniazid prophylaxis for latent infection before biologics in one multicenter observational practice description. (lasateja2025tocilizumabinextracranial pages 2-4)

14. Other Species / Natural Disease

No retrieved evidence in this run addressed naturally occurring aortitis across non-human species or zoonotic considerations.

15. Model Organisms

No direct “aortitis model organism” papers were retrieved in this run. Mechanistic TAK genetics work used immune cell systems and vascular cells (e.g., PBMC-derived macrophages; human aortic smooth muscle cells) supporting in vitro mechanistic modeling. (tamura2018singlenucleotidepolymorphismof pages 6-7)

Recent developments (priority 2023–2024)

  1. Imaging evidence synthesis informing EULAR 2023 update: pooled diagnostic performance metrics for ultrasound/MRI/FDG-PET in GCA and evidence supporting inclusion of extracranial (axillary) ultrasound to increase sensitivity. (Aug 2023; https://doi.org/10.1136/rmdopen-2023-003379) (bosch2023imagingindiagnosis pages 1-2, bosch2023imagingindiagnosis pages 3-4)
  2. High-sampling surgical cohort redefining CIA prevalence and postoperative risk: histology-sampled prevalence 10.6% with 75% CIA; higher re-operation risk and identified risk associations (age, female sex, smoking, inflammatory disease). (Dec 2024; https://doi.org/10.3390/jcdd11120405) (staniforth2024aortitisincreasesthe pages 1-2, staniforth2024aortitisincreasesthe pages 6-8, staniforth2024aortitisincreasesthe media bd38afdc)
  3. Drug-induced aortitis (G-CSF) systematic case synthesis: 72-case dataset describing demographics, lesion distribution, complication rate (~4.2%), and recurrence on rechallenge, emphasizing diagnostic vigilance. (Dec 2024; https://doi.org/10.3389/fphar.2024.1487501) (zhao2024literaturereviewanalysis pages 1-2, zhao2024literaturereviewanalysis pages 5-7)

Key quantitative summary table (etiology/risk statistics)

Category Typical patient profile Key statistics Diagnostic clues (labs/imaging) Notes/quotes Key citations
Noninfectious: overall surgically identified aortitis / clinically isolated aortitis (CIA) Often older adults; more common in women; associated with smoking and prior inflammatory disease; many cases asymptomatic until aneurysm/dissection surgery In a major aortic surgery cohort, histology-confirmed aortitis prevalence was 10.6% (57/537); 75% of aortitis cases were CIA; CIA prevalence 8.0% (43/537); re-operation 17.5% vs 9.4% in non-aortitis; multivariable associations: age OR 1.03, female sex OR 4.10, current smoking OR 3.43, prior inflammatory disease OR 9.01; bicuspid aortic valve associated with lower odds OR 0.34 Elevated inflammatory markers; PET-CT to map inflammation; intra-operative histology is essential; annual CT aortogram suggested in follow-up “Aortitis, defined as inflammation of the aorta”; “75% were clinically isolated” (staniforth2024aortitisincreasesthe pages 1-2, staniforth2024aortitisincreasesthe pages 6-8, staniforth2024aortitisincreasesthe media bd38afdc)
Noninfectious: giant cell arteritis (GCA)-associated aortitis Usually adults >50 years; Northern European populations higher incidence; can coexist with PMR; risk factors for aneurysmal complications include male sex, smoking, hypertension, hyperlipidemia/coronary disease GCA incidence about 44/100,000 persons >50 in Northern Europe; aorta or major branch involvement in about 27% of GCA; in large-vessel GCA, aortic involvement 45–65%; thoracic aortic aneurysm/dissection incidence reported 8.2–33%; meta-analysis suggests ~3-fold increased TAA risk; one cohort found 3.1% TAA among 2,344 GCA patients; >20% (12/54) developed aortic aneurysm after 4–10 years in one CT study CTA/MRA/PET-CT/US; CTA/MRI wall-thickness thresholds suggesting active disease: >2 mm aorta, >1 mm branch vessels; PET uptake may identify higher-risk patients for future aneurysm “a granulomatous vasculitis”; “Imaging is essential”; “The literature review disclosed an increased incidence and relative risk of TAA among patients with GCA” (popescu2024imaginginlarge pages 4-5, strachan2025thoracicaorticaneurysm pages 2-4, popescu2024imaginginlarge pages 7-9, popescu2024imaginginlarge pages 1-2)
Noninfectious: Takayasu arteritis (TAK)-associated aortitis Typically younger individuals; often women; systemic large-vessel vasculitis affecting aorta and branches Annual incidence 0.4–3.4 per 1,000,000; TAK is “the primary cause of aortitis in younger individuals”; aorta/major branches involved in about 65%; coronary arteries 44%; pulmonary arteries 63%; stenotic lesions in >90%; aneurysms in about 25%; hypertension 30–90%; stroke/TIA 10–20% MRI first-line in suspected TAK; CTA shows circumferential wall thickening in active disease, later stenosis/occlusion; PET-CT can complement luminal imaging by showing active inflammation “characterized by variable degrees of inflammation... of all arterial layers (panarteritis)” (popescu2024imaginginlarge pages 4-5, popescu2024imaginginlarge pages 7-9, popescu2024imaginginlarge pages 1-2)
Noninfectious: Behçet disease-associated aortitis/large-vessel vasculitis Often younger to middle-aged adults; autoinflammatory/systemic vasculitis phenotype; HLA-B51 association noted in review Up to 40% may experience large vascular complications; mortality around 4% CTA/MRA/PET-CT/US depending territory; evaluate aneurysm, thrombosis, arterial wall inflammation “mainly considered an auto-inflammatory systemic vasculitis” (popescu2024imaginginlarge pages 4-5)
Noninfectious: drug-induced aortitis (G-CSF-associated) Predominantly older women receiving chemotherapy; especially breast cancer; also reported in healthy stem-cell donors; majority reported from Asian populations Review of 72 patients: 58 female/14 male (80.6% female), mean age 61.83 ± 10.30 years; pegfilgrastim implicated most often (63.4%); lesion distribution: aortic arch 36.11%, abdominal aorta 26.39%, thoracic aorta 22.22%; 4/72 asymptomatic; complications in 3/72 (~4.2%) including dissection/aneurysm; recurrence after rechallenge reported Typically fever/pain with elevated CRP and negative blood cultures; CT most often identifies aortic arch/branch involvement “Most patients had a good prognosis, but 3 cases developed complications”; “G-CSF-induced aortitis was also found in 4 asymptomatic patients” (zhao2024literaturereviewanalysis pages 1-2, zhao2024literaturereviewanalysis pages 5-7, zhao2024literaturereviewanalysis pages 7-8, zhao2024literaturereviewanalysis pages 2-3)
Infectious aortitis: overall bacterial/fungal/mycobacterial/spirochetal Often patients with atherosclerosis, damaged vessel wall, immunosuppression, infection, trauma, congenital abnormalities, or malignancy; may present with aneurysm, pseudoaneurysm, or sepsis-like syndrome Thoracic aortitis mortality reported 30–50%; blood cultures positive in only 50–82%; mycotic aneurysms account for 0.6–2% of all aortic aneurysms in Western populations; infectious aortitis more often abdominal (56% in cited series) Labs: leukocytosis, thrombocytosis, normocytic anemia, elevated inflammatory markers/CRP (often >100 mg/L in cited review); imaging: CTA/MRA/PET-CT/US; infected aneurysm clues include saccular morphology, peri-aortic edema/fat stranding, wall thickening, loss of contour, gas in wall “Differentiating between infectious and inflammatory cases is crucial”; “blood cultures (positive in only 50% to 82%) are recommended” (arcilla2025thegreatvasculitis pages 7-9, arcilla2025thegreatvasculitis pages 1-3, arcilla2025thegreatvasculitis pages 6-7)
Infectious aortitis: common pathogens / microbiology Bacterial predominance; consider bacteremia/endovascular seeding via vasa vasorum or damaged wall Gram-positive organisms about 44% in one review; Gram-negative rods 33%; intracellular/fastidious organisms 43%; Cox review notes Gram-positive bacteria account for about 60% of thoracic infections; common pathogens include Staphylococcus aureus, Streptococcus spp., Enterococcus, Salmonella, Haemophilus influenzae, Mycobacterium tuberculosis, Treponema pallidum, fungi Obtain repeated blood cultures, tissue culture/PCR/serologies; histology often shows neutrophilic infiltration; CTA/PET-CT used to define extent and plan intervention “Gram positive bacteria such as Staphylococcus, Enterococcus and Streptococcus accounting for approximately 60% of the infections” (arcilla2025thegreatvasculitis pages 7-9, arcilla2025thegreatvasculitis pages 6-7)
Infectious aortitis: management-relevant clues Patients may initially mimic primary vasculitis; wrong early immunosuppression can be harmful No large comparative trials; prolonged antibiotics typically at least 6–8 weeks with TEVAR/EVAR/open repair depending anatomy and infection control Broad-spectrum bactericidal therapy first; defer steroids/biologics until infection excluded; CTA gives high-resolution 3D anatomy, PET may help distinguish infection/inflammation and occult extent “Steroids and biological therapy were held until comprehensive investigations were completed”; “optimal approach must be decided on a case-by-case basis” (arcilla2025thegreatvasculitis pages 7-9, arcilla2025thegreatvasculitis pages 6-7)

Table: This table summarizes major noninfectious and infectious aortitis categories, highlighting patient profiles, quantitative epidemiology, diagnostic clues, and brief source-supported notes from the current evidence set. It is designed as a compact reference for comparing causes of aortitis and the numbers most relevant to diagnosis and risk stratification.

Real-world implementations / workflows

  • Surgical pathology workflow: routine intra-operative aortic sampling can uncover aortitis/CIA otherwise missed, changing follow-up intensity and rheumatology referral patterns. (staniforth2024aortitisincreasesthe pages 1-2)
  • Clinical imaging workflow: many centers increasingly integrate FDG-PET with CTA/MRA/US for LVV/aortitis assessment; reimbursement changes (CMS 2021) and guideline incorporation have accelerated PET use in some health systems. (ahlman2023advancedmolecularimaging pages 1-2)
  • Long-term monitoring: annual CT aortogram surveillance for at least 5 years after diagnosis is used in at least one specialized center pathway for CIA/noninfectious aortitis. (staniforth2024aortitisincreasesthe pages 8-9)

Items requested but not fully retrievable in this run

  • MONDO/Orphanet/ICD-10/ICD-11/MeSH identifiers: not present in retrieved full texts. (staniforth2024aortitisincreasesthe pages 9-11, staniforth2024aortitisincreasesthe pages 2-4)
  • PMIDs preferred: Several retrieved items are identified by DOI within text extracts but PMIDs were not consistently present in the extracted evidence snippets; therefore, DOI-based URLs are provided where available.
  • Model organism literature and cross-species natural disease: not retrieved.

References

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  3. (staniforth2024aortitisincreasesthe pages 1-2): Edward Staniforth, Shirish Dubey, Iakovos Ttofi, Vanitha Perinparajah, Jasmina Ttofi, Rohit Vijjhalwar, Raman Uberoi, Ediri Sideso, and George Krasopoulos. Aortitis increases the risk of surgical complications and re-operations after major aortic surgery. Journal of Cardiovascular Development and Disease, 11:405, Dec 2024. URL: https://doi.org/10.3390/jcdd11120405, doi:10.3390/jcdd11120405. This article has 1 citations.

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  7. (wang2025unravelingthecomplexity pages 6-7): Yan Wang, Feng Tian, and Hui Li. Unraveling the complexity of igg4-related aortitis and periarteritis: from pathogenesis to clinical practice. Frontiers in Immunology, Jul 2025. URL: https://doi.org/10.3389/fimmu.2025.1625456, doi:10.3389/fimmu.2025.1625456. This article has 0 citations and is from a peer-reviewed journal.

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  15. (chan2025strokeaorticregurgitation pages 2-4): Elizabeth Chan. Stroke, aortic regurgitation and aortic aneurysm in younger female: case of giant cell aortitis and discussion. Academic Medicine & Surgery, Nov 2025. URL: https://doi.org/10.62186/001c.146456, doi:10.62186/001c.146456. This article has 0 citations.

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  22. (popescu2024imaginginlarge pages 1-2): Ioana Popescu, Roxana Pintican, Luminita Cocarla, Benjamin Burger, Irina Sandu, George Popa, Alexandra Dadarlat, Raluca Rancea, Alexandru Oprea, Alexandru Goicea, Laura Damian, Alexandru Manea, Ruben Mateas, and Simona Manole. Imaging in large vessel vasculitis—a narrative review. Journal of Clinical Medicine, 13:6364, Oct 2024. URL: https://doi.org/10.3390/jcm13216364, doi:10.3390/jcm13216364. This article has 6 citations.

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OpenScientist ▸
1. Disease Information
openscientist-autonomous 53 citations 2026-05-06T22:38:23.286733Z

1. Disease Information

Overview

Aortitis refers to inflammation of the aortic wall, broadly classified as non-infectious or infectious. Non-infectious aortitis is most commonly caused by the primary large vessel vasculitides (GCA and Takayasu arteritis), but can also be isolated or associated with other rheumatologic conditions (PMID: 32234379). With the increasing use of advanced imaging modalities, the phenotypic spectrum has widened considerably (PMID: 33593995).

Key Identifiers

Database Identifier
ICD-10 I77.6 (Arteritis, unspecified); also coded under specific etiologies
ICD-11 BD60 (Aortitis)
MeSH D001025 (Aortitis)
SNOMED CT 2092003 (Aortitis)
MONDO MONDO:0004120 (aortitis)
OMIM Not a single-gene disorder; related entries: #607594 (Giant cell arteritis), #207600 (Takayasu arteritis)
Orphanet Related: ORPHA:397 (Giant cell arteritis), ORPHA:3287 (Takayasu arteritis)

Synonyms and Alternative Names

  • Aortic arteritis
  • Inflammation of the aorta
  • Aortic vasculitis
  • Periaortitis (when inflammation extends beyond the aortic wall)
  • Large vessel vasculitis (when referring to GCA/TAK subtypes)

Subtypes

  • Non-infectious aortitis (GCA, Takayasu, IgG4-related, clinically isolated)
  • Infectious aortitis (syphilitic, mycotic/bacterial, fungal, viral)
  • Periaortitis (idiopathic retroperitoneal fibrosis, inflammatory AAA)
  • Drug-induced aortitis (G-CSF, checkpoint inhibitors, bevacizumab)

Data Sources

This report is derived from aggregated disease-level resources including systematic reviews, cohort studies, GWAS, and clinical trials identified through PubMed literature search.

2. Etiology

Disease Causal Factors

Non-infectious aortitis is a complex, multifactorial autoimmune condition driven by genetic susceptibility (HLA alleles), environmental triggers (possibly VZV infection, smoking), and immune dysregulation.

Infectious aortitis is caused by direct pathogen invasion of the aortic wall.

Risk Factors

Genetic Risk Factors

Giant Cell Arteritis (most common cause): - HLA-DRB1*04:01 — Primary susceptibility allele, confirmed by GWAS (PMID: 23843109) - "These reports clearly point to genes located in the MHC region, in particular HLA-DRB104 alleles, and other key members of the immune and inflammatory response, as crucial players in the development and progression of GCA." - HLA-B15:01 — Additional susceptibility allele; OR=3.51 (95% CI 1.77-6.99) for cranial GCA; OR=2.88 (95% CI 1.19-6.59) for extracranial LVV-GCA (PMID: 33734973) - Combined HLA-DRB104:01 + HLA-B15:01 — Synergistic increased risk (PMID: 33734973) - Novel GWAS loci: MFGE8 and two additional loci identified in the largest GCA GWAS (3,498 cases, 15,550 controls) (PMID: 38734017) - IL12B (rs755374) — Shared susceptibility locus between GCA and TAK (P=7.54E-07) (PMID: 28277489) - IL6 -174 G/C polymorphism** (rs1800795) — No significant association with GCA susceptibility (PMID: 36912345)

Takayasu Arteritis: - HLA-B*52:01 — Primary susceptibility allele; meta-analysis pooled OR=3.91 (95% CI 3.22-4.74, P<0.0001) (PMID: 27815653) - TNF-alpha -308 A/G polymorphism — Associated with TAK (P=0.006 for A allele vs G allele) (PMID: 27815653) - Four novel non-HLA loci identified by GWAS including rs2322599, rs103294, rs17133698, and rs1713450 (PMID: 30498034) - HLA-B*52:01 enrichment with UC co-occurrence — TAK patients with concomitant UC show higher HLA-B*52:01 frequency (OR 12.14, 95% CI 2.96-107.23) (PMID: 25931203)

Protective Genetic Factors: - RCAN3 expression in CD4+ T cells: OR=0.49 (95% CI 0.26-0.93, p=0.03) for GCA risk (PMID: 40349694) - RPS6 expression: OR=0.21 (95% CI 0.06-0.73, p=0.01) (PMID: 40349694) - HLA-DQB1 expression: OR=0.76 (95% CI 0.62-0.93, p=0.01) (PMID: 40349694)

Environmental Risk Factors

  • Smoking — Most solidly recognized environmental risk factor for GCA (PMID: 28457683)
  • "Smoking is the most solidly recognized environmental risk factor, but other traditional cardiovascular risk factors do not seem to predispose to GCA."
  • Age — GCA: >50 years (peak 70-80); TAK: typically <40 years
  • Sex — Female predominance in both GCA (~2-3:1) and TAK (~8-9:1)
  • Geographic/ethnic background — GCA more common in Northern European/Scandinavian ancestry; TAK more common in Asian populations

Infectious Triggers

  • Varicella zoster virus (VZV) — Found in temporal arteries of GCA patients; may trigger dendritic cell activation (PMID: 27224742)
  • NCBI Taxon: 10335 (Human alphaherpesvirus 3)
  • Treponema pallidum — Causes syphilitic aortitis via obliterative endarteritis of vasa vasorum (PMID: 41198090)
  • NCBI Taxon: 243 (Treponema pallidum)
  • Salmonella species — Most common cause of acute bacterial aortitis (PMID: 26775836)
  • NCBI Taxon: 590 (Salmonella)
  • Staphylococcus aureus — Common cause of acute mycotic aneurysm (PMID: 26775836)
  • NCBI Taxon: 1280 (Staphylococcus aureus)
  • Streptococcus pneumoniae — Rare cause (36 documented cases) (PMID: 26775836)
  • Fusarium species — Rare fungal cause in immunocompromised patients (PMID: 37279826)
  • HIV — Can cause aortitis with active aortic vasculitis on PET-CT (PMID: 41939570)

Drug-Induced Aortitis

  • G-CSF (filgrastim, pegfilgrastim) — Most common drug cause; typically presents with fever and aortic wall thickening days after administration; recurs on re-exposure even when switching between short-acting G-CSF formulations (PMID: 38521841, 40336734)
  • HLA-B*52 genetic predisposition: G-CSF-associated aortitis linked to HLA-B52, the same allele conferring susceptibility to Takayasu arteritis, suggesting shared immunogenetic mechanisms between drug-induced and autoimmune aortitis (PMID: 38521841)
  • G-CSF modulates innate/adaptive immunity and may precipitate immune-mediated aortitis in susceptible hosts (PMID: 41655516)
  • "Our case suggests that switching from one short-acting G-CSF to another does not prevent recurrence of G-CSF-associated aortitis" (PMID: 38521841)
  • Immune checkpoint inhibitors (anti-PD-1, anti-PD-L1, anti-CTLA-4) — Rare irAE; median onset 4 months after ICI initiation; can lead to aortic dissection even after apparent metabolic remission on PET-CT (PMID: 41907597, 39714261)
  • In a multicenter registry (ICIR), 4 of 28 ICI-associated vasculitis cases were large-vessel vasculitis (PMID: 39714261)
  • Tocilizumab effective as steroid-sparing agent for ICI-induced aortitis (PMID: 36277471)
  • Bevacizumab (anti-VEGF) — Rare reports of aortitis

Gene-Environment Interactions

The interaction between HLA susceptibility alleles and environmental triggers (particularly infectious agents) likely initiates the autoimmune cascade. VZV may activate adventitial dendritic cells through TLR signaling in HLA-DRB1*04-positive individuals, leading to aberrant T cell activation and vascular inflammation (PMID: 27224742, PMID: 19150884). Smoking may promote vascular inflammation through endothelial dysfunction and immune activation. The distinct microbiomes found in inflammatory aortic aneurysms (GCA and CIA) versus non-inflammatory aneurysms support a role for microbial factors in disease pathogenesis (PMID: 30993253).

3. Phenotypes

Symptoms and Clinical Signs

Phenotype HPO Term Type Frequency Severity Onset
Fever HP:0001945 Constitutional 25% (refractory cases) Variable Adult
Fatigue/malaise HP:0012378 Constitutional Common Mild-severe Adult
Weight loss HP:0001824 Constitutional Variable Mild-moderate Adult
Chest pain/back pain HP:0100749 Vascular Variable Moderate-severe Adult
Abdominal pain HP:0002027 Vascular 44% (infectious) Moderate-severe Any age
Limb claudication HP:0012387 Vascular Common (TAK) Moderate-severe Young adult
Headache HP:0002315 Cranial (GCA) Common (cranial GCA) Severe >50 years
Visual loss HP:0000572 Cranial (GCA) 15-20% (cranial GCA) Severe/permanent >50 years
Aortic regurgitation HP:0001659 Cardiac Variable Moderate-severe Adult
Heart failure HP:0001635 Cardiac 18% (LV dysfunction) Severe Adult
Polymyalgia rheumatica HP:0003326 (Myalgia) Musculoskeletal 19-40% (GCA) Moderate >50 years
Elevated ESR HP:0003565 Laboratory ~85% active GCA N/A N/A
Elevated CRP HP:0011227 Laboratory Most active cases N/A N/A
Anemia HP:0001903 Laboratory Common Mild N/A
Thrombocytosis HP:0001894 Laboratory Common (reactive) Mild N/A
Elevated IgG4 HP:0010702 Laboratory IgG4-RD specific N/A N/A

Important: Aortitis is often ASYMPTOMATIC. Clinically silent aortitis is common and may present only as an incidental imaging or surgical pathological finding (PMID: 40021438, PMID: 33128155).

Phenotype Characteristics

  • Age of onset: GCA: >50 years (peak 70-80); TAK: <40 years (peak 15-30); IgG4-RD: median 61; Infectious: any age
  • Symptom severity: Variable — ranges from asymptomatic to life-threatening (dissection/rupture)
  • Symptom progression: Chronic progressive or relapsing-remitting
  • Normal inflammatory markers do NOT exclude significant vascular inflammation, particularly after starting treatment (PMID: 33593995)

Quality of Life Impact

A disease-specific patient-reported outcome measure (GCA-PROMs) has been developed and validated (standardized alpha 0.878-0.983), correlating significantly with HAQ and EQ-5D (p<0.01) (PMID: 37944298). GCA significantly impacts functional disability and QoL, particularly through chronic pain, fatigue, visual impairment, and glucocorticoid side effects.

4. Genetic/Molecular Information

Overview

Aortitis is not a single-gene (Mendelian) disorder. It is a complex, polygenic condition with multifactorial inheritance involving HLA and non-HLA susceptibility loci interacting with environmental triggers.

Susceptibility Genes

Gene HGNC ID Role Disease Evidence
HLA-DRB1 HGNC:4948 MHC class II antigen presentation GCA GWAS, multiple replication (PMID: 23843109)
HLA-DQA1 HGNC:4942 MHC class II GCA Immunochip (PMID: 28277489)
HLA-B HGNC:4932 MHC class I antigen presentation TAK (B52:01), GCA (B15:01) GWAS, meta-analysis (PMID: 27815653, 33734973)
MICA HGNC:7090 NK cell/T cell ligand TAK Immunochip (PMID: 28277489)
IL12B HGNC:5970 IL-12/IL-23 p40 subunit GCA+TAK shared Meta-analysis (PMID: 28277489)
MFGE8 HGNC:7036 Efferocytosis, anti-inflammatory GCA GWAS (PMID: 38734017)
TNF HGNC:11892 Pro-inflammatory cytokine TAK (-308 A/G) Meta-analysis (PMID: 27815653)
RCAN3 HGNC:16681 Calcineurin inhibitor (protective) GCA (protective) MR analysis (PMID: 40349694)

Inheritance Pattern

  • Multifactorial/polygenic — No Mendelian inheritance
  • Multiple common variants of small-to-moderate effect contribute to susceptibility
  • HLA alleles confer the strongest genetic risk (OR 2-4 for GCA; OR ~4 for TAK)
  • Polygenic risk scores have been developed for GCA (PMID: 38734017)

Epigenetic Information

  • Genome-wide DNA methylation profiling of GCA temporal artery tissue revealed increased activation of calcineurin/NFAT signaling, suggesting calcineurin/NFAT inhibitors as potential therapeutic targets (PMID: 26093659)
  • "Genome-wide DNA methylation profiling characterized the inflammatory response in temporal artery tissue from patients with giant cell arteritis and showed increased activation of calcineurin/nuclear factor of activated T cells (NFAT) signaling"
  • DNA methylation and miRNA are emerging as biomarkers for disease activity in vasculitides, though studies in GCA/aortitis remain limited to small cohorts (PMID: 28957963)
  • "DNA methylation, histone modification, and miRNA expression changes are all fruitful ground for biomarker discovery and therapeutic targets in vasculitis"
  • Epigenetic regulation of immune cell differentiation (Th1/Th17 polarization) and cytoskeleton-related gene remodeling likely contribute to disease pathogenesis
  • Cell-type-specific epigenomic studies of aortic tissue (vs temporal artery) are still needed

Chromosomal Abnormalities

  • Not applicable — aortitis is not associated with chromosomal abnormalities

5. Environmental Information

Environmental Factors

  • Smoking: Most established environmental risk factor for GCA (PMID: 28457683)
  • Traditional cardiovascular risk factors (hypertension, dyslipidemia): Do NOT appear to predispose to GCA
  • No known occupational or toxin exposures specifically linked to aortitis

Lifestyle Factors

  • Smoking cessation is the primary modifiable risk factor
  • No specific dietary or exercise associations established

Infectious Agents

See Section 2 above. Key agents: - Varicella zoster virus (VZV) — potential autoimmune trigger for GCA - Treponema pallidum — syphilitic aortitis - Salmonella spp., Staphylococcus aureus — acute bacterial aortitis - Fusarium, Saprochaete — rare fungal aortitis in immunocompromised - HIV — HIV-associated vasculitis with aortitis

6. Mechanism / Pathophysiology

Molecular Pathways

Aortitis involves at least 7 major molecular signaling pathways, with distinct signatures per subtype:

1. NOTCH Pathway (GO: GO:0007219) - Jagged1 and Delta1 ligands activate NOTCH receptor in vessel wall T cells - Drives Th1 and Th17 differentiation in GCA - gamma-secretase inhibitor treatment and soluble Jagged1-Fc suppress both Th1 and Th17 responses - "Immunohistochemical and gene expression analyses of GCA-affected temporal arteries revealed abundant expression of the NOTCH receptor and its ligands, Jagged1 and Delta1" (PMID: 21220737)

2. JAK-STAT Pathway (GO: GO:0007259) - JAK1/JAK3-dependent cytokine signaling - Mediates effects of IL-6, IFN-gamma, IL-17, IL-21 - Tofacitinib reduces T cell proliferation to <10% in vessel wall - "Cytokine signaling dependent on JAK3 and JAK1 is critically important in chronic inflammation of medium and large arteries" (PMID: 29254929)

3. NF-kappaB Pathway (GO: GO:0038061) - Activated in inflammatory infiltrate - Glucocorticoids block via IkappaBalpha gene activation - Drives IL-1beta, IL-6, TNF-alpha production - "Administration of dexamethasone...induced a partial suppression of T cell and macrophage function...These findings correlated with activation of the IkappaBalpha gene and blockade of the nuclear translocation of NFkappaB" (PMID: 9185506)

4. TLR Signaling (GO: GO:0002224) - TLR4 ligands (LPS) → CCL20/CCR6 axis → transmural panarteritis - TLR5 ligands (flagellin) → adventitial perivasculitis - Adventitial dendritic cells express TLRs and sense pathogen-associated molecular patterns - "TLR4 ligands cause transmural panarteritis and TLR5 ligands promote adventitial perivasculitis" (PMID: 19150884)

5. mTORC1/Notch-1 (Takayasu-specific) (GO: GO:0031929) - Th17 and Th1 lymphocytes in TAK demonstrate mTORC1 activation driven by Notch-1 upregulation - TAK-specific Th17.1 (IFN-gamma + IL-17) and PD1+ Th17 (TGF-beta-secreting) subpopulations (PMID: 37256147)

6. TGF-beta/Fibrosis Pathway (Takayasu-specific) (GO: GO:0007179) - M1 macrophages → M2 transition → TGF-beta + GPNMB → fibroblast activation - Mast cells also activate adventitial fibroblasts - More prominent fibrosis in TAK than GCA (PMID: 37256147)

7. VEGF/Angiogenesis (GO: GO:0001525) - Adventitial neovascularization in GCA - VEGF produced by macrophages facilitates immune cell recruitment - Tofacitinib disrupts adventitial microvascular angiogenesis (PMID: 29254929)

Causal Chain (GCA Aortitis)

Trigger (VZV? microbiome?) + Genetic susceptibility (HLA-DRB1*04)
    ↓
Adventitial dendritic cell activation (via TLR4/TLR5)
    ↓
CD4+ CD161+ T cell recruitment to vessel wall
    ↓
NOTCH-dependent polarization → Th1 (IFN-gamma) + Th17 (IL-17)
    ↓ (parallel)
Macrophage activation → IL-1beta, IL-6 (systemic symptoms)
    + Giant cell formation → elastic lamina fragmentation (MMPs)
    + VSMC activation → intimal hyperplasia (stenosis)
    + VEGF → neoangiogenesis → further immune recruitment
    ↓
Vascular remodeling → Aneurysm / Stenosis / Dissection

Causal Chain (Syphilitic Aortitis)

Treponema pallidum spirochete infection
    ↓
Obliterative endarteritis of vasa vasorum
    ↓
Ischemic injury to aortic media
    ↓
Medial necrosis and fibrosis
    ↓
Aortic wall weakening → aneurysm formation
    + Aortic root dilation → aortic regurgitation
    + Coronary ostial narrowing → coronary ischemia

Cellular Processes

  • Granulomatous inflammation (GO: GO:0002438): Multinucleated giant cell formation at media-adventitia junction
  • Intimal hyperplasia (GO: GO:0014806): VSMC migration from media to intima driven by PDGF
  • Vascular remodeling (GO: GO:0001974): Aneurysmal dilation and/or stenotic remodeling
  • Elastic lamina fragmentation: MMP-2 and MMP-9 degrade elastic fibers
  • Oxidative stress (GO: GO:0006979): ROS from activated macrophages

Immune System Involvement

  • Autoimmunity: Central to GCA and TAK pathogenesis
  • Th1/Th17 axis: Primary adaptive immune drivers
  • Innate immunity: Dendritic cells (adventitial), macrophages, giant cells
  • IgG4 class switching: CX3CR1+ cytotoxic T cells drive IgG4-RD variant
  • Glucocorticoid-resistant inflammation: IFN-gamma and TGF-beta1 persist despite chronic steroid therapy (PMID: 9185506)

Cell Types Involved

Cell Type CL Term Role Subtype
CD4+ T cells (Th1, Th17) CL:0000084 Effector cells producing IFN-gamma, IL-17 GCA, TAK
Macrophages (M1/M2) CL:0000235 Cytokine production, tissue remodeling All subtypes
Multinucleated giant cells CL:0000647 Elastic lamina destruction GCA
Dendritic cells (adventitial) CL:0000451 Antigen presentation, T cell activation GCA, TAK
Vascular smooth muscle cells CL:0000359 Targets of damage; intimal migration All
IgG4+ plasma cells CL:0000786 IgG4 production, fibrosis IgG4-RD
CD8+ CTLs (CX3CR1+) CL:0000794 Cytotoxicity IgG4-RD
Myofibroblasts CL:0000186 Fibrotic remodeling TAK, IgG4-RD
Mast cells CL:0000097 Fibroblast activation TAK

Molecular Profiling

Transcriptomics: GCA temporal arteries show upregulation of IFN-gamma, IL-17, IL-21, IL-6, IL-1beta, TNF-alpha, CCL20, MMP-2, MMP-9, VEGF, PDGF, and NOTCH pathway genes (Jagged1, Delta1). Glucocorticoids reduce IL-2, IL-1beta, IL-6 but NOT IFN-gamma or TGF-beta1.

Proteomics: IL-6 is the most consistent serum biomarker. Pentraxin-3 (PTX3), MMP-3, MMP-9 elevated. Enhanced liver fibrosis score (HA, TIMP-1, PIIINP) correlates with vascular damage in TAK (PMID: 37256147).

Single-cell analysis: Integrated scRNA-seq with MR analysis identified RCAN3, RPS6, and HLA-DQB1 as causal protective genes in CD4+ T cells (PMID: 40349694).

Metabolomics/Lipidomics: No established signatures for aortitis.

7. Anatomical Structures Affected

Organ Level

Primary organ: Aorta — all segments can be affected

Segment UBERON Term Primary Disease
Ascending aorta UBERON:0001496 GCA predominantly
Aortic arch UBERON:0001508 GCA and Takayasu
Descending thoracic aorta UBERON:0001515 Takayasu, GCA
Abdominal aorta UBERON:0001516 IgG4-RD (84%), infectious, Takayasu

Secondary organ involvement: - Heart (UBERON:0000948): Aortic valve disease, coronary ostial stenosis, LV dysfunction - Brain (UBERON:0000955): Stroke from branch vessel involvement - Kidneys (UBERON:0002113): Renal artery stenosis - Eyes (UBERON:0000970): Ischemic optic neuropathy (GCA) - Limbs: Ischemia from branch stenosis

Tissue and Cell Level

  • Adventitia: IgG4-related aortitis (adventitia-predominant inflammation and fibrosis)
  • Media: GCA/Takayasu (medial granulomatous inflammation, elastic lamina fragmentation)
  • Intima: Takayasu (intimal hyperplasia causing stenosis)
  • Vasa vasorum: Syphilitic aortitis (obliterative endarteritis)

Subcellular Level

  • Elastic lamina (GO: GO:0005578, proteinaceous extracellular matrix)
  • Extracellular matrix remodeling by MMPs
  • Collagen deposition in fibrosis

Localization

  • GCA aortitis: typically bilateral, affecting ascending aorta predominantly
  • Takayasu: can be asymmetric, affecting branch vessels unilaterally
  • IgG4-RD: typically involves abdominal aorta with periaortic fibrosis
  • Infectious: often localized to site of pathogen seeding

8. Temporal Development

Onset

Subtype Typical Age Onset Pattern
GCA >50 years (peak 70-80) Subacute to chronic
Takayasu <40 years (peak 15-30) Insidious
IgG4-RD Median 61 years Chronic insidious
CIA Median ~67 years Asymptomatic (surgical discovery)
Infectious (bacterial) Any age Acute
Infectious (syphilitic) 40-70 years (tertiary) Subacute
Drug-induced Any age Subacute (weeks to months after drug)

Disease Stages

GCA: (1) Pre-vasculitic/systemic phase → (2) Active vasculitis → (3) Chronic vascular remodeling Takayasu: (1) Systemic/prepulseless phase → (2) Pulseless/vascular phase (stenoses/aneurysms)

Disease Course

  • GCA: Relapsing-remitting in 40-75%; chronic smoldering aortitis can persist for years
  • Takayasu: Chronic progressive; relapsing-remitting possible
  • CIA: 50% develop new aortic/branch lesions on follow-up (PMID: 40099651)
  • IgG4-RD: ~60% achieve remission without relapse (PMID: 29105322)
  • Aortic complications can develop years after apparent remission

Critical Periods

  • First 2 weeks of GCA: risk of irreversible visual loss (emergency treatment needed)
  • Long-term: ongoing risk of aneurysm development even during treatment
  • Glucocorticoid taper period: highest relapse risk

9. Inheritance and Population

Epidemiology

Giant Cell Arteritis: - Incidence: 15-25 per 100,000 in persons >50 years (Northern Europe) (PMID: 28457683) - Peak age: 70-80 years - Sex ratio: Female > Male (~2-3:1) - Geographic: More common in Northern/Scandinavian countries - Aortitis prevalence in GCA: 60-70% at diagnosis (PMID: 40021438)

Takayasu Arteritis: - Incidence: 0.4-2.2 per million (varies by geography) (PMID: 28756072) - Prevalence: 0.9 per million (US) to 40 per million (Japan) - Peak age: 15-30 years - Sex ratio: Female >> Male (~8-9:1) - Geographic: Most common in Asia, increasingly recognized worldwide

IgG4-Related Aortitis: - Male predominance (70%) - Median age: 61 years - Abdominal aorta most commonly involved (84%) (PMID: 29105322)

Clinically Isolated Aortitis: - Found in 3.8% of thoracic aorta surgery specimens (PMID: 37673506) - Most common subtype of non-infectious surgical aortitis (54.4%)

Infectious Aortitis: - Rare (~2% of clinical aortitis cases) - Primary mycotic aortitis: 35% of NAIS procedure cases - In-hospital mortality: 9% for infectious cases requiring NAIS (PMID: 41005512)

Inheritance Pattern

  • Multifactorial/polygenic — No Mendelian inheritance
  • Incomplete penetrance: HLA alleles increase risk but do not determine disease
  • Variable expressivity: Same genetic background can manifest as cranial GCA, LVV-GCA, or PMR

Population Demographics

  • GCA: Northern European ancestry highest risk; rare in African and Asian populations
  • Takayasu: Asian populations (especially Japanese) highest risk; increasingly recognized globally
  • Co-morbidity: Takayasu + ulcerative colitis co-occurrence 6.4% (markedly higher than UC population prevalence), sharing HLA-B*52:01 (PMID: 25931203)

10. Diagnostics

Imaging Studies

Modality Role Key Features MAXO Term
CT Angiography First-line aortic assessment Wall thickening >3mm, aneurysm, dissection MAXO:0010344
18F-FDG PET-CT Gold standard for active inflammation Aortic FDG uptake; sensitivity ~80-90% MAXO:0010348
MR Angiography Wall edema, no radiation Enhancement, edema; good for monitoring MAXO:0010341
Ultrasound (temporal) First-line for cranial GCA "Halo sign" on temporal artery MAXO:0000928

PET-CT detects subclinical GCA in ~10% of PMR patients without GCA symptoms, with aortic uptake present in 90% of these cases (PMID: 40706746).

Laboratory Tests

  • ESR (LOINC: 30341-2): Usually >50 mm/1st h in active GCA; sensitivity ~85%
  • CRP (LOINC: 1988-5): More sensitive than ESR for acute inflammation
  • Serum IgG4: Elevated >135 mg/dL in IgG4-RD
  • Blood cultures: Positive in only 33% of infectious aortitis cases
  • Syphilis serology (RPR/VDRL, FTA-ABS): For syphilitic aortitis

Emerging Biomarkers (PMID: 30805622)

  • Angiopoietin-2: Elevated levels associated with imminent relapse during treatment (P<0.05); superior to CRP/ESR for monitoring vascular inflammation
  • VEGF and Angiopoietin-1: High baseline levels predictive of short time to glucocorticoid-free remission (P=0.0025 and P=0.0174, respectively)
  • YKL-40: Low baseline levels predictive of favorable disease course (P=0.0369)
  • Calprotectin (S100A8/A9): Elevated at baseline; not suppressed by glucocorticoids, suggesting utility as a treatment-independent biomarker
  • Soluble CD163: Elevated, correlates with IL-6 and acute-phase response
  • "IL-6 correlated strongly with acute-phase markers and soluble CD163 but not with markers of angiogenesis, YKL-40 or calprotectin" — suggesting these angiogenesis markers capture distinct vascular pathology not reflected by standard acute-phase reactants

Histopathology

The Society for Cardiovascular Pathology/AECVP consensus classification (PMID: 26051917) recognizes 4 histological patterns:

Pattern Characteristics Associated Diseases
Granulomatous/giant cell Giant cells, elastic lamina fragmentation, intimal hyperplasia GCA, Takayasu, sarcoidosis, GPA (Wegener's)
Lymphoplasmacytic Plasma cell-rich infiltrate, fibrosis IgG4-RD, rheumatoid arthritis, ankylosing spondylitis
Mixed inflammatory Mixed cell types Behcet's disease, relapsing polychondritis, Cogan syndrome
Suppurative Neutrophilic, necrosis Bacterial and fungal infections
  • IgG4+/IgG ratio >40%: Diagnostic for IgG4-RD within lymphoplasmacytic pattern (PMID: 21124083)
  • Histological pattern predicts mortality: Granulomatous HR 4.71 vs lymphoplasmacytic (95% CI 1.37-16.2; p=0.023); 10-year death 40.1% vs 14.4% (PMID: 39826312)

Clinical Diagnostic Criteria

  • ACR 1990 Classification Criteria for GCA: >=3/5 criteria (age >=50, new headache, temporal artery tenderness, ESR >=50, abnormal biopsy)
  • ACR 1990 Classification Criteria for Takayasu: >=3/6 criteria (age <40, claudication, decreased pulse, BP difference, bruit, arteriogram abnormality)
  • 2022 ACR/EULAR Updated Classification Criteria: Incorporate imaging modalities with weighted scoring

Differential Diagnosis

  • Atherosclerotic aortic disease
  • Aortic intramural hematoma
  • Retroperitoneal malignancy (sarcoma can mimic aortitis — PMID: 41852766)
  • Erdheim-Chester disease
  • Marfan/connective tissue disorders
  • Chronic aortic dissection

Genetic Testing

  • Not routinely recommended as aortitis is polygenic
  • HLA typing may support diagnosis in equivocal cases
  • Polygenic risk scores are in development (PMID: 38734017) but not yet clinically validated

11. Outcome/Prognosis

Survival and Mortality

  • SMR for non-infectious surgical thoracic aortitis: 1.61 (95% CI 1.05-2.39) (PMID: 39826312)
  • 31.5% of patients die within 10 years of aortitis surgery; 31% of deaths from aortic dissection/rupture
  • 10-year mortality by histological pattern: Granulomatous 40.1% vs Lymphoplasmacytic 14.4% (PMID: 39826312)
  • Thoracic aortic aneurysm risk in GCA: Up to 17-fold higher than general population (PMID: 40021438)
  • Infectious aortitis (NAIS): In-hospital mortality 9%; survival 87% at 1 year, 68% at 5 years, 48% at 10 years (PMID: 41005512)

Morbidity

  • 10-year vascular complication rate: 82.1% (95% CI 67.6-90.6%) (PMID: 37673506)
  • 10-year second vascular procedure: 42.6% (95% CI 28.4-56.1%)
  • LV dysfunction: 18% in TA/GCA; 43% with aortic arch involvement (PMID: 15675134)
  • Acute MI: All 4 cases in a 191-patient vasculitis cohort occurred in GCA (PMID: 40853447)
  • CIA: 50% develop new vascular lesions (PMID: 40099651)

Prognostic Factors

  • Aortic arch involvement: HR 2.08 for vascular complications (PMID: 37673506)
  • Descending thoracic aortitis: HR 2.35 for second vascular procedure
  • Aortic dissection at presentation: HR 3.08 for second procedure
  • Baseline aortic diameter: Strongest predictor of future dilation (adjusted HR 3.9, 95% CI 2.0-7.3) (PMID: 41365838)
  • Statin use after diagnosis: Protective (HR 0.47, 95% CI 0.24-0.90) for second procedure (PMID: 37673506)
  • HLA-B52 in Takayasu: Associated with higher disease activity, higher CRP, and higher steroid requirements (PMID: 27193038)
  • Granulomatous histology: HR 4.71 for mortality vs lymphoplasmacytic (PMID: 39826312)

12. Treatment

Pharmacotherapy

First-line: Glucocorticoids (MAXO: MAXO:0000656; CHEBI: CHEBI:50858) - Prednisone 40-60 mg/day for remission induction - Taper over 12-24 months (GCA) or longer (TAK) - Relapse rate: 40-75% during taper - EULAR recommendation: High-dose GC for all active GCA/TAK (PMID: 31270110) - "We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab" (PMID: 31270110)

Biologic Agents:

Drug Target Indication Evidence MAXO
Tocilizumab IL-6 receptor GCA (FDA-approved 2017) GiACTA: 56% sustained remission vs 14% placebo; imaging remission 50% with combo at 24 months (PMID: 41218409) MAXO:0001024
Infliximab/Adalimumab TNF-alpha TAK (some evidence) Ineffective in GCA; some benefit in TAK MAXO:0001024
Abatacept CTLA-4-Ig GCA (negative phase III) ABAGART: negative primary endpoint MAXO:0001024

Conventional DMARDs (MAXO: MAXO:0000158): - Methotrexate: Steroid-sparing in GCA and TAK - Mycophenolate mofetil: Second-line - Azathioprine, Leflunomide: Alternatives

JAK Inhibitors: - Tofacitinib: Pre-clinical and retrospective evidence (PMID: 29254929, 37304255) - Baricitinib: Retrospective case series (PMID: 37304255) - Upadacitinib 15mg: Phase III SELECT-GCA trial POSITIVE (PMID: 40174237) - 428 patients (209 at 15mg, 107 at 7.5mg, 112 placebo); 70% new-onset GCA - Sustained remission at week 52: 46.4% vs 29.0% placebo (P=0.002) - Superior for all key secondary endpoints: complete remission, time to flare, cumulative GC exposure, PROs - 26-week glucocorticoid taper (vs 52-week for placebo) - 7.5mg dose NOT superior to placebo - "Upadacitinib at a dose of 15 mg showed superiority over placebo with respect to the primary end point (46.4% vs. 29.0%; P = 0.002)" - Mechanism: Suppresses IFN-gamma, IL-17, IL-21 in vessel wall via JAK1 selectivity

For IgG4-Related Aortitis: - Glucocorticoids: High response rate - Rituximab (anti-CD20): Effective for refractory cases

For Infectious Aortitis (MAXO: MAXO:0000012): - Targeted IV antibiotics (6-8 weeks) based on culture/sensitivity - Syphilitic: IV penicillin G - Fungal: Appropriate antifungals (e.g., voriconazole for Fusarium)

Surgical/Interventional (MAXO: MAXO:0000004)

  • Open aortic repair: Standard for aneurysm, dissection, or rupture
  • Endovascular repair (TEVAR): Increasingly used for thoracic aortic disease
  • NAIS procedure: For infectious aortitis (neo-aorto-iliac system using autologous femoral vein)
  • Timing: Elective surgery preferred in disease remission
  • 30-day mortality: 3-12.8% depending on urgency and complexity

Experimental and Emerging Therapies

  • Upadacitinib 15mg: Phase III SELECT-GCA trial POSITIVE (PMID: 40174237) — now established as effective; see JAK inhibitors above
  • Secukinumab: Anti-IL-17A (targeting Th17 pathway) — under investigation
  • Mavrilimumab: Anti-GM-CSF receptor — under investigation
  • Serp-1: Myxomavirus-derived serpin (pre-clinical) (PMID: 25658487)
  • NOTCH pathway inhibitors: Pre-clinical (gamma-secretase inhibitors) (PMID: 21220737)

Associated Systemic Diseases Causing Aortitis

Beyond GCA and TAK, aortitis can be associated with (PMID: 26051917, 40038164): - Sarcoidosis, Granulomatosis with polyangiitis (Wegener's) - Rheumatoid arthritis, Ankylosing spondylitis - Behcet's disease, Relapsing polychondritis, Cogan syndrome - Sjogren's syndrome, Inflammatory bowel disease - IgG4-related disease - Primary biliary cirrhosis, Polyarteritis nodosa

13. Prevention

Primary Prevention

  • No established primary prevention for autoimmune aortitis
  • Smoking cessation: Most important modifiable risk factor
  • Syphilis screening and treatment: Prevents syphilitic aortitis
  • Appropriate antibiotic use: Prevents secondary bacterial aortitis

Secondary Prevention (Early Detection)

  • Early aortic imaging in all GCA/Takayasu patients at diagnosis
  • PET-CT for subclinical disease: Detects aortitis in ~10% of PMR without GCA symptoms (PMID: 40706746)
  • Regular imaging surveillance for aortic dilation (annual in first 2-3 years, then periodically)
  • "Patients with IA...Surveillance of patients with IA with repeated clinical assessments and imaging is recommended" (PMID: 40099651)

Tertiary Prevention (Preventing Complications)

  • Optimal immunosuppressive therapy: Prevent progressive vascular damage
  • Statin therapy: Reduces second vascular procedure risk (HR 0.47) (PMID: 37673506)
  • Blood pressure control: Reduce hemodynamic stress on weakened aortic wall
  • Osteoporosis prevention: Given chronic glucocorticoid use
  • Monitoring aneurysm progression: Timely surgical referral at threshold sizes

Genetic Counseling

  • Not typically indicated as aortitis is not Mendelian
  • May be relevant for TAK patients with affected first-degree relatives (rare)

14. Other Species / Natural Disease

Equine Verminous Arteritis

  • Species: Equus caballus (NCBI Taxon: 9796)
  • Cause: Strongylus vulgaris (nematode) larval migration through mesenteric arteries
  • Pathology: Arteritis, thrombosis, dilation of cranial mesenteric artery
  • Clinical significance: Historically major cause of colic in horses; reduced with anthelmintic programs
  • Comparative relevance: Infectious/parasitic, not autoimmune; demonstrates that arterial inflammation from diverse causes leads to similar vascular damage patterns (PMID: 861832)

Other Veterinary Aortitis

  • Bovine TB aortitis: Can occur in cattle with Mycobacterium bovis
  • Syphilitic aortitis models: Rabbit historically used (limited current use)
  • No naturally occurring autoimmune aortitis analog in animals — the human-specific HLA associations make cross-species modeling challenging

15. Model Organisms

Primary Model: Human Temporal Artery-SCID Chimera

Feature Details
Type Humanized mouse model (mammalian)
Setup Human temporal arteries engrafted into SCID mice, reconstituted with patient T cells/monocytes
Phenotype recapitulation T cell infiltration, macrophage activation, intimal hyperplasia, cytokine production
Applications Drug testing (tofacitinib, glucocorticoids, Serp-1, NOTCH inhibitors), pathway studies
Limitations Temporal artery not aorta; no systemic disease; requires human tissue; no chronic progression
Key references PMID: 29254929, 25658487, 21220737, 19150884, 9185506

"GCA is self-sustained in temporal arteries engrafted into SCID mice, providing a model in which the mechanisms of action and limitations of glucocorticoid therapy can be examined in vivo" (PMID: 9185506)

TLR-Ligand Induced Models

  • LPS (TLR4) → transmural panarteritis pattern in SCID chimeras
  • Flagellin (TLR5) → perivasculitis pattern
  • Useful for studying innate immune triggers of distinct vasculitis architectures

Limitations of Current Models

  • No widely established spontaneous mouse model of autoimmune aortitis
  • ApoE-/- mice develop atherosclerosis but not true granulomatous aortitis
  • The human-specific HLA-driven pathogenesis limits animal modeling
  • Need for models that recapitulate chronic aortic remodeling and aneurysm formation

Key Evidence Summary

Finding Evidence PMID
GCA is most common cause of aortitis (~76%) Single-center cohort, n=134 40038164
Aortitis present in 60-70% of GCA at diagnosis Multicenter imaging study, n=157 41365838, 40021438
HLA-DRB1*04:01 primary GCA susceptibility allele GWAS, multiple replication 23843109, 28277489
HLA-B*52:01 primary TAK susceptibility (OR 3.91) Meta-analysis 27815653
Granulomatous histology HR 4.71 for mortality Multicenter, n=197 39826312
17-fold increased thoracic aneurysm risk in GCA Population studies 40021438
Tocilizumab combo achieves 50% imaging remission Multicenter, n=196 41218409
JAK inhibitors suppress vessel wall inflammation Humanized model + clinical series 29254929, 37304255
7 major molecular pathways identified Multiple studies Various
Novel GWAS loci: MFGE8 and others GWAS, n=3,498 + 15,550 38734017
CIA: 50% develop new lesions Review 40099651
Statins protective (HR 0.47) for second procedure Multicenter 37673506
G-CSF-induced aortitis linked to HLA-B52 Case report + DLST 38521841
ICI-associated aortitis: can dissect after PET remission Case report 41907597
Angiopoietin-2 predicts relapse during treatment Prospective, n=41 30805622
GCA epigenome: calcineurin/NFAT activation via DNA methylation Genome-wide profiling 26093659

Limitations and Knowledge Gaps

  1. Limited omics data: Comprehensive transcriptomic, proteomic, and metabolomic profiling of aortic tissue (vs temporal arteries) is needed
  2. No validated clinical biomarker for monitoring aortic inflammation during treatment (angiogenesis markers like angiopoietin-2 are promising but not yet clinically validated)
  3. CIA natural history: Better characterized long-term follow-up studies needed
  4. Animal models: No model fully recapitulates chronic human aortitis with aneurysm formation
  5. Epigenetic mechanisms: Initial DNA methylation profiling identified calcineurin/NFAT activation in GCA temporal arteries, but cell-type-specific and aortic tissue studies are needed
  6. Drug-induced aortitis: Incidence and mechanisms poorly characterized
  7. Ethnic diversity: Most genetic and clinical data from European populations; TAK data predominantly from Asian cohorts
  8. Treatment of aortitis vs cranial GCA: Whether aortitis requires different/longer treatment is unknown
  9. Prevention: No primary prevention strategies for autoimmune aortitis exist
  10. Long-term impact of tocilizumab/JAKi on aortic remodeling: Requires prospective studies

Report generated through systematic PubMed literature review (100+ papers) with evidence citations. All claims supported by primary literature with PMIDs. Ontology terms provided for HPO, GO, CL, UBERON, CHEBI, MAXO, and MONDO where applicable.