A rare chronic granulomatous large-vessel vasculitis primarily affecting the aorta and its major branches. Predominantly affects young women, with highest incidence in East Asia. The disease causes segmental stenosis, occlusion, dilatation, and aneurysm formation of affected arteries, leading to limb claudication, absent pulses, hypertension, and potentially life-threatening ischemic complications.
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name: Takayasu Arteritis
creation_date: "2026-03-06T00:00:00Z"
updated_date: "2026-05-01T00:00:00Z"
category: Autoimmune
disease_term:
preferred_term: Takayasu arteritis
term:
id: MONDO:0017991
label: Takayasu arteritis
parents:
- Vasculitis
- Autoimmune Disease
description: >-
A rare chronic granulomatous large-vessel vasculitis primarily affecting the
aorta and its major branches. Predominantly affects young women, with highest
incidence in East Asia. The disease causes segmental stenosis, occlusion,
dilatation, and aneurysm formation of affected arteries, leading to limb
claudication, absent pulses, hypertension, and potentially life-threatening
ischemic complications.
pathophysiology:
- name: Granulomatous Vascular Inflammation
description: >-
T-cell and macrophage-mediated granulomatous inflammation targets the
adventitia and media of the aorta and major branches. CD4+ and CD8+ T cells,
Th17 cells, and dendritic cells infiltrate the vessel wall, along with
macrophages and multinucleated giant cells. Dendritic cells with upregulated
TLR signaling release IL-12 and IL-23, recruiting vasculitogenic T cells.
Th1/Th17 subsets contribute via IFN-gamma/IL-17/IL-6 pathways. This
inflammatory infiltrate leads to intimal hyperplasia, medial destruction,
and adventitial fibrosis. IL-6 plays a key role in driving the inflammatory
cascade.
cell_types:
- preferred_term: CD4+ T cell
term:
id: CL:0000624
label: CD4-positive, alpha-beta T cell
- preferred_term: CD8+ T cell
term:
id: CL:0000625
label: CD8-positive, alpha-beta T cell
- preferred_term: T-helper 17 cell
term:
id: CL:0000899
label: T-helper 17 cell
- preferred_term: Dendritic cell
term:
id: CL:0000451
label: dendritic cell
- preferred_term: Macrophage
term:
id: CL:0000235
label: macrophage
- preferred_term: Multinucleated giant cell
term:
id: CL:0000647
label: multinucleated giant cell
- preferred_term: Vascular smooth muscle cell
term:
id: CL:0000192
label: smooth muscle cell
biological_processes:
- preferred_term: Inflammatory response
term:
id: GO:0006954
label: inflammatory response
- preferred_term: T cell mediated immunity
term:
id: GO:0002456
label: T cell mediated immunity
- preferred_term: Macrophage activation
term:
id: GO:0042116
label: macrophage activation
downstream:
- target: Vascular Remodeling and Stenosis
evidence:
- reference: PMID:29191819
reference_title: "Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a randomised, double-blind, placebo-controlled, phase 3 trial in Japan (the TAKT study)."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
To investigate the efficacy and safety of the interleukin-6 receptor
antibody tocilizumab in patients with Takayasu arteritis (TAK).
explanation: >-
The TAKT study targets IL-6 receptor signaling, confirming the
central role of IL-6 in TAK pathogenesis.
- reference: PMID:33026580
reference_title: "Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Medical treatment, based on pathogenetic insights into the roles of
humoral and cell-mediated immune mechanisms, included glucocorticoids
mostly combined with steroid-sparing immunosuppressive agents and, in
patients with relapsing/refractory disease, biologic drugs.
explanation: >-
The Italian cohort study confirms the role of both humoral and
cell-mediated immune mechanisms in TAK pathogenesis.
- name: Vascular Remodeling and Stenosis
description: >-
Chronic inflammation of the arterial wall leads to intimal hyperplasia,
medial smooth muscle cell destruction, and adventitial fibrosis. These
structural changes result in segmental stenosis, occlusion, dilatation,
and aneurysm formation. Stenotic lesions are far more common than
aneurysms.
biological_processes:
- preferred_term: Extracellular matrix organization
term:
id: GO:0030198
label: extracellular matrix organization
evidence:
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Sixty-eight percent of patients had extensive vascular disease;
stenotic lesions were 3.6-fold more common than were aneurysms (98%
compared with 27%).
explanation: >-
The NIH cohort demonstrates that stenotic lesions predominate over
aneurysmal disease, confirming the vascular remodeling pattern.
- name: HLA-B*52 Genetic Susceptibility
description: >-
The HLA-B*52:01 allele is the strongest genetic susceptibility factor for
Takayasu arteritis across multiple populations. IL12B and MLX loci also
contribute to disease susceptibility. HLA-B*52:01 is associated not only
with disease susceptibility but also with clinical phenotype severity.
biological_processes:
- preferred_term: Immune response
term:
id: GO:0006955
label: immune response
downstream:
- target: Granulomatous Vascular Inflammation
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: HUMAN_CLINICAL
snippet: >-
The HLA-B*52 allele was found to be associated with TA (pooled OR
3.91, 95 % CI 3.22-4.74, P < 0.0001).
explanation: >-
Meta-analysis of 20 studies with 1864 TA patients confirms strong
HLA-B*52 association with Takayasu arteritis.
- reference: PMID:26178430
reference_title: "Revisited HLA and non-HLA genetics of Takayasu arteritis--where are we?"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
HLA-B*52:01 is associated with TAK beyond population. Many of the
associations other than HLA-B*52:01 can be explained by a haplotype
with HLA-B*52:01.
explanation: >-
Review confirms HLA-B*52:01 as the primary susceptibility allele across
populations, with IL12B having a central role in onset and progression.
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HLA-B | major histocompatibility complex, class I, B | hgnc:4932 | Major susceptibility factor in"
explanation: >-
Orphanet lists HLA-B as a major susceptibility factor for Takayasu arteritis.
genetic:
- name: HLA-B*52:01 Susceptibility Allele
association: Major susceptibility factor
gene_term:
preferred_term: HLA-B
term:
id: hgnc:4932
label: HLA-B
notes: >-
The HLA-B*52:01 allele confers approximately 4-fold increased risk for
Takayasu arteritis. This association has been confirmed across Japanese,
Korean, Indian, Turkish, and other populations.
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: HUMAN_CLINICAL
snippet: >-
The HLA-B*52 allele was found to be associated with TA (pooled OR
3.91, 95 % CI 3.22-4.74, P < 0.0001).
explanation: >-
Meta-analysis demonstrates a highly significant association between
HLA-B*52 and TA susceptibility.
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HLA-B | major histocompatibility complex, class I, B | hgnc:4932 | Major susceptibility factor in"
explanation: >-
Orphanet identifies HLA-B as a major susceptibility factor.
- name: IL12B Susceptibility Locus
association: Major susceptibility factor
gene_term:
preferred_term: IL12B
term:
id: hgnc:5970
label: IL12B
notes: >-
The IL12B locus, encoding interleukin-12B, is a non-HLA susceptibility
gene for Takayasu arteritis identified by genome-wide association studies.
IL12B may have a central role in disease onset and progression.
evidence:
- reference: PMID:26178430
reference_title: "Revisited HLA and non-HLA genetics of Takayasu arteritis--where are we?"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Recent genome-wide association studies of TAK revealed multiple non-HLA
susceptibility genes. In particular, the IL12B region seems to have a
central role in TAK onset and its progression.
explanation: >-
GWAS review identifies IL12B as a key non-HLA susceptibility locus.
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "IL12B | interleukin 12B | hgnc:5970 | Major susceptibility factor in"
explanation: >-
Orphanet lists IL12B as a major susceptibility factor.
- name: MLX Susceptibility Locus
association: Major susceptibility factor
gene_term:
preferred_term: MLX
term:
id: hgnc:11645
label: MLX
notes: >-
The MLX locus (MAX dimerization protein MLX) has been identified as a
susceptibility factor for Takayasu arteritis.
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "MLX | MAX dimerization protein MLX | hgnc:11645 | Major susceptibility factor in"
explanation: >-
Orphanet lists MLX as a major susceptibility factor for Takayasu arteritis.
phenotypes:
- category: Cardiovascular
name: Arteritis
frequency: VERY_FREQUENT
diagnostic: true
notes: >-
Inflammation of the arterial wall is the defining feature of Takayasu
arteritis, primarily affecting the aorta and its major branches.
phenotype_term:
preferred_term: Arteritis
term:
id: HP:0012089
label: Arteritis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0012089 | Arteritis | Very frequent (99-80%)"
explanation: >-
Orphanet classifies arteritis as very frequent in Takayasu arteritis.
- reference: PMID:33026580
reference_title: "Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Takayasu arteritis (TAK) is a rare granulomatous vasculitis of unknown
etiology that mainly affects the aorta and its major branches.
explanation: >-
The Italian cohort study confirms arteritis of the aorta and branches
as the core feature.
- category: Cardiovascular
name: Arterial Stenosis
frequency: VERY_FREQUENT
diagnostic: true
phenotype_term:
preferred_term: Arterial stenosis
term:
id: HP:0100545
label: Arterial stenosis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0100545 | Arterial stenosis | Very frequent (99-80%)"
explanation: >-
Orphanet classifies arterial stenosis as very frequent.
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
stenotic lesions were 3.6-fold more common than were aneurysms (98%
compared with 27%).
explanation: >-
The NIH cohort found stenotic lesions in 98% of patients.
- category: Cardiovascular
name: Vascular Dilatation
frequency: VERY_FREQUENT
notes: >-
Abnormal dilatation of the aorta and major branches, including aneurysm
formation. Aneurysms occur in approximately 27% of patients.
phenotype_term:
preferred_term: Vascular dilatation
term:
id: HP:0002617
label: Vascular dilatation
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002617 | Dilatation | Very frequent (99-80%)"
explanation: >-
Orphanet classifies vascular dilatation as very frequent.
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Sixty-eight percent of patients had extensive vascular disease;
stenotic lesions were 3.6-fold more common than were aneurysms (98%
compared with 27%).
explanation: >-
The NIH cohort found aneurysms in 27% of patients.
- category: Cardiovascular
name: Vasculitis
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Vasculitis
term:
id: HP:0002633
label: Vasculitis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002633 | Vasculitis | Very frequent (99-80%)"
explanation: >-
Orphanet classifies vasculitis as very frequent.
- category: Cardiovascular
name: Hypertensive Crisis
frequency: VERY_FREQUENT
notes: >-
Severe hypertension episodes, often related to renal artery stenosis.
phenotype_term:
preferred_term: Hypertensive crisis
term:
id: HP:0100735
label: Hypertensive crisis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0100735 | Hypertensive crisis | Very frequent (99-80%)"
explanation: >-
Orphanet classifies hypertensive crisis as very frequent.
- category: Cardiovascular
name: Abnormal Heart Valve Morphology
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Abnormal heart valve morphology
term:
id: HP:0001654
label: Abnormal heart valve morphology
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001654 | Abnormal heart valve morphology | Very frequent (99-80%)"
explanation: >-
Orphanet classifies abnormal heart valve morphology as very frequent.
- category: Cardiovascular
name: Absent Pulses
frequency: FREQUENT
diagnostic: true
notes: >-
Diminished or absent peripheral pulses, particularly in the upper
extremities, due to arterial stenosis. Gives rise to the historical
name "pulseless disease."
phenotype_term:
preferred_term: Absent pulse
term:
id: HP:0032554
label: Absent pulse
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0032554 | Absent pulse | Frequent (79-30%)"
explanation: >-
Orphanet classifies absent pulse as frequent.
- reference: PMID:35466620
reference_title: "Takayasu Arteritis: Pattern Of Clinical And Radiological Features, Experience From Pakistan."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Limb claudication (44.4%), absent pulses (38.9%), were the common
initial manifestation.
explanation: >-
The Pakistan cohort found absent pulses in 38.9% of patients.
- category: Cardiovascular
name: Hypertension
frequency: FREQUENT
notes: >-
Renovascular hypertension due to renal artery stenosis is common and
may be the presenting feature.
phenotype_term:
preferred_term: Hypertension
term:
id: HP:0000822
label: Hypertension
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0000822 | Hypertension | Frequent (79-30%)"
explanation: >-
Orphanet classifies hypertension as frequent.
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Hypertension was most often associated with renal artery stenosis.
explanation: >-
NIH cohort confirms hypertension commonly linked to renal artery stenosis.
- reference: PMID:35466620
reference_title: "Takayasu Arteritis: Pattern Of Clinical And Radiological Features, Experience From Pakistan."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Hypertension (61.5%), blood pressure discrepancy between arms (88.9%)
and bruit (72.2%) over major vessels were common systemic features.
explanation: >-
The Pakistan cohort found hypertension in 61.5% of patients.
- category: Cardiovascular
name: Abnormal Aortic Valve Morphology
frequency: FREQUENT
phenotype_term:
preferred_term: Abnormal aortic valve morphology
term:
id: HP:0001646
label: Abnormal aortic valve morphology
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001646 | Abnormal aortic valve morphology | Frequent (79-30%)"
explanation: >-
Orphanet classifies abnormal aortic valve morphology as frequent.
- category: Cardiovascular
name: Myocardial Infarction
frequency: FREQUENT
phenotype_term:
preferred_term: Myocardial infarction
term:
id: HP:0001658
label: Myocardial infarction
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001658 | Myocardial infarction | Frequent (79-30%)"
explanation: >-
Orphanet classifies myocardial infarction as frequent.
- category: Cardiovascular
name: Ascending Aorta Aneurysm
frequency: FREQUENT
phenotype_term:
preferred_term: Ascending aortic aneurysm
term:
id: HP:0004970
label: Ascending tubular aorta aneurysm
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0004970 | Ascending tubular aorta aneurysm | Frequent (79-30%)"
explanation: >-
Orphanet classifies ascending aortic aneurysm as frequent.
- category: Cardiovascular
name: Renal Artery Stenosis
frequency: FREQUENT
phenotype_term:
preferred_term: Renal artery stenosis
term:
id: HP:0001920
label: Renal artery stenosis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001920 | Renal artery stenosis | Frequent (79-30%)"
explanation: >-
Orphanet classifies renal artery stenosis as frequent.
- reference: PMID:35466620
reference_title: "Takayasu Arteritis: Pattern Of Clinical And Radiological Features, Experience From Pakistan."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Subclavian artery (72.2%), renal artery (33.3%), iliofemoral arteries
(27.8%), and coronary artery involvement (16.7%) were the common lesions.
explanation: >-
The Pakistan cohort found renal artery involvement in 33.3% of patients.
- category: Cardiovascular
name: Intermittent Claudication
frequency: FREQUENT
phenotype_term:
preferred_term: Intermittent claudication
term:
id: HP:0004417
label: Intermittent claudication
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0004417 | Intermittent claudication | Frequent (79-30%)"
explanation: >-
Orphanet classifies intermittent claudication as frequent.
- reference: PMID:35466620
reference_title: "Takayasu Arteritis: Pattern Of Clinical And Radiological Features, Experience From Pakistan."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Limb claudication (44.4%), absent pulses (38.9%), were the common
initial manifestation.
explanation: >-
The Pakistan cohort found limb claudication in 44.4% as the most common
initial presentation.
- category: Cardiovascular
name: Pulmonary Arterial Hypertension
frequency: FREQUENT
phenotype_term:
preferred_term: Pulmonary arterial hypertension
term:
id: HP:0002092
label: Pulmonary arterial hypertension
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002092 | Pulmonary arterial hypertension | Frequent (79-30%)"
explanation: >-
Orphanet classifies pulmonary arterial hypertension as frequent.
- category: Cardiovascular
name: Blood Pressure Higher in Legs Than Arms
frequency: FREQUENT
phenotype_term:
preferred_term: Blood pressure substantially higher in legs than arms
term:
id: HP:0020141
label: Blood pressure substantially higher in legs than arms
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0020141 | Blood pressure substantially higher in legs than arms | Frequent (79-30%)"
explanation: >-
Orphanet classifies blood pressure discrepancy as frequent.
- category: Cardiovascular
name: Asymmetric Blood Pressure Between Arms
frequency: FREQUENT
diagnostic: true
phenotype_term:
preferred_term: Asymmetric blood pressure between arms
term:
id: HP:6000945
label: Asymmetric blood pressure between arms
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:6000945 | Asymmetric blood pressure between arms | Frequent (79-30%)"
explanation: >-
Orphanet classifies asymmetric blood pressure between arms as frequent.
- reference: PMID:35466620
reference_title: "Takayasu Arteritis: Pattern Of Clinical And Radiological Features, Experience From Pakistan."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
blood pressure discrepancy between arms (88.9%) and bruit (72.2%)
over major vessels were common systemic features.
explanation: >-
The Pakistan cohort found blood pressure discrepancy between arms in
88.9% of patients.
- category: Cardiovascular
name: Chest Pain
frequency: FREQUENT
phenotype_term:
preferred_term: Chest pain
term:
id: HP:0100749
label: Chest pain
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0100749 | Chest pain | Frequent (79-30%)"
explanation: >-
Orphanet classifies chest pain as frequent.
- category: Cardiovascular
name: Gangrene
frequency: FREQUENT
phenotype_term:
preferred_term: Gangrene
term:
id: HP:0100758
label: Gangrene
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0100758 | Gangrene | Frequent (79-30%)"
explanation: >-
Orphanet classifies gangrene as frequent.
- category: Cardiovascular
name: Carotidynia
frequency: FREQUENT
phenotype_term:
preferred_term: Carotidynia
term:
id: HP:6000944
label: Carotidynia
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:6000944 | Carotidynia | Frequent (79-30%)"
explanation: >-
Orphanet classifies carotidynia as frequent.
- category: Cardiovascular
name: Aortic Regurgitation
frequency: OCCASIONAL
phenotype_term:
preferred_term: Aortic regurgitation
term:
id: HP:0001659
label: Aortic regurgitation
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001659 | Aortic regurgitation | Occasional (29-5%)"
explanation: >-
Orphanet classifies aortic regurgitation as occasional.
- category: Cardiovascular
name: Congestive Heart Failure
frequency: OCCASIONAL
phenotype_term:
preferred_term: Congestive heart failure
term:
id: HP:0001635
label: Congestive heart failure
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001635 | Congestive heart failure | Occasional (29-5%)"
explanation: >-
Orphanet classifies congestive heart failure as occasional.
- category: Cardiovascular
name: Dilated Cardiomyopathy
frequency: OCCASIONAL
phenotype_term:
preferred_term: Dilated cardiomyopathy
term:
id: HP:0001644
label: Dilated cardiomyopathy
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001644 | Dilated cardiomyopathy | Occasional (29-5%)"
explanation: >-
Orphanet classifies dilated cardiomyopathy as occasional.
- category: Cardiovascular
name: Abnormal Endocardium Morphology
frequency: OCCASIONAL
phenotype_term:
preferred_term: Abnormal endocardium morphology
term:
id: HP:0004306
label: Abnormal endocardium morphology
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0004306 | Abnormality of the endocardium | Occasional (29-5%)"
explanation: >-
Orphanet classifies endocardial abnormality as occasional.
- category: Constitutional
name: Fatigue
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Fatigue
term:
id: HP:0012378
label: Fatigue
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0012378 | Fatigue | Very frequent (99-80%)"
explanation: >-
Orphanet classifies fatigue as very frequent.
- category: Constitutional
name: Fever
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Fever
term:
id: HP:0001945
label: Fever
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001945 | Fever | Very frequent (99-80%)"
explanation: >-
Orphanet classifies fever as very frequent.
- category: Constitutional
name: Weight Loss
frequency: VERY_FREQUENT
phenotype_term:
preferred_term: Weight loss
term:
id: HP:0001824
label: Weight loss
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001824 | Weight loss | Very frequent (99-80%)"
explanation: >-
Orphanet classifies weight loss as very frequent.
- category: Constitutional
name: Anorexia
frequency: FREQUENT
phenotype_term:
preferred_term: Anorexia
term:
id: HP:0002039
label: Anorexia
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002039 | Anorexia | Frequent (79-30%)"
explanation: >-
Orphanet classifies anorexia as frequent.
- category: Constitutional
name: Malaise
frequency: OCCASIONAL
phenotype_term:
preferred_term: Malaise
term:
id: HP:0033834
label: Malaise
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0033834 | Malaise | Occasional (29-5%)"
explanation: >-
Orphanet classifies malaise as occasional.
- category: Constitutional
name: Night Sweats
frequency: OCCASIONAL
phenotype_term:
preferred_term: Night sweats
term:
id: HP:0030166
label: Night sweats
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0030166 | Night sweats | Occasional (29-5%)"
explanation: >-
Orphanet classifies night sweats as occasional.
- category: Neurological
name: Headache
frequency: FREQUENT
phenotype_term:
preferred_term: Headache
term:
id: HP:0002315
label: Headache
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002315 | Headache | Frequent (79-30%)"
explanation: >-
Orphanet classifies headache as frequent.
- category: Neurological
name: Migraine
frequency: FREQUENT
phenotype_term:
preferred_term: Migraine
term:
id: HP:0002076
label: Migraine
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002076 | Migraine | Frequent (79-30%)"
explanation: >-
Orphanet classifies migraine as frequent.
- category: Neurological
name: Seizure
frequency: FREQUENT
phenotype_term:
preferred_term: Seizure
term:
id: HP:0001250
label: Seizure
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001250 | Seizure | Frequent (79-30%)"
explanation: >-
Orphanet classifies seizure as frequent.
- category: Neurological
name: Vertigo
frequency: OCCASIONAL
phenotype_term:
preferred_term: Vertigo
term:
id: HP:0002321
label: Vertigo
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002321 | Vertigo | Occasional (29-5%)"
explanation: >-
Orphanet classifies vertigo as occasional.
- category: Neurological
name: Stroke
frequency: OCCASIONAL
phenotype_term:
preferred_term: Stroke
term:
id: HP:0001297
label: Stroke
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001297 | Stroke | Occasional (29-5%)"
explanation: >-
Orphanet classifies stroke as occasional.
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The clinical presentation ranged from asymptomatic to catastrophic
with stroke.
explanation: >-
The NIH cohort identifies stroke as part of the clinical spectrum.
- category: Neurological
name: Transient Ischemic Attack
frequency: OCCASIONAL
phenotype_term:
preferred_term: Transient ischemic attack
term:
id: HP:0002326
label: Transient ischemic attack
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002326 | Transient ischemic attack | Occasional (29-5%)"
explanation: >-
Orphanet classifies transient ischemic attack as occasional.
- category: Neurological
name: Cerebral Ischemia
frequency: OCCASIONAL
phenotype_term:
preferred_term: Cerebral ischemia
term:
id: HP:0002637
label: Cerebral ischemia
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002637 | Cerebral ischemia | Occasional (29-5%)"
explanation: >-
Orphanet classifies cerebral ischemia as occasional.
- category: Neurological
name: Reduced Consciousness
frequency: OCCASIONAL
phenotype_term:
preferred_term: Reduced consciousness
term:
id: HP:0004372
label: Reduced consciousness
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0004372 | Reduced consciousness/confusion | Occasional (29-5%)"
explanation: >-
Orphanet classifies reduced consciousness as occasional.
- category: Neurological
name: Abnormal Speech Pattern
frequency: OCCASIONAL
phenotype_term:
preferred_term: Abnormal speech pattern
term:
id: HP:0002167
label: Abnormal speech pattern
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002167 | Abnormality of speech or vocalization | Occasional (29-5%)"
explanation: >-
Orphanet classifies speech abnormality as occasional.
- category: Musculoskeletal
name: Arthritis
frequency: FREQUENT
phenotype_term:
preferred_term: Arthritis
term:
id: HP:0001369
label: Arthritis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001369 | Arthritis | Frequent (79-30%)"
explanation: >-
Orphanet classifies arthritis as frequent.
- category: Musculoskeletal
name: Myalgia
frequency: FREQUENT
phenotype_term:
preferred_term: Myalgia
term:
id: HP:0003326
label: Myalgia
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0003326 | Myalgia | Frequent (79-30%)"
explanation: >-
Orphanet classifies myalgia as frequent.
- category: Musculoskeletal
name: Muscle Weakness
frequency: FREQUENT
phenotype_term:
preferred_term: Muscle weakness
term:
id: HP:0001324
label: Muscle weakness
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001324 | Muscle weakness | Frequent (79-30%)"
explanation: >-
Orphanet classifies muscle weakness as frequent.
- category: Musculoskeletal
name: Arthralgia
frequency: OCCASIONAL
phenotype_term:
preferred_term: Arthralgia
term:
id: HP:0002829
label: Arthralgia
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002829 | Arthralgia | Occasional (29-5%)"
explanation: >-
Orphanet classifies arthralgia as occasional.
- category: Ophthalmologic
name: Retinopathy
frequency: OCCASIONAL
phenotype_term:
preferred_term: Retinopathy
term:
id: HP:0000488
label: Retinopathy
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0000488 | Retinopathy | Occasional (29-5%)"
explanation: >-
Orphanet classifies retinopathy as occasional.
- category: Ophthalmologic
name: Visual Impairment
frequency: OCCASIONAL
phenotype_term:
preferred_term: Visual impairment
term:
id: HP:0000505
label: Visual impairment
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0000505 | Visual impairment | Occasional (29-5%)"
explanation: >-
Orphanet classifies visual impairment as occasional.
- category: Ophthalmologic
name: Amaurosis Fugax
frequency: OCCASIONAL
phenotype_term:
preferred_term: Amaurosis fugax
term:
id: HP:0100576
label: Amaurosis fugax
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0100576 | Amaurosis fugax | Occasional (29-5%)"
explanation: >-
Orphanet classifies amaurosis fugax as occasional.
- category: Respiratory
name: Dyspnea
frequency: OCCASIONAL
phenotype_term:
preferred_term: Dyspnea
term:
id: HP:0002094
label: Dyspnea
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002094 | Dyspnea | Occasional (29-5%)"
explanation: >-
Orphanet classifies dyspnea as occasional.
- category: Respiratory
name: Hemoptysis
frequency: OCCASIONAL
phenotype_term:
preferred_term: Hemoptysis
term:
id: HP:0002105
label: Hemoptysis
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0002105 | Hemoptysis | Occasional (29-5%)"
explanation: >-
Orphanet classifies hemoptysis as occasional.
- category: Dermatologic
name: Skin Ulcer
frequency: FREQUENT
phenotype_term:
preferred_term: Skin ulcer
term:
id: HP:0200042
label: Skin ulcer
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0200042 | Skin ulcer | Frequent (79-30%)"
explanation: >-
Orphanet classifies skin ulcer as frequent.
- category: Dermatologic
name: Erythema Nodosum
frequency: OCCASIONAL
phenotype_term:
preferred_term: Erythema nodosum
term:
id: HP:0012219
label: Erythema nodosum
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0012219 | Erythema nodosum | Occasional (29-5%)"
explanation: >-
Orphanet classifies erythema nodosum as occasional.
- category: Hematologic
name: Anemia
frequency: FREQUENT
phenotype_term:
preferred_term: Anemia
term:
id: HP:0001903
label: Anemia
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0001903 | Anemia | Frequent (79-30%)"
explanation: >-
Orphanet classifies anemia as frequent.
- category: Hematologic
name: Increased Inflammatory Response
frequency: FREQUENT
phenotype_term:
preferred_term: Increased inflammatory response
term:
id: HP:0012649
label: Increased inflammatory response
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0012649 | Increased inflammatory response | Frequent (79-30%)"
explanation: >-
Orphanet classifies increased inflammatory response as frequent.
- category: Gastrointestinal
name: Gastrointestinal Infarctions
frequency: OCCASIONAL
phenotype_term:
preferred_term: Gastrointestinal infarctions
term:
id: HP:0005244
label: Gastrointestinal infarctions
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0005244 | Gastrointestinal infarctions | Occasional (29-5%)"
explanation: >-
Orphanet classifies gastrointestinal infarctions as occasional.
biochemical:
- name: Erythrocyte Sedimentation Rate (ESR)
presence: Elevated
context: >-
Elevated ESR is common during active disease, though normal ESR does not
exclude active inflammation.
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0003565 | Elevated erythrocyte sedimentation rate | Frequent (79-30%)"
explanation: >-
Orphanet classifies elevated ESR as frequent in TAK.
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
The erythrocyte sedimentation rate was not a consistently reliable
surrogate marker of disease activity.
explanation: >-
The NIH cohort found ESR elevated but unreliable as a sole disease
activity marker.
- name: C-Reactive Protein (CRP)
presence: Elevated
context: >-
CRP is elevated during active inflammation but may not correlate perfectly
with disease activity.
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "HP:0011227 | Elevated circulating C-reactive protein concentration | Occasional (29-5%)"
explanation: >-
Orphanet classifies elevated CRP as occasional.
treatments:
- name: Glucocorticoids
description: >-
First-line treatment for active Takayasu arteritis. High-dose
corticosteroids (prednisone 1 mg/kg/day) induce remission in the majority
of patients, but relapse is common upon tapering.
evidence:
- reference: PMID:29191819
reference_title: "Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a randomised, double-blind, placebo-controlled, phase 3 trial in Japan (the TAKT study)."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Patients with TAK who had relapsed within the previous 12 weeks were
induced into remission with oral glucocorticoid therapy.
explanation: >-
The TAKT study used glucocorticoids to induce remission in all
patients before randomization, confirming their role as standard
first-line therapy.
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Medical therapy was required for 80% of patients, whereas 20% had
monophasic self-limiting disease. Immunosuppressive treatment with
glucocorticoids alone or in combination with a cytotoxic agent failed
to induce remission in one fourth of patients; about half of those who
achieved remission later relapsed.
explanation: >-
NIH cohort confirms glucocorticoid use in 80% of patients with
significant relapse rate.
treatment_term:
preferred_term: glucocorticoid therapy
term:
id: NCIT:C122080
label: Systemic Corticosteroid Therapy
therapeutic_agent:
- preferred_term: glucocorticoid
term:
id: CHEBI:24261
label: glucocorticoid
- name: Methotrexate
description: >-
Conventional immunosuppressive agent used as steroid-sparing therapy.
Commonly combined with glucocorticoids to maintain remission and reduce
steroid dose.
evidence:
- reference: PMID:33026580
reference_title: "Takayasu arteritis: a cohort of Italian patients and recent pathogenetic and therapeutic advances."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Medical treatment, based on pathogenetic insights into the roles of
humoral and cell-mediated immune mechanisms, included glucocorticoids
mostly combined with steroid-sparing immunosuppressive agents and, in
patients with relapsing/refractory disease, biologic drugs.
explanation: >-
Confirms standard use of steroid-sparing immunosuppressive agents
including methotrexate alongside glucocorticoids.
treatment_term:
preferred_term: immunosuppressive pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: methotrexate
term:
id: CHEBI:44185
label: methotrexate
- name: Tocilizumab
description: >-
Anti-IL-6 receptor monoclonal antibody used as steroid-sparing agent.
In the TAKT phase 3 trial, tocilizumab showed a trend toward reduced
relapse risk compared to placebo.
evidence:
- reference: PMID:29191819
reference_title: "Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a randomised, double-blind, placebo-controlled, phase 3 trial in Japan (the TAKT study)."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
Although the primary endpoint was not met, the results suggest favour
for tocilizumab over placebo for time to relapse of TAK without new
safety concerns. Further investigation is warranted to confirm the
efficacy of tocilizumab in patients with refractory TAK.
explanation: >-
The TAKT trial showed a trend favoring tocilizumab but did not
meet its primary endpoint, providing partial support for efficacy.
treatment_term:
preferred_term: tocilizumab therapy
term:
id: NCIT:C15986
label: Pharmacotherapy
target_mechanisms:
- target: Granulomatous Vascular Inflammation
treatment_effect: INHIBITS
- name: Vascular Surgery
description: >-
Surgical revascularization or angioplasty for critical stenoses or
aneurysms when disease is in remission. Bypass grafting is preferred
over endovascular procedures due to lower restenosis rates.
evidence:
- reference: PMID:7909656
reference_title: "Takayasu arteritis."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Although clinically significant palliation usually occurred after
angioplasty or bypass of severely stenotic vessels, restenosis was
common.
explanation: >-
NIH cohort confirms that surgical revascularization provides
palliation but restenosis is a significant problem.
treatment_term:
preferred_term: surgical procedure
term:
id: MAXO:0000004
label: surgical procedure
prevalence:
- population: Europe
percentage: "0.001-0.009"
notes: >-
Point prevalence in Europe is estimated at 1-9 per 100,000.
Annual incidence is less than 1 per 1,000,000 in most European countries.
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "1-9 / 100 000 | Europe | Point prevalence | PMID:27159262"
explanation: >-
Orphanet epidemiology data for European point prevalence.
- population: Japan
percentage: "0.001-0.009"
notes: >-
Point prevalence in Japan is estimated at 1-9 per 100,000, with annual
incidence of 1-9 per 1,000,000. Japan has one of the highest incidence
rates worldwide.
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "1-9 / 100 000 | Japan | Point prevalence | PMID:9119531"
explanation: >-
Orphanet epidemiology data for Japanese point prevalence.
- population: Worldwide
percentage: "0.001-0.009"
notes: >-
Worldwide point prevalence is estimated at 1-9 per 100,000.
Highest incidence in East Asia with lower rates in Europe and North America.
evidence:
- reference: ORPHA:3287
reference_title: "Takayasu arteritis"
supports: SUPPORT
snippet: "1-9 / 100 000 | Worldwide | Point prevalence | ORPHANET"
explanation: >-
Orphanet epidemiology data for worldwide point prevalence.
datasets:
Takayasu arteritis is a chronic granulomatous inflammatory large-vessel vasculitis that predominantly involves the aorta and its primary branches, leading to vessel-wall thickening and remodeling with stenosis/occlusion and/or aneurysm formation; clinical consequences arise from organ ischemia and vascular complications. This definition is explicit in the 2023 Chinese guideline (published 2024). (tian2024chineseguidelinefor pages 1-2)
The 2023 International Heart Journal review similarly defines TAK as “a chronic large vessel vasculitis with predilection to affect the aorta and its branches,” highlighting vessel-wall thickening/fibrosis and ischemic complications. (as2023currentdiagnosisand pages 1-2)
Authoritative sources and trial records show the following synonyms/alternative names: * Aortic arch syndrome, non-specific aortoarteritis, pulseless disease (explicitly listed as alternative names in the Korean population-based study). (jang2021survivalandcauses pages 1-2) * Aorto-arteritis, reversed coarctation of aorta (keywords in the 2023 Int Heart J review). (as2023currentdiagnosisand pages 1-2) * Abbreviations: TA, TAK. (as2023currentdiagnosisand pages 1-2)
The precise etiology remains incompletely defined, but TAK is strongly supported as an immune-mediated disease with both cellular and humoral immune components, featuring granulomatous arterial-wall inflammation that evolves into vascular remodeling. (bhandari2023pathophysiologydiagnosisand pages 3-4, as2023currentdiagnosisand pages 1-2)
TAK preferentially affects young women and is more common in Asian populations in multiple reviews/guidelines. (tian2024chineseguidelinefor pages 1-2, as2023currentdiagnosisand pages 1-2)
A 2023 meta-analysis of observational studies (30 studies; n=5,548) quantified TB burden among TAK patients: * Any TB infection prevalence: 31.27% (95% CI 20.48–43.11%) * Latent TB infection: 50.01% (95% CI 31.25–68.77%) * Active TB: 14.40% (95% CI 9.03–20.68%) * Strong regional variation (e.g., Western Pacific 16.93% vs African region 63.58%). (li2023prevalenceoftuberculosis pages 1-2)
These data support the expert conclusion to consider “rigorous TB screening measures and preventive interventions specifically tailored for the TAK population.” (li2023prevalenceoftuberculosis pages 1-2)
A 2023 review describes TAK genetic susceptibility loci (e.g., HLA-B*52 highlighted in reviews; additional loci including IL12B, IL6 noted), consistent with a complex susceptibility architecture rather than a single causal gene. (bhandari2023pathophysiologydiagnosisand pages 8-9, as2023currentdiagnosisand pages 1-2)
No protective genetic or environmental factors were explicitly identified in the retrieved evidence; therefore, no protection claims are made here. (bhandari2023pathophysiologydiagnosisand pages 3-4)
The retrieved evidence supports an epidemiologic and mechanistic plausibility for infection-triggering hypotheses (e.g., TB antigens as triggers) but does not provide explicit gene–environment interaction datasets; therefore, this remains an evidence gap in this tool run. (as2023currentdiagnosisand pages 1-2, li2023prevalenceoftuberculosis pages 1-2)
Pediatric data provide concrete phenotype frequencies. In a 15-year ambispective cohort of 101 childhood-onset TAK: * Hypertension: 70.3% * Blood pressure discrepancy: 55.4% * Bruits: 51.5% * Pulse deficits: 37.6% * Common arterial involvement: renal artery 62.4%, subclavian artery 43.6%, abdominal aorta 42.6%, carotid artery 42.6%. (fan2019clinicalcourseand pages 1-2)
Adult phenotypes are more variably described across cohorts, but ischemia-related manifestations (claudication, pulse inequality/loss, bruits) are consistently emphasized in reviews and guidelines. (tian2024chineseguidelinefor pages 1-2, as2023currentdiagnosisand pages 1-2)
The TAKT long-term extension study evaluated patient-reported outcomes via the SF-36 and reported clinically improved physical and mental component summary scores maintained over 96 weeks of tocilizumab treatment. (nakaoka2020longtermefficacyand pages 1-2)
(Phenotype frequencies are best supported by pediatric cohort data above; adult frequencies were not quantified in the retrieved evidence.) (fan2019clinicalcourseand pages 1-2)
TAK is not supported as a single-gene (Mendelian) disorder in the retrieved evidence. Instead, susceptibility loci and immune pathway genes (e.g., IL12B, IL6, HLA-B52) are implicated in association studies and mechanistic frameworks. (bhandari2023pathophysiologydiagnosisand pages 8-9, as2023currentdiagnosisand pages 1-2)
No ClinVar/ACMG-style variant assertions, population allele frequencies (gnomAD), or chromosomal abnormalities were present in the retrieved sources. Therefore, this section is an evidence gap in the current tool-accessible corpus. (bhandari2023pathophysiologydiagnosisand pages 8-9)
No TAK-specific methylation/histone evidence was present in retrieved sources. (misra2023arterialwallfibrosis pages 1-2)
Mycobacterium tuberculosis is the main infectious agent highlighted across recent review and meta-analysis literature as a potential trigger/association; the 2023 meta-analysis quantified TB prevalence in TAK (see Etiology). (li2023prevalenceoftuberculosis pages 1-2, as2023currentdiagnosisand pages 1-2)
No specific toxins/lifestyle exposures were quantified as risk modifiers in the retrieved evidence; one review mentions non-pharmacologic measures (e.g., smoking cessation, exercise) as supportive care but not as primary prevention evidence. (bhandari2023pathophysiologydiagnosisand pages 7-8)
A consistent causal chain from recent mechanistic reviews can be summarized as: 1) Initiation at vasa vasorum / medio-adventitial junction with immune activation → panarteritis and wall thickening. (bhandari2023pathophysiologydiagnosisand pages 3-4) 2) Innate sensing and antigen presentation: aberrant vascular dendritic cells with upregulated TLR signaling release cytokines including IL-12, IL-23, IL-1β, recruiting vasculitogenic T cells. (bhandari2023pathophysiologydiagnosisand pages 3-4) 3) Effector lymphocyte injury: cytotoxic CD8+ T cells release perforin/granzymes; Th1/Th17 subsets contribute via IFN-γ/IL-17/IL-6-related pathways. (bhandari2023pathophysiologydiagnosisand pages 3-4, misra2023arterialwallfibrosis pages 1-2) 4) Macrophage polarization and tissue remodeling: M1 macrophages (IL-6) dominate inflammatory stages; with resolution, M2 macrophages secrete TGF-β and GPNMB, activating adventitial fibroblasts and promoting extracellular matrix deposition and fibrosis. (bhandari2023pathophysiologydiagnosisand pages 3-4, misra2023arterialwallfibrosis pages 1-2) 5) Fibrosis and stenosis: IL-6 and IL-17 promote fibroblast activation; Notch-1–driven mTORC1 activation in Th1/Th17 cells is described, linking immune activation to persistent remodeling/fibrosis. (misra2023arterialwallfibrosis pages 1-2, bhandari2023pathophysiologydiagnosisand pages 3-4)
This chain provides a mechanistic rationale for targeting cytokines (IL-6; TNF), T-cell pathways, and JAK/STAT signaling and (in future) anti-fibrotic strategies. (misra2023arterialwallfibrosis pages 1-2, bhandari2023pathophysiologydiagnosisand pages 5-7)
The retrieved evidence discusses molecular profiling and biomarkers as future directions but does not provide a specific single-cell/spatial multi-omics dataset in the accessible excerpts; thus, detailed omics signatures are not asserted here. (bhandari2023pathophysiologydiagnosisand pages 8-9, bhandari2023pathophysiologydiagnosisand pages 5-7)
Primary: aorta and major branches (large vessel vasculitis). (tian2024chineseguidelinefor pages 1-2, as2023currentdiagnosisand pages 1-2)
Common branch vessels (pediatric cohort frequencies): renal artery, subclavian artery, carotid artery, abdominal aorta involvement. (fan2019clinicalcourseand pages 1-2)
TAK often begins insidiously with non-specific constitutional symptoms; one review reports a mean delay from symptom onset to diagnosis of ~1.3 years (SD ±0.6). (as2023currentdiagnosisand pages 1-2)
A staged concept (active/chronic/healing; or inflammatory to fibrotic progression) is described in reviews, aligning clinical phases with inflammatory activity and subsequent fibrosis. (bhandari2023pathophysiologydiagnosisand pages 3-4, bhandari2023pathophysiologydiagnosisand pages 5-7)
Female predominance is consistent: * French multicenter cohort: 86.8% women (276/318). (comarmond2017longtermoutcomesand pages 1-4) * Pediatric cohort: 76.2% female. (fan2019clinicalcourseand pages 1-2)
The retrieved evidence supports complex susceptibility (e.g., HLA association) rather than Mendelian inheritance; no penetrance/segregation data were available. (as2023currentdiagnosisand pages 1-2)
The 2022 ACR/EULAR classification criteria incorporate imaging characteristics as an absolute requirement (key recent change). (as2023currentdiagnosisand pages 1-2)
Disease activity assessment approaches in the Chinese guideline include Kerr criteria and ITAS2010/ITAS-A thresholds (e.g., ITAS2010 ≥2; ITAS-A ≥5 for active disease). (tian2024chineseguidelinefor pages 6-7)
ESR and CRP are widely used but have limited accuracy for disease activity; pentraxin-3 (PTX3) is described as “relatively superior” and correlating with ITAS2010 in several studies (per review). (as2023currentdiagnosisand pages 1-2)
Other candidate biomarkers mentioned as promising but requiring validation include anti-endothelial cell antibodies, VEGF, IL-6, IL-8, and PTX3. (bhandari2023pathophysiologydiagnosisand pages 5-7)
Current imaging principles in retrieved sources include: * CTA often recommended as an initial diagnostic imaging modality for availability and resolution (review). (bhandari2023pathophysiologydiagnosisand pages 5-7) * DSA remains a diagnostic gold standard/reference but is invasive and unsuitable for repeated follow-up. (bhandari2023pathophysiologydiagnosisand pages 5-7) * MRI/MRA preferred for surveillance in younger patients due to lack of radiation exposure. (bhandari2023pathophysiologydiagnosisand pages 5-7) * FDG-PET/CT can localize metabolically active arterial inflammation but has radiation limitations and variable interpretation of low-grade activity. (bhandari2023pathophysiologydiagnosisand pages 5-7) * A 2023 imaging SLR informing the EULAR update reported: “No new studies on diagnostic imaging for Takayasu arteritis (TAK) were found” for the 2017–2022 update window. (bosch2023imagingindiagnosis pages 1-2)
The Chinese guideline provides an algorithmic diagnostic/treatment pathway (Figure 1). (tian2024chineseguidelinefor media 1be88471)
Not formally mapped in retrieved texts; typical monitoring includes ESR and CRP. (bhandari2023pathophysiologydiagnosisand pages 7-8, as2023currentdiagnosisand pages 1-2)
In a French nationwide multicenter study (n=318; median follow-up 6.1 years): * Relapses: 43% * Vascular complications: 38% * Death: 5% * 5- and 10-year event-free survival: 48.2% and 36.4% * 5- and 10-year relapse-free survival: 58.6% and 47.7% * 5- and 10-year complication-free survival: 69.9% and 53.7% (comarmond2017longtermoutcomesand pages 1-4)
Risk factors highlighted included progressive disease course and carotidodynia for worse EFS; male sex, elevated CRP, carotidodynia for relapse; thoracic aorta involvement and retinopathy for vascular complications. (comarmond2017longtermoutcomesand pages 1-4)
In childhood-onset TAK (n=101; median follow-up 2.4 years): events 44.6%, vascular complications 44.6%, flares 26.7%, death 3%; 5-year event-free survival 42.8%. (fan2019clinicalcourseand pages 1-2)
Across reviews and guidelines, glucocorticoids plus steroid-sparing immunosuppressants are emphasized, with biologics for refractory disease and revascularization procedures for critical lesions once inflammation is controlled. (bhandari2023pathophysiologydiagnosisand pages 7-8, tian2024chineseguidelinefor pages 1-2, bhandari2023pathophysiologydiagnosisand pages 5-7)
Commonly referenced agents include methotrexate, azathioprine, mycophenolate mofetil, leflunomide, cyclophosphamide. (bhandari2023pathophysiologydiagnosisand pages 5-7, bhandari2023pathophysiologydiagnosisand pages 7-8)
A 2023 meta-analysis (19 studies, 466 refractory TAK patients) reported: * Remission rate: 79% (95% CI 69–86%) * Relapse rate: 17% (95% CI 5–45%) * Imaging progression: 16% (95% CI 9–27%) * Retention rate: 68% (95% CI 50–82%) * Adverse events: 16% (infection 12%) * ~76% achieved glucocorticoid dose reduction (kang2023systematicreviewand pages 1-2)
The TAKT randomized trial long-term extension (up to 96 weeks) showed steroid-sparing with median glucocorticoid dose decreasing from 0.223 mg/kg/day (pre-entry relapse) to 0.131 at 48 weeks and 0.105 at 96 weeks; 46.4% reduced to <0.1 mg/kg/day; imaging mostly stable/improved (improved 17.9%, stable 67.9%). (nakaoka2020longtermefficacyand pages 1-2)
TNF inhibitors (e.g., infliximab, etanercept, adalimumab) are described as used for refractory TAK with partial clinical responses in many patients (uncontrolled/observational evidence). (bhandari2023pathophysiologydiagnosisand pages 5-7)
Recent guideline/review material notes JAK inhibitors as potentially effective and increasingly used/considered for refractory disease, but acknowledges limited controlled evidence and the need for careful safety consideration. (arita2024currentimmunosuppressivetreatment pages 5-5)
Revascularization (endovascular or open surgery) is used for critical symptomatic stenoses; guidance emphasizes delaying interventions until disease activity is controlled and performing procedures in specialized centers. (bhandari2023pathophysiologydiagnosisand pages 7-8)
ClinicalTrials.gov records retrieved in this run include: * Tocilizumab in TAK: NCT02101333 (completed) (NCT02101333 chunk 2) * Baricitinib for refractory TAK: NCT06662721 (completed; phase 2) (clinical trial listing retrieved in tool run; see overall trial set) (NCT04300686 chunk 3) * Ustekinumab: NCT04882072 (terminated; phase 3) (NCT04882072 chunk 4) * Biologic withdrawal in sustained remission: NCT07491913 (recruiting) (NCT07491913 chunk 2)
No established primary prevention strategy exists in the retrieved evidence.
Given high TB prevalence in TAK populations and the immunosuppressive treatment context, TB screening and preventive strategies are strongly supported by the 2023 meta-analysis conclusion. (li2023prevalenceoftuberculosis pages 1-2)
Tertiary prevention includes aggressive control of inflammation to prevent stenosis/aneurysm complications and procedure timing during inactive disease. (bhandari2023pathophysiologydiagnosisand pages 7-8)
No naturally occurring TAK analog in non-human species was identified in the retrieved sources. This section is an evidence gap for this tool run. (misra2023arterialwallfibrosis pages 1-2)
The retrieved sources reference experimental/“in vitro” mechanistic findings relevant to fibrosis modulation but do not provide a specific, well-validated animal model description in the accessible excerpts; therefore, model organism details are not asserted here. (misra2023arterialwallfibrosis pages 1-2)
1) Classification/diagnostics shifting toward imaging: 2022 ACR/EULAR criteria require imaging; imaging-centric pathways are emphasized in guidelines and reviews. (as2023currentdiagnosisand pages 1-2, tian2024chineseguidelinefor media 1be88471) 2) Biologic and targeted therapies expanding: 2024 therapeutic review emphasizes growth of biologic DMARD use (notably tocilizumab) and anticipated increase in use; JAK inhibitors discussed as emerging options. (arita2024currentimmunosuppressivetreatment pages 5-5) 3) Fibrosis and damage as therapeutic targets: 2023 mechanistic review frames fibrosis as prominent in TAK and discusses potential anti-fibrotic modulation alongside immunosuppression. (misra2023arterialwallfibrosis pages 1-2) 4) Comorbidity screening implications: 2023 TB prevalence meta-analysis provides quantitative rationale for TB screening policies in TAK care pathways. (li2023prevalenceoftuberculosis pages 1-2)
The following table consolidates key quantitative facts and sources extracted in this run.
| Domain | Key points (with numbers) | Key sources (PMID/DOI/URL when available) |
|---|---|---|
| Core definition / overview | Chronic granulomatous large-vessel vasculitis involving the aorta and major branches; causes wall thickening, stenosis/occlusion, aneurysm formation, and ischemic complications; often affects young women, especially in Asia (tian2024chineseguidelinefor pages 1-2, as2023currentdiagnosisand pages 1-2) | Chinese guideline 2024, doi:10.1515/rir-2024-0002, https://doi.org/10.1515/rir-2024-0002 (tian2024chineseguidelinefor pages 1-2); Int Heart J 2023, doi:10.1536/ihj.23-195, https://doi.org/10.1536/ihj.23-195 (as2023currentdiagnosisand pages 1-2) |
| Epidemiology | Annual incidence reported as ~1.11 per million person-years in one review; worldwide annual incidence ~2.6/million in Chinese guideline; global prevalence/frequency reported as 3.2–40.0 cases per million; Japan prevalence cited as ~60/million; women comprise 86.8% in a 318-patient multicenter cohort; childhood cohort 76.2% female (bhandari2023pathophysiologydiagnosisand pages 3-4, tian2024chineseguidelinefor pages 1-2, as2023currentdiagnosisand pages 1-2, nakaoka2020longtermefficacyand pages 1-2, fan2019clinicalcourseand pages 1-2, comarmond2017longtermoutcomesand pages 1-4) | Rheumatology (Oxford) 2020, doi:10.1093/rheumatology/kez630, https://doi.org/10.1093/rheumatology/kez630 (nakaoka2020longtermefficacyand pages 1-2); Circulation 2017, doi:10.1161/CIRCULATIONAHA.116.027094, https://doi.org/10.1161/CIRCULATIONAHA.116.027094 (comarmond2017longtermoutcomesand pages 1-4) |
| Infectious / risk-factor signal | Meta-analysis of 30 studies, n=5,548: pooled TB infection prevalence 31.27% (95% CI 20.48–43.11%); latent TB 50.01% (31.25–68.77%); active TB 14.40% (9.03–20.68%); African Region 63.58% vs Western Pacific 16.93%, supporting TB screening in TAK populations (li2023prevalenceoftuberculosis pages 1-2) | Sci Rep 2023, doi:10.1038/s41598-023-49998-y, https://doi.org/10.1038/s41598-023-49998-y (li2023prevalenceoftuberculosis pages 1-2) |
| Pathophysiology | Inflammation begins around vasa vasorum/medio-adventitial junction causing panarteritis; aberrant dendritic cells/TLR signaling release IL-12, IL-23, IL-1β; CD8+ T cells release perforin/granzymes; B-cell autoimmunity/anti-endothelial or anti-aorta antibodies implicated; M1 macrophages dominate active inflammation (IL-6, MMPs, ROS), M2 macrophages dominate fibrotic phase (TGF-β, PDGF, GPNMB) and activate fibroblasts; Th1/Th17, Th17.1, PD1+Th17, Notch-1/mTORC1, IL-6/IL-17/TGF-β pathways link inflammation to fibrosis (bhandari2023pathophysiologydiagnosisand pages 3-4, bhandari2023pathophysiologydiagnosisand pages 9-9, misra2023arterialwallfibrosis pages 1-2) | Front Immunol 2023, doi:10.3389/fimmu.2023.1174249, https://doi.org/10.3389/fimmu.2023.1174249 (misra2023arterialwallfibrosis pages 1-2); Cureus 2023, doi:10.7759/cureus.42667, https://doi.org/10.7759/cureus.42667 (bhandari2023pathophysiologydiagnosisand pages 3-4) |
| Diagnostics: classification criteria | 2022 ACR/EULAR classification criteria incorporate imaging as an absolute requirement; older criteria include Ishikawa 1988 and ACR 1990; Sharma modification improved sensitivity from 60% to 92.5% while preserving specificity; 2022 criteria reportedly sensitivity 90.5% and specificity 98.3% in DCVAS-related comparison vs ACR 1990 sensitivity 73.6% and specificity 97.8% (as2023currentdiagnosisand pages 1-2, bhandari2023pathophysiologydiagnosisand pages 9-10) | Int Heart J 2023, doi:10.1536/ihj.23-195, https://doi.org/10.1536/ihj.23-195 (as2023currentdiagnosisand pages 1-2) |
| Diagnostics: activity scores / biomarkers | Kerr criteria define active disease by ≥2 of 4 items; ITAS2010 active if score ≥2; ITAS-A active if ≥5; ESR/CRP have limited accuracy for activity; pentraxin-3 appears relatively superior in several studies; ESR remains commonly used and is included in activity frameworks; novel candidates include anti-endothelial cell antibodies, VEGF, IL-6, IL-8, PTX3 (bhandari2023pathophysiologydiagnosisand pages 5-7, tian2024chineseguidelinefor pages 6-7, as2023currentdiagnosisand pages 1-2) | Chinese guideline 2024, doi:10.1515/rir-2024-0002, https://doi.org/10.1515/rir-2024-0002 (tian2024chineseguidelinefor pages 6-7); Int Heart J 2023, doi:10.1536/ihj.23-195, https://doi.org/10.1536/ihj.23-195 (as2023currentdiagnosisand pages 1-2) |
| Diagnostics: imaging | CTA often favored as initial modality for availability/resolution; DSA remains reference gold standard but is invasive; MRI/MRA preferred for surveillance and younger patients due to no radiation; Doppler US/CEUS assess intima-media thickness and neovascularization; FDG-PET/CT can detect metabolically active arterial inflammation but has radiation burden and variable follow-up utility; 2023 EULAR imaging review found no new diagnostic imaging studies for TAK in 2017–2022 update (bhandari2023pathophysiologydiagnosisand pages 5-7, bhandari2023pathophysiologydiagnosisand pages 7-8, bosch2023imagingindiagnosis pages 1-2) | RMD Open 2023, doi:10.1136/rmdopen-2023-003379, https://doi.org/10.1136/rmdopen-2023-003379 (bosch2023imagingindiagnosis pages 1-2); Cureus 2023, doi:10.7759/cureus.42667, https://doi.org/10.7759/cureus.42667 (bhandari2023pathophysiologydiagnosisand pages 5-7) |
| Treatments: standard and steroid-sparing | Current practice uses glucocorticoids plus upfront steroid-sparing immunosuppressants rather than steroid monotherapy; csDMARDs include methotrexate, azathioprine, mycophenolate mofetil, leflunomide, cyclophosphamide; biologics for refractory disease include tocilizumab and TNF inhibitors; JAK inhibitors have emerging supportive reports (bhandari2023pathophysiologydiagnosisand pages 5-7, bhandari2023pathophysiologydiagnosisand pages 7-8, as2023currentdiagnosisand pages 1-2, arita2024currentimmunosuppressivetreatment pages 5-5) | Circulation Journal 2024, doi:10.1253/circj.cj-23-0780, https://doi.org/10.1253/circj.cj-23-0780 (arita2024currentimmunosuppressivetreatment pages 5-5); Int Heart J 2023, doi:10.1536/ihj.23-195, https://doi.org/10.1536/ihj.23-195 (as2023currentdiagnosisand pages 1-2) |
| Treatments: tocilizumab outcomes | Meta-analysis of 19 studies, n=466 refractory TAK: remission 79% (95% CI 69–86%), relapse 17% (5–45%), imaging progression 16% (9–27%), retention 68% (50–82%), adverse events 16% (5–39%), infection 12% (5–28%), ~76% achieved glucocorticoid reduction; long-term TAKT extension: median glucocorticoid dose fell from 0.223 mg/kg/day pre-entry relapse to 0.131 at 48 weeks and 0.105 at 96 weeks; 46.4% reduced to <0.1 mg/kg/day; imaging improved in 17.9% and stable in 67.9% after 96 weeks (kang2023systematicreviewand pages 1-2, nakaoka2020longtermefficacyand pages 1-2) | Front Immunol 2023, doi:10.3389/fimmu.2023.1084558, https://doi.org/10.3389/fimmu.2023.1084558 (kang2023systematicreviewand pages 1-2); Rheumatology 2020, doi:10.1093/rheumatology/kez630, https://doi.org/10.1093/rheumatology/kez630 (nakaoka2020longtermefficacyand pages 1-2) |
| Outcomes / prognosis | Multicenter 318-patient cohort: after median 6.1 years, relapses 43%, vascular complications 38%, death 5%; 5-/10-year event-free survival 48.2%/36.4%, relapse-free survival 58.6%/47.7%, complication-free survival 69.9%/53.7%; childhood cohort (n=101): events 44.6%, rehospitalization 37.6%, vascular complications 44.6%, flares 26.7%, death 3% at median 2.4 years; 5-year event-free survival 42.8% (fan2019clinicalcourseand pages 1-2, comarmond2017longtermoutcomesand pages 1-4) | Circulation 2017, doi:10.1161/CIRCULATIONAHA.116.027094, https://doi.org/10.1161/CIRCULATIONAHA.116.027094 (comarmond2017longtermoutcomesand pages 1-4); Arthritis Res Ther 2019, doi:10.1186/s13075-018-1790-x, https://doi.org/10.1186/s13075-018-1790-x (fan2019clinicalcourseand pages 1-2) |
Table: This table condenses the main evidence already retrieved in the chat on Takayasu arteritis, including definition, epidemiology, mechanisms, diagnosis, treatment, and prognosis. It is useful as a quick-reference artifact for building the full disease knowledge base entry with cited quantitative findings.
The Chinese guideline includes a diagnosis-and-treatment algorithm figure.
(tian2024chineseguidelinefor media 1be88471)
References
(tian2024chineseguidelinefor pages 1-2): Xinping Tian and Xiaofeng Zeng. Chinese guideline for the diagnosis and treatment of takayasu’s arteritis (2023). Rheumatology and Immunology Research, 5:5-26, Mar 2024. URL: https://doi.org/10.1515/rir-2024-0002, doi:10.1515/rir-2024-0002. This article has 14 citations.
(as2023currentdiagnosisand pages 1-2): Chandhu AS and Debashish Danda. Current diagnosis and management of takayasu arteritis. International heart journal, 64 4:519-534, Jul 2023. URL: https://doi.org/10.1536/ihj.23-195, doi:10.1536/ihj.23-195. This article has 14 citations and is from a peer-reviewed journal.
(jang2021survivalandcauses pages 1-2): Shin Yi Jang, Taek Kyu Park, and Duk‐Kyung Kim. Survival and causes of death for takayasu’s arteritis in korea: a retrospective population‐based study. International Journal of Rheumatic Diseases, 24:69-73, Oct 2021. URL: https://doi.org/10.1111/1756-185x.14005, doi:10.1111/1756-185x.14005. This article has 24 citations and is from a peer-reviewed journal.
(NCT07491913 chunk 2): Fatma Alibaz Oner. Biologic Treatment Withdrawal in Takayasu Arteritis Patients in Sustained Remission. Marmara University. 2025. ClinicalTrials.gov Identifier: NCT07491913
(NCT02101333 chunk 2): Efficacy and Tolerance of Tocilizumab In Takayasu Arteritis. Assistance Publique - Hôpitaux de Paris. 2014. ClinicalTrials.gov Identifier: NCT02101333
(NCT04882072 chunk 4): A Study of Ustekinumab in Participants With Takayasu Arteritis (TAK). Janssen Pharmaceutical K.K.. 2021. ClinicalTrials.gov Identifier: NCT04882072
(NCT02101333 chunk 3): Efficacy and Tolerance of Tocilizumab In Takayasu Arteritis. Assistance Publique - Hôpitaux de Paris. 2014. ClinicalTrials.gov Identifier: NCT02101333
(li2023prevalenceoftuberculosis pages 1-2): Liping Li, Fang Zhou, Fen Li, Jinwei Chen, and Xi Xie. Prevalence of tuberculosis infection among patients with takayasu arteritis: a meta-analysis of observational studies. Scientific Reports, Dec 2023. URL: https://doi.org/10.1038/s41598-023-49998-y, doi:10.1038/s41598-023-49998-y. This article has 10 citations and is from a peer-reviewed journal.
(kang2023systematicreviewand pages 1-2): Limei Kang, Yang Liu, Zhongling Luo, Yueyuan Zhou, Bo Chen, Geng Yin, and Qibing Xie. Systematic review and meta-analysis of the current literature on tocilizumab in patients with refractory takayasu arteritis. Frontiers in Immunology, Feb 2023. URL: https://doi.org/10.3389/fimmu.2023.1084558, doi:10.3389/fimmu.2023.1084558. This article has 15 citations and is from a peer-reviewed journal.
(bosch2023imagingindiagnosis pages 1-2): Philipp Bosch, Milena Bond, Christian Dejaco, Cristina Ponte, Sarah Louise Mackie, Louise Falzon, Wolfgang A Schmidt, and Sofia Ramiro. 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. RMD Open, 9:e003379, Aug 2023. URL: https://doi.org/10.1136/rmdopen-2023-003379, doi:10.1136/rmdopen-2023-003379. This article has 108 citations and is from a peer-reviewed journal.
(comarmond2017longtermoutcomesand pages 1-4): Cloé Comarmond, Lucie Biard, Marc Lambert, Arsène Mekinian, Yasmina Ferfar, Jean-Emmanuel Kahn, Ygal Benhamou, Laurent Chiche, Fabien Koskas, Philippe Cluzel, Eric Hachulla, Emmanuel Messas, Matthieu Resche-Rigon, Patrice Cacoub, Tristan Mirault, and David Saadoun. Long-term outcomes and prognostic factors of complications in takayasu arteritis: a multicenter study of 318 patients. Circulation, 136:1114–1122, Sep 2017. URL: https://doi.org/10.1161/circulationaha.116.027094, doi:10.1161/circulationaha.116.027094. This article has 278 citations and is from a highest quality peer-reviewed journal.
(fan2019clinicalcourseand pages 1-2): Luyun Fan, Huimin Zhang, Jun Cai, Lirui Yang, Bin Liu, Dongmei Wei, Jiachen Yu, Jiali Fan, Lei Song, Wenjun Ma, Xianliang Zhou, Haiying Wu, and Ying Lou. Clinical course and prognostic factors of childhood takayasu’s arteritis: over 15-year comprehensive analysis of 101 patients. Arthritis Research & Therapy, Jan 2019. URL: https://doi.org/10.1186/s13075-018-1790-x, doi:10.1186/s13075-018-1790-x. This article has 70 citations and is from a domain leading peer-reviewed journal.
(nakaoka2020longtermefficacyand pages 1-2): Yoshikazu Nakaoka, Mitsuaki Isobe, Yoshiya Tanaka, Tomonori Ishii, Seido Ooka, Hiroaki Niiro, Naoto Tamura, Shogo Banno, Hajime Yoshifuji, Yasushi Sakata, Atsushi Kawakami, Tatsuya Atsumi, Shunsuke Furuta, Hitoshi Kohsaka, Katsuya Suzuki, Ryoki Hara, Yasuhiro Maejima, Hiroshi Tsukamoto, Yoshinari Takasaki, Katsuhisa Yamashita, Norihiro Okada, Shinji Yamakido, Syuji Takei, Shumpei Yokota, and Norihiro Nishimoto. Long-term efficacy and safety of tocilizumab in refractory takayasu arteritis: final results of the randomized controlled phase 3 takt study. Rheumatology (Oxford, England), 59:2427-2434, Jan 2020. URL: https://doi.org/10.1093/rheumatology/kez630, doi:10.1093/rheumatology/kez630. This article has 128 citations.
(bhandari2023pathophysiologydiagnosisand pages 3-4): Sagar Bhandari, Samia Rauf R Butt, Anzal Ishfaq, Mohamed H Attaallah, Chukwuyem Ekhator, Raghu Halappa Nagaraj, Asmita Mulmi, Muhammad Kamran, Amanda Karski, Karla I Vargas, Slobodan Lazarevic, Mohammad Uzair Zaman, Gautham Lakshmipriya Vetrivendan, S M Iram Shahzed, Archana Das, Vikas Yadav, Sophia B Bellegarde, and Ashraf Ullah. Pathophysiology, diagnosis, and management of takayasu arteritis: a review of current advances. Cureus, Jul 2023. URL: https://doi.org/10.7759/cureus.42667, doi:10.7759/cureus.42667. This article has 57 citations.
(bhandari2023pathophysiologydiagnosisand pages 8-9): Sagar Bhandari, Samia Rauf R Butt, Anzal Ishfaq, Mohamed H Attaallah, Chukwuyem Ekhator, Raghu Halappa Nagaraj, Asmita Mulmi, Muhammad Kamran, Amanda Karski, Karla I Vargas, Slobodan Lazarevic, Mohammad Uzair Zaman, Gautham Lakshmipriya Vetrivendan, S M Iram Shahzed, Archana Das, Vikas Yadav, Sophia B Bellegarde, and Ashraf Ullah. Pathophysiology, diagnosis, and management of takayasu arteritis: a review of current advances. Cureus, Jul 2023. URL: https://doi.org/10.7759/cureus.42667, doi:10.7759/cureus.42667. This article has 57 citations.
(misra2023arterialwallfibrosis pages 1-2): Durga Prasanna Misra, Kritika Singh, Aman Sharma, and Vikas Agarwal. Arterial wall fibrosis in takayasu arteritis and its potential for therapeutic modulation. Frontiers in Immunology, May 2023. URL: https://doi.org/10.3389/fimmu.2023.1174249, doi:10.3389/fimmu.2023.1174249. This article has 31 citations and is from a peer-reviewed journal.
(bhandari2023pathophysiologydiagnosisand pages 7-8): Sagar Bhandari, Samia Rauf R Butt, Anzal Ishfaq, Mohamed H Attaallah, Chukwuyem Ekhator, Raghu Halappa Nagaraj, Asmita Mulmi, Muhammad Kamran, Amanda Karski, Karla I Vargas, Slobodan Lazarevic, Mohammad Uzair Zaman, Gautham Lakshmipriya Vetrivendan, S M Iram Shahzed, Archana Das, Vikas Yadav, Sophia B Bellegarde, and Ashraf Ullah. Pathophysiology, diagnosis, and management of takayasu arteritis: a review of current advances. Cureus, Jul 2023. URL: https://doi.org/10.7759/cureus.42667, doi:10.7759/cureus.42667. This article has 57 citations.
(bhandari2023pathophysiologydiagnosisand pages 5-7): Sagar Bhandari, Samia Rauf R Butt, Anzal Ishfaq, Mohamed H Attaallah, Chukwuyem Ekhator, Raghu Halappa Nagaraj, Asmita Mulmi, Muhammad Kamran, Amanda Karski, Karla I Vargas, Slobodan Lazarevic, Mohammad Uzair Zaman, Gautham Lakshmipriya Vetrivendan, S M Iram Shahzed, Archana Das, Vikas Yadav, Sophia B Bellegarde, and Ashraf Ullah. Pathophysiology, diagnosis, and management of takayasu arteritis: a review of current advances. Cureus, Jul 2023. URL: https://doi.org/10.7759/cureus.42667, doi:10.7759/cureus.42667. This article has 57 citations.
(tian2024chineseguidelinefor pages 6-7): Xinping Tian and Xiaofeng Zeng. Chinese guideline for the diagnosis and treatment of takayasu’s arteritis (2023). Rheumatology and Immunology Research, 5:5-26, Mar 2024. URL: https://doi.org/10.1515/rir-2024-0002, doi:10.1515/rir-2024-0002. This article has 14 citations.
(tian2024chineseguidelinefor media 1be88471): Xinping Tian and Xiaofeng Zeng. Chinese guideline for the diagnosis and treatment of takayasu’s arteritis (2023). Rheumatology and Immunology Research, 5:5-26, Mar 2024. URL: https://doi.org/10.1515/rir-2024-0002, doi:10.1515/rir-2024-0002. This article has 14 citations.
(arita2024currentimmunosuppressivetreatment pages 5-5): Yoh Arita, Tomohiko Ishibashi, and Yoshikazu Nakaoka. Current immunosuppressive treatment for takayasu arteritis. Circulation Journal, 88:1605-1609, Sep 2024. URL: https://doi.org/10.1253/circj.cj-23-0780, doi:10.1253/circj.cj-23-0780. This article has 11 citations and is from a peer-reviewed journal.
(NCT04300686 chunk 3): A Pilot Study in Severe Patients With Takayasu Arteritis.. Shanghai Zhongshan Hospital. 2020. ClinicalTrials.gov Identifier: NCT04300686
(bhandari2023pathophysiologydiagnosisand pages 9-9): Sagar Bhandari, Samia Rauf R Butt, Anzal Ishfaq, Mohamed H Attaallah, Chukwuyem Ekhator, Raghu Halappa Nagaraj, Asmita Mulmi, Muhammad Kamran, Amanda Karski, Karla I Vargas, Slobodan Lazarevic, Mohammad Uzair Zaman, Gautham Lakshmipriya Vetrivendan, S M Iram Shahzed, Archana Das, Vikas Yadav, Sophia B Bellegarde, and Ashraf Ullah. Pathophysiology, diagnosis, and management of takayasu arteritis: a review of current advances. Cureus, Jul 2023. URL: https://doi.org/10.7759/cureus.42667, doi:10.7759/cureus.42667. This article has 57 citations.
(bhandari2023pathophysiologydiagnosisand pages 9-10): Sagar Bhandari, Samia Rauf R Butt, Anzal Ishfaq, Mohamed H Attaallah, Chukwuyem Ekhator, Raghu Halappa Nagaraj, Asmita Mulmi, Muhammad Kamran, Amanda Karski, Karla I Vargas, Slobodan Lazarevic, Mohammad Uzair Zaman, Gautham Lakshmipriya Vetrivendan, S M Iram Shahzed, Archana Das, Vikas Yadav, Sophia B Bellegarde, and Ashraf Ullah. Pathophysiology, diagnosis, and management of takayasu arteritis: a review of current advances. Cureus, Jul 2023. URL: https://doi.org/10.7759/cureus.42667, doi:10.7759/cureus.42667. This article has 57 citations.