Primary polyarteritis nodosa is idiopathic, non-hepatitis B-related systemic polyarteritis nodosa, a rare necrotizing vasculitis that predominantly targets medium-sized arteries. Vessel-wall inflammation can cause angiographic or biopsy-confirmed arterial injury, downstream ischemia, and heterogeneous organ involvement ranging from limited disease to polyvisceral failure.
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name: Primary Polyarteritis Nodosa
creation_date: "2026-05-05T11:41:54Z"
updated_date: "2026-05-05T11:41:54Z"
description: >-
Primary polyarteritis nodosa is idiopathic, non-hepatitis B-related systemic
polyarteritis nodosa, a rare necrotizing vasculitis that predominantly
targets medium-sized arteries. Vessel-wall inflammation can cause
angiographic or biopsy-confirmed arterial injury, downstream ischemia, and
heterogeneous organ involvement ranging from limited disease to polyvisceral
failure.
category: Complex
disease_term:
preferred_term: primary polyarteritis nodosa
term:
id: MONDO:0018593
label: primary polyarteritis nodosa
parents:
- Vascular disorder
synonyms:
- Primary PAN
- Primary periarteritis nodosa
pathophysiology:
- name: Necrotizing medium-artery inflammation
description: >-
Transmural inflammatory injury affects medium-sized muscular arteries,
damaging the vessel wall and promoting fibrinoid necrosis, aneurysm,
stenosis, thrombosis, or other ischemia-producing vascular lesions.
cell_types:
- preferred_term: endothelial cell
term:
id: CL:0000115
label: endothelial cell
- preferred_term: macrophage
term:
id: CL:0000235
label: macrophage
- preferred_term: T cell
term:
id: CL:0000084
label: T 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
downstream:
- target: Arterial ischemic organ injury
description: Medium-artery inflammation can narrow, thrombose, or weaken vessels, leading to tissue ischemia and organ-specific PAN manifestations.
evidence:
- reference: PMID:33569653
reference_title: Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries.
explanation: This directly defines PAN as necrotizing inflammation of medium-sized arteries.
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly targeting medium-sized arteries.
explanation: This independently supports the core medium-artery necrotizing vasculitis mechanism.
- name: Idiopathic immune-mediated vascular injury
description: >-
In primary or idiopathic PAN, the specific initiating mechanism remains
uncertain, but clinical response to immunosuppression supports an immune
contribution to vessel-wall inflammation.
biological_processes:
- preferred_term: immune response
modifier: ABNORMAL
term:
id: GO:0006955
label: immune response
downstream:
- target: Necrotizing medium-artery inflammation
description: Immune-mediated inflammatory pathways sustain necrotizing medium-artery injury in idiopathic PAN.
evidence:
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: PARTIAL
evidence_source: HUMAN_CLINICAL
snippet: >-
The pathogenesis of "idiopathic PAN" remains enigmatic, although the clinical responses to immunosuppressive therapy support the concept that immunological mechanisms play an active pathogenic role.
explanation: This supports an immune-mediated contribution while preserving the source's stated uncertainty.
- name: Arterial ischemic organ injury
description: >-
Vascular lesions in medium arteries can produce single-organ disease,
cutaneous PAN, or severe systemic disease with polyvisceral failure.
evidence:
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The spectrum of disease ranges from involving a single organ to polyvisceral failure.
explanation: This links PAN vascular injury to organ involvement severity.
histopathology:
- name: Arterial wall leukocyte infiltrate
diagnostic: true
description: >-
Biopsy may show granulocyte or mixed leukocyte infiltration in arterial
walls, supporting the diagnosis of polyarteritis nodosa.
finding_term:
preferred_term: Arterial wall leukocyte infiltrate
evidence:
- reference: PMID:1975174
reference_title: The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
presence of granulocyte or mixed leukocyte infiltrate in an arterial wall on biopsy.
explanation: This ACR classification criterion supports arterial-wall leukocyte infiltration as a diagnostic histopathologic finding.
phenotypes:
- category: Cardiovascular
name: Necrotizing medium-vessel vasculitis
diagnostic: true
description: Necrotizing inflammation of medium-sized arteries is the defining diagnostic vascular lesion.
phenotype_term:
preferred_term: Medium-vessel vasculitis
term:
id: HP:0002633
label: Vasculitis
evidence:
- reference: PMID:33569653
reference_title: Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries.
explanation: This supports necrotizing medium-vessel vasculitis as the core phenotype.
- category: Multisystem
name: Polyvisceral involvement
description: Systemic PAN may involve a single organ or progress to polyvisceral failure.
phenotype_term:
preferred_term: Polyvisceral involvement
evidence:
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The spectrum of disease ranges from involving a single organ to polyvisceral failure.
explanation: This directly supports broad organ involvement in PAN.
- category: Constitutional
name: Fever
description: Fever is a general symptom in PAN.
phenotype_term:
preferred_term: Fever
term:
id: HP:0001945
label: Fever
evidence:
- reference: DOI:10.3390/ijms242316668
reference_title: "Polyarteritis Nodosa: Old Disease, New Etiologies"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The clinical presentation is heterogeneous and characterized by a significant number of patients exhibiting general symptoms, including asthenia, fever, and unintended weight loss.
explanation: This review lists fever among general PAN symptoms.
- category: Constitutional
name: Unintended weight loss
description: Unintended weight loss is a general symptom in PAN.
phenotype_term:
preferred_term: Weight loss
term:
id: HP:0001824
label: Weight loss
evidence:
- reference: DOI:10.3390/ijms242316668
reference_title: "Polyarteritis Nodosa: Old Disease, New Etiologies"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The clinical presentation is heterogeneous and characterized by a significant number of patients exhibiting general symptoms, including asthenia, fever, and unintended weight loss.
explanation: This review lists unintended weight loss among general PAN symptoms.
- category: Dermatologic
name: Cutaneous polyarteritis nodosa
description: Cutaneous PAN is a recognized clinical variant of the disease.
phenotype_term:
preferred_term: Cutaneous polyarteritis nodosa
evidence:
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
In addition to the systemic idiopathic form, called "idiopathic generalized PAN," there are 2 clinical variants of this disease: "cutaneous PAN" and "hepatitis B virus (HBV)-associated PAN".
explanation: This supports cutaneous PAN as a recognized clinical form; the primary page focuses on idiopathic/non-HBV disease.
- category: Dermatologic
name: Skin involvement
description: PAN preferentially affects the skin among organ systems.
phenotype_term:
preferred_term: Abnormality of the skin
term:
id: HP:0000951
label: Abnormality of the skin
evidence:
- reference: DOI:10.3390/ijms242316668
reference_title: "Polyarteritis Nodosa: Old Disease, New Etiologies"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract.
explanation: This supports skin involvement as a common organ-system phenotype in PAN.
- category: Neurological
name: Nervous system involvement
description: PAN preferentially affects the nervous system among organ systems.
phenotype_term:
preferred_term: Nervous system involvement
term:
id: HP:0000707
label: Abnormality of the nervous system
evidence:
- reference: DOI:10.3390/ijms242316668
reference_title: "Polyarteritis Nodosa: Old Disease, New Etiologies"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract.
explanation: This supports nervous-system involvement in PAN.
- category: Gastrointestinal
name: Gastrointestinal tract involvement
description: PAN preferentially affects the gastrointestinal tract among organ systems.
phenotype_term:
preferred_term: Gastrointestinal tract involvement
evidence:
- reference: DOI:10.3390/ijms242316668
reference_title: "Polyarteritis Nodosa: Old Disease, New Etiologies"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract.
explanation: This supports gastrointestinal involvement in PAN.
- category: Neurological
name: Peripheral neuropathy
description: Mononeuropathy or polyneuropathy is a characteristic neurologic manifestation included in PAN classification criteria.
phenotype_term:
preferred_term: Peripheral neuropathy
term:
id: HP:0009830
label: Peripheral neuropathy
evidence:
- reference: PMID:1975174
reference_title: The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
mononeuropathy or polyneuropathy
explanation: This classification criterion supports peripheral neuropathy as a PAN manifestation.
- category: Musculoskeletal
name: Myalgia
description: Myalgias are included among PAN classification criteria and are a common musculoskeletal manifestation.
phenotype_term:
preferred_term: Myalgia
term:
id: HP:0003326
label: Myalgia
evidence:
- reference: PMID:1975174
reference_title: The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
myalgias
explanation: This supports myalgia as a PAN classification feature.
- category: Cardiovascular
name: Hypertension
description: Elevated diastolic blood pressure can reflect renal vascular involvement and is included in PAN classification criteria.
phenotype_term:
preferred_term: Hypertension
term:
id: HP:0000822
label: Hypertension
evidence:
- reference: PMID:1975174
reference_title: The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
diastolic blood pressure greater than 90 mm Hg
explanation: This classification criterion supports hypertension as a PAN feature.
- category: Laboratory
name: ANCA negativity and absence of glomerulonephritis
description: >-
PAN is distinguished from other vasculitides by absence of granulomas,
glomerulonephritis, and anti-neutrophil cytoplasmic antibodies.
phenotype_term:
preferred_term: Anti-neutrophil cytoplasmic antibody negative
evidence:
- reference: DOI:10.3390/ijms242316668
reference_title: "Polyarteritis Nodosa: Old Disease, New Etiologies"
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
The absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies serves to distinguish PAN from other types of vasculitis.
explanation: This supports ANCA negativity and absent glomerulonephritis as distinguishing diagnostic features.
- category: Cardiovascular
name: Angiographic vascular abnormality
diagnostic: true
description: Angiographic findings are part of diagnostic integration for PAN.
phenotype_term:
preferred_term: Angiographic vascular abnormality
evidence:
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Diagnosis requires the integration of clinical, angiographic, and biopsy findings.
explanation: This supports angiographic vascular findings as part of PAN diagnosis.
diagnosis:
- name: Clinical, angiographic, and biopsy integration
diagnosis_term:
preferred_term: diagnostic imaging
description: >-
Primary PAN diagnosis integrates compatible clinical features with
angiographic vascular findings and tissue biopsy when available, while
distinguishing idiopathic disease from hepatitis B-associated PAN.
results: >-
Angiographic and biopsy findings supporting systemic non-HBV PAN establish
the vascular diagnosis in the appropriate clinical context.
evidence:
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Diagnosis requires the integration of clinical, angiographic, and biopsy findings.
explanation: This review states the diagnostic integration approach.
- reference: PMID:34235889
reference_title: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis.
explanation: This guideline supports imaging and tissue biopsy for PAN diagnosis.
treatments:
- name: Glucocorticoids and cyclophosphamide for severe primary PAN
description: >-
Severe idiopathic or non-HBV-related PAN is treated early with
glucocorticoids plus cyclophosphamide, with efforts to limit long-term
toxicity.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: cyclophosphamide
term:
id: CHEBI:4027
label: cyclophosphamide
- preferred_term: glucocorticoid
term:
id: CHEBI:24261
label: glucocorticoid
evidence:
- reference: PMID:34235889
reference_title: 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis.
explanation: This guideline supports early cyclophosphamide and glucocorticoid treatment for severe systemic non-HBV PAN.
- reference: PMID:26884100
reference_title: "Polyarteritis nodosa: A contemporary overview."
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Idiopathic generalized PAN should be treated with a combination of glucocorticoids and cyclophosphamide.
explanation: This review specifically supports glucocorticoid plus cyclophosphamide therapy for idiopathic generalized PAN.
- name: Subtype- and severity-guided immunomodulatory therapy
description: >-
Primary PAN therapy is individualized by subtype and severity, using
glucocorticoids, conventional immunomodulatory agents, and selected biologic
therapies when appropriate.
treatment_term:
preferred_term: Pharmacotherapy
term:
id: NCIT:C15986
label: Pharmacotherapy
therapeutic_agent:
- preferred_term: glucocorticoid
term:
id: CHEBI:24261
label: glucocorticoid
- preferred_term: cyclophosphamide
term:
id: CHEBI:4027
label: cyclophosphamide
- preferred_term: tumor necrosis factor inhibitor
- preferred_term: interleukin 6 inhibitor
evidence:
- reference: PMID:33569653
reference_title: Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.
supports: SUPPORT
evidence_source: HUMAN_CLINICAL
snippet: >-
Idiopathic and hereditary variants of polyarteritis nodosa are treated with immunomodulatory medications such as glucocorticoids, conventional immunomodulatory drugs (e.g., cyclophosphamide) and biologic agents (e.g., tumor necrosis factor inhibitors, interleukin 6 inhibitor), while hepatitis B virus-associated polyarteritis nodosa primarily requires antiviral therapy combined with plasma exchange.
explanation: This supports immunomodulatory therapy for idiopathic PAN and distinguishes it from HBV-associated PAN.
Primary polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis that predominantly targets medium-sized arteries and is classically distinguished from ANCA-associated vasculitis by absence of glomerulonephritis and typical ANCA negativity, with pathology showing segmental, transmural necrotizing arteritis with fibrinoid necrosis and without granulomas. (wolff2023polyarteritisnodosaold pages 1-2, chung20212021americancollegea pages 1-2, puechal2022polyarteritisnodosastate pages 1-2)
A major 2023–2024 development is the ongoing “re-framing” of what was historically called idiopathic PAN into (i) truly primary/idiopathic disease and (ii) PAN phenotypes driven by identifiable causes or mimics (e.g., HBV-associated PAN, monogenic ADA2 deficiency/DADA2, VEXAS), with important treatment implications (etiology-directed therapy rather than uniform immunosuppression). (wolff2023polyarteritisnodosaold pages 2-3, wolff2023polyarteritisnodosareviewed pages 8-9)
The following table consolidates key evidence items and quantitative data for rapid curation.
| Domain | Key points | Quantitative data | Key sources (with year, DOI/URL if known) |
|---|---|---|---|
| Identifiers / definitions | Primary polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of predominantly medium-sized arteries; diagnosis/classification emphasizes absence of glomerulonephritis, granulomas, and usually ANCA. CHCC 2012/overview definitions separate PAN from microscopic polyangiitis and other small-vessel vasculitides. ACR/VF 2021 guideline focuses on systemic, non-HBV PAN. | CHCC wording summarized as necrotizing inflammation of medium/small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, venules; ACR/VF guideline issued 16 recommendations + 1 ungraded statement. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Jennette, 2013, https://doi.org/10.1007/s10157-013-0869-6; Chung et al., 2021, https://doi.org/10.1002/acr.24633 (wolff2023polyarteritisnodosaold pages 1-2, chung20212021americancollegea pages 1-2, hocevar2021clinicalapproachto pages 1-2) |
| Key distinguishing features | Distinguished from AAV/MPA by lack of pauci-immune glomerulonephritis and usual ANCA negativity; histology shows segmental, transmural necrotizing arteritis with fibrinoid necrosis and no granulomas; angiography may show aneurysms/stenoses of mesenteric, hepatic, and renal arteries. | ACR 1990 criteria require >=3/10 items for classification. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2; Puéchal, 2022, https://doi.org/10.1016/j.jbspin.2021.105320 (wolff2023polyarteritisnodosaold pages 2-3, chung20212021americancollegea pages 1-2, hocevar2021clinicalapproachto pages 5-6, puechal2022polyarteritisnodosastate pages 1-2) |
| Epidemiology | PAN is rare in Europe and has become less common since reduction of HBV-related disease; contemporary idiopathic PAN is considered uncommon. Adult cohorts show middle age at onset and male predominance. | Prevalence in Europe about 2-31 per million; mean age ~51-54 years (one cohort 53.6 ± 18 years); male:female ratio ~1.5-1.7. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Walter et al., 2024, https://doi.org/10.1186/s12872-024-03841-y (wolff2023polyarteritisnodosaold pages 1-2, wolff2023polyarteritisnodosaold pages 2-3, walter2024shiftingperspectivesin pages 7-9) |
| Etiologies / associations: HBV | Historically a major cause; pathogenesis linked to HBsAg-containing immune complexes. Modern vaccination/public-health measures markedly reduced HBV-PAN frequency. HBV-associated PAN is treated differently from primary PAN. | Historical importance high; no current pooled rate given in extracted evidence. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2; Puéchal, 2022, https://doi.org/10.1016/j.jbspin.2021.105320 (wolff2023polyarteritisnodosaold pages 2-3, wolff2023polyarteritisnodosareviewed pages 8-9, hocevar2021clinicalapproachto pages 1-2, puechal2022polyarteritisnodosastate pages 1-2) |
| Etiologies / associations: HCV, HIV, parvovirus B19 | HCV has been reported but is uncommon in PAN; HIV has occasional associations; parvovirus B19 has been investigated without convincing enrichment versus controls in one PCR-based study. | HCV association estimated at ~5% of PAN patients in reviewed evidence. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (wolff2023polyarteritisnodosaold pages 2-3) |
| Etiologies / associations: drugs / vaccines | Drug-induced PAN-like disease reported, including minocycline; post-COVID-19 vaccination PAN/cPAN-like cases have been reported, but causal inference remains limited. | No robust incidence estimate available in extracted evidence. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (wolff2023polyarteritisnodosaold pages 2-3) |
| Etiologies / associations: malignancy | PAN can occur as a paraneoplastic/associated vasculitis, especially with hematologic disease including myelodysplastic syndrome (MDS); this is important in “secondary” PAN differential diagnosis. | In one series of 70 patients with MDS and vasculitis, 9% had PAN. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (wolff2023polyarteritisnodosaold pages 2-3) |
| Etiologies / associations: DADA2 / ADA2 | Deficiency of ADA2 (CECR1/ADA2) is a key monogenic PAN mimic/cause, especially in early-onset disease; testing is important in childhood and young-adult PAN or unusual phenotypes. Anti-TNF therapy is favored in DADA2. | >300 cases reported since 2014; 4.3% of 118 adults with idiopathic PAN had biallelic ADA2 pathogenic variants; in DADA2, ~85% manifest before age 12, mucocutaneous involvement ~75%, livedo reticularis ~50%, neurologic involvement ~51%, ischemic stroke ~27%. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2 (wolff2023polyarteritisnodosaold pages 1-2, wolff2023polyarteritisnodosaold pages 2-3, wolff2023polyarteritisnodosaold pages 7-8, hocevar2021clinicalapproachto pages 5-6, hocevar2021clinicalapproachto pages 1-2) |
| Etiologies / associations: VEXAS, FMF, SAVI | VEXAS (UBA1 somatic mutations), FMF-associated PAN, and SAVI/interferonopathy-related PAN-like disease broaden PAN nosology and are key differentials in “primary” PAN workup. | VEXAS inaugural cohort: 3/12 met PAN criteria; FMF-associated PAN prevalence ~0.9%. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (wolff2023polyarteritisnodosaold pages 2-3) |
| Clinical phenotypes | Heterogeneous multisystem disease with constitutional symptoms plus skin, peripheral nerve, GI, renal vascular, musculoskeletal, cardiac, CNS, and gonadal involvement. Orchitis/testicular pain is uncommon but relatively specific; coronary involvement may occur. | >90% systemic symptoms in meta-analysis; fever ~52%, myalgia ~53%, cutaneous involvement ~56%; muscle biopsy diagnostic in up to 50%; cutaneous and myalgia common among relapsers. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Taprantzis et al., 2026, https://doi.org/10.1007/s00296-026-06082-8; Walter et al., 2024, https://doi.org/10.1186/s12872-024-03841-y (wolff2023polyarteritisnodosaold pages 1-2, wolff2023polyarteritisnodosareviewed pages 7-8, taprantzis2026clinicalmanifestationsprognostic pages 1-2, walter2024shiftingperspectivesin pages 7-9) |
| Diagnostics: biopsy | Tissue biopsy is the diagnostic gold standard when feasible. Recommended targets are symptomatic tissues (deep skin/subcutis, muscle, sural nerve; combined nerve+muscle biopsy if neuropathy). Deep skin biopsy is preferred over superficial punch because medium-sized vessels lie deeper. | Histology: segmental necrotizing transmural inflammation with fibrinoid necrosis; muscle biopsy positive in up to 50% of cases. | Chung et al., 2021, https://doi.org/10.1002/acr.24633; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2; Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (chung20212021americancollegea pages 5-6, chung20212021americancollegea pages 1-2, hocevar2021clinicalapproachto pages 5-6, wolff2023polyarteritisnodosareviewed pages 7-8) |
| Diagnostics: angiography / imaging | Angiography is used when biopsy is not available or unsafe. Conventional angiography remains highest-resolution standard; CTA may better visualize distal mesenteric branches; MRA is an alternative when iodinated contrast is undesirable. Angiography can also define disease extent and detect aneurysms. | Typical findings: saccular/fusiform aneurysms and stenotic lesions in mesenteric, hepatic, renal arteries. | Chung et al., 2021, https://doi.org/10.1002/acr.24633; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2; Puéchal, 2022, https://doi.org/10.1016/j.jbspin.2021.105320 (chung20212021americancollegea pages 5-6, chung20212021americancollegea pages 1-2, hocevar2021clinicalapproachto pages 5-6, puechal2022polyarteritisnodosastate pages 1-2) |
| Diagnostics: labs / classification criteria | No specific serologic biomarker exists for primary PAN. ANCA is typically absent and its presence should prompt reconsideration of AAV/MPA. ACR 1990 criteria remain widely cited but were developed before MPA separation; EMA algorithm plus CHCC definition improves classification. | ACR 1990 sensitivity 82.2%, specificity 86.6%; later performance lower after MPA separation (sensitivity reportedly fell to 40.6% in re-evaluation). | Lin et al., 2021, https://doi.org/10.1002/acr2.11189; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2; Puéchal, 2022, https://doi.org/10.1016/j.jbspin.2021.105320; Walter et al., 2024, https://doi.org/10.1186/s12872-024-03841-y (lin2021polyarteritisnodosaa pages 1-2, hocevar2021clinicalapproachto pages 5-6, puechal2022polyarteritisnodosastate pages 2-3, walter2024shiftingperspectivesin pages 7-9) |
| Prognosis / Five-Factor Score | Prognosis is commonly stratified using the Five-Factor Score (FFS), which informs intensity of induction therapy. Poor-prognosis organ involvement drives morbidity and mortality. | FFS items: proteinuria >1 g/day, creatinine >140 µmol/L, cardiomyopathy, severe GI involvement, CNS involvement (1 point each). Meta-analysis: relapse ~27%, mortality ~13%, remission ~65%. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668; Taprantzis et al., 2026, https://doi.org/10.1007/s00296-026-06082-8 (wolff2023polyarteritisnodosareviewed pages 7-8, wolff2023polyarteritisnodosaold pages 7-8, taprantzis2026clinicalmanifestationsprognostic pages 1-2) |
| Treatment by severity: mild / FFS 0 | For newly diagnosed nonsevere PAN, glucocorticoids are often effective, but guidelines and reviews increasingly favor adding a nonglucocorticoid immunosuppressant (AZA or MTX) to reduce relapse/steroid exposure. | GC monotherapy associated with relapse concern; review cites ~40% relapse rate for mild PAN treated with GC alone. ACR/VF suggests AZA 2-3 mg/kg/day or MTX 0.3 mg/kg/week as options. | Chung et al., 2021, https://doi.org/10.1002/acr.24633; Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (chung20212021americancollegea pages 5-6, wolff2023polyarteritisnodosareviewed pages 7-8, wolff2023polyarteritisnodosaold pages 7-8) |
| Treatment by severity: severe / FFS >0 | Severe or organ-/life-threatening PAN is treated with pulse IV glucocorticoids or high-dose oral glucocorticoids plus cyclophosphamide; rituximab is not preferred first-line because evidence is sparse. If cyclophosphamide cannot be used, AZA/MTX may be combined with glucocorticoids rather than GC alone. | Induction typically 3-6 months; prednisone about 1 mg/kg/day (max 60 mg/day); cyclophosphamide usually limited to <=6 months. One cited comparison found 12 monthly cyclophosphamide doses superior to a shorter 6-dose strategy for survival (HR 0.44) and sustained remission (HR 0.34) at 32 months. | Chung et al., 2021, https://doi.org/10.1002/acr.24633; Walter et al., 2024, https://doi.org/10.1186/s12872-024-03841-y; Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (chung20212021americancollegea pages 5-6, walter2024shiftingperspectivesin pages 7-9, wolff2023polyarteritisnodosareviewed pages 7-8, wolff2023polyarteritisnodosaold pages 7-8) |
| Maintenance therapy | After remission induction, cyclophosphamide should be transitioned to a less toxic maintenance agent such as azathioprine or methotrexate; maintenance generally continues 12-18 months, with some guidance allowing discontinuation after ~18 months of sustained remission. | Maintenance duration commonly 12-18 months; French recommendations for systemic necrotizing vasculitides describe maintenance 12-48 months depending on context. | Chung et al., 2021, https://doi.org/10.1002/acr.24633; Terrier et al., 2020, https://doi.org/10.1186/s13023-020-01621-3; Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (chung20212021americancollegea pages 5-6, wolff2023polyarteritisnodosareviewed pages 8-9, wolff2023polyarteritisnodosaold pages 7-8) |
| Refractory / relapsing disease | Biologics are reserved for refractory/relapsing PAN or specific molecular subtypes. Evidence is limited to retrospective cohorts and case reports; etiology-directed therapy is increasingly emphasized (anti-TNF for DADA2; JAK inhibition/tocilizumab in selected VEXAS; disease-specific therapy for malignancy-associated PAN). | European retrospective series complete remission: tocilizumab 50%, TNF inhibitors 40%, rituximab 33%. | Wolff et al., 2023, https://doi.org/10.3390/ijms242316668 (wolff2023polyarteritisnodosareviewed pages 7-8, wolff2023polyarteritisnodosareviewed pages 8-9, wolff2023polyarteritisnodosaold pages 7-8) |
| HBV-PAN treatment | HBV-associated PAN is managed differently from primary PAN: antiviral therapy is central, with short-course glucocorticoids and plasma exchange used in severe disease. Classic older trial data did not show benefit of plasma exchange added to steroids alone in non-HBV PAN/CSS, underscoring etiology-specific use. | Older randomized trial in PAN/CSS: no superiority of steroids + plasma exchange over steroids alone; HBV-PAN recommendations are based largely on uncontrolled studies/guidelines rather than modern RCTs. | Guillevin et al., 1992, https://doi.org/10.1002/art.1780350214; Hočevar et al., 2021, https://doi.org/10.1007/s11926-021-00983-2; Puéchal, 2022, https://doi.org/10.1016/j.jbspin.2021.105320 (hocevar2021clinicalapproachto pages 1-2, puechal2022polyarteritisnodosastate pages 1-2) |
Table: This table consolidates core disease-characteristic evidence for primary polyarteritis nodosa, including definition, epidemiology, etiologic associations, phenotypes, diagnostic criteria, prognosis, and treatment. It is designed as a compact evidence map for knowledge-base curation and report drafting.
Definition (CHCC concept and modern reviews): PAN is defined as necrotizing inflammation of medium-sized (and sometimes small muscular) arteries, without glomerulonephritis and without vasculitis of arterioles/capillaries/venules. (puechal2022polyarteritisnodosastate pages 1-2)
Guideline clinical framing (ACR/Vasculitis Foundation 2021): PAN is a systemic necrotizing vasculitis of medium-sized vessels; typical clinical patterns include constitutional symptoms plus cutaneous, neurologic (peripheral neuropathy/mononeuritis multiplex), gastrointestinal, and renovascular manifestations; diagnosis is generally confirmed by biopsy of an affected organ or by angiography when biopsy cannot be obtained. (chung20212021americancollegea pages 1-2)
ICD-10: Polyarteritis nodosa is commonly coded as M30.0 (standard clinical coding; not directly extracted from retrieved texts).
MeSH / MONDO / Orphanet: A specific MONDO ID and ORPHAcode for “primary/idiopathic systemic PAN” were not retrievable from the tool-accessible corpus in this run; knowledge base ingestion should pull these directly from MONDO and Orphanet inventories. (Evidence gap; see “Limitations”.)
Current understanding is derived from a mixture of (i) cohort/registry experience and guideline syntheses (ACR/VF 2021; French recommendations), (ii) older randomized trials in systemic necrotizing vasculitides, (iii) observational studies and case series in refractory disease, and (iv) increasingly, genotype-first studies that identify monogenic causes/mimics in “idiopathic” cohorts. (chung20212021americancollegea pages 5-6, wolff2023polyarteritisnodosaold pages 2-3)
Primary (idiopathic) PAN is diagnosed after excluding secondary causes (notably HBV) and important mimics/phenocopies (e.g., DADA2/ADA2 deficiency; VEXAS). Modern reviews emphasize that PAN is now better viewed as a spectrum where identifying an underlying driver changes management. (wolff2023polyarteritisnodosaold pages 2-3, wolff2023polyarteritisnodosareviewed pages 8-9)
Drug-induced PAN-like cases (e.g., minocycline) and post–COVID-19 vaccination temporal associations have been reported, but population-level causality is not established in the extracted evidence. (wolff2023polyarteritisnodosaold pages 2-3)
PAN may occur in association with hematologic malignancy, including myelodysplastic syndrome (MDS); in one referenced series, among 70 patients with MDS and vasculitis, 9% presented with PAN. (wolff2023polyarteritisnodosaold pages 2-3)
No robust protective genetic or environmental factors were identified in the tool-accessible evidence.
Not specifically established in the retrieved evidence; a plausible interaction is genetic predisposition (e.g., ADA2 deficiency) plus infectious triggers, but this remains incompletely defined here. (wolff2023polyarteritisnodosaold pages 2-3)
PAN can involve virtually any organ, with preferential involvement of skin, peripheral nervous system, and GI tract in many series/reviews. (wolff2023polyarteritisnodosaold pages 1-2)
Constitutional/systemic - Fever, asthenia/fatigue, weight loss (e.g., HP:0001945 Fever; HP:0001254 Lethargy/fatigue; HP:0001824 Weight loss). (wolff2023polyarteritisnodosaold pages 1-2, taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Cutaneous - Livedo reticularis/racemosa (HP:0000973), subcutaneous nodules (HP:0001480), skin ulcers (HP:0001053), palpable purpura (HP:0000964). (chung20212021americancollegea pages 1-2, taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Peripheral nervous system - Peripheral neuropathy and mononeuritis multiplex (HP:0009830 Peripheral neuropathy; HP:0001305 Mononeuritis multiplex). (chung20212021americancollegea pages 1-2, taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Gastrointestinal/mesenteric ischemia - Abdominal pain (HP:0002027), bowel ischemia/perforation/bleeding (HP:0031944 Intestinal ischemia; HP:0004395 Gastrointestinal hemorrhage). (taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Renovascular - Hypertension (HP:0000822), renal artery stenosis/aneurysm with hematuria/proteinuria (HP:0000790 Hematuria; HP:0000093 Proteinuria). (taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Musculoskeletal - Myalgia (HP:0003326), arthralgia (HP:0002829). (taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Cardiac - Coronary arteritis/stenosis/aneurysm leading to myocardial ischemia or heart failure (e.g., HP:0001658 Myocardial infarction; HP:0001635 Congestive heart failure). (wolff2023polyarteritisnodosaold pages 1-2, walter2024shiftingperspectivesin pages 7-9)
Genitourinary - Orchitis/testicular pain (HP:0000035 Orchitis; HP:0030547 Testicular pain). Orchitis is described as rare but relatively specific for PAN. (wolff2023polyarteritisnodosaold pages 1-2, wolff2023polyarteritisnodosareviewed pages 7-8)
A 2026 systematic review/meta-analysis (not within 2023–2024 but recent and quantitative) reported: fever ~52%, myalgia ~53%, cutaneous involvement ~56%, relapse ~27%, mortality ~13%, remission ~65%, and >90% with systemic manifestations. (taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Direct validated QoL instruments (SF-36/EQ-5D) were not extractable from the retrieved texts; however, PAN is consistently framed as potentially disabling due to neuropathy, ischemic complications, and treatment toxicity. (chung20212021americancollegea pages 1-2)
Primary/idiopathic PAN is generally not monogenic, but a major modern development is recognition that a subset of cases labeled “idiopathic PAN” actually reflect monogenic vasculitis/vasculopathy, especially ADA2 (CECR1/ADA2) deficiency. (wolff2023polyarteritisnodosaold pages 2-3)
Suggested molecular diagnostic actions (MAXO-style actions, illustrative): - ADA2 enzyme activity assay (MAXO:0000115 “enzyme activity measurement”) - CECR1/ADA2 sequencing (MAXO:0000127 “gene sequencing”) (hocevar2021clinicalapproachto pages 5-6)
No PAN-specific epigenetic or multi-omics profiling signatures were identified in the retrieved evidence.
No robust toxin/pollution/occupational exposures were identified. The dominant “environmental” component supported here is infectious association (HBV; occasional HCV/HIV reports). (wolff2023polyarteritisnodosaold pages 2-3)
Histopathology features emphasized in modern reviews include segmental, necrotizing, transmural arteritis with fibrinoid necrosis, microaneurysms, and mixed inflammatory infiltrates, typically without granulomas. (wolff2023polyarteritisnodosaold pages 2-3, chung20212021americancollegea pages 1-2)
Cytokine elevations and immune cell infiltrates reported in a 2023 review include IFN-α, IL-2, TNFα, and IL-1β, with macrophage and T-cell involvement in lesions. (wolff2023polyarteritisnodosaold pages 2-3)
Upstream trigger (often unknown in primary PAN; or immune complexes in HBV-PAN; or intrinsic immune dysregulation in monogenic phenocopies) → medium-artery wall inflammation and fibrinoid necrosis → stenosis, thrombosis, aneurysm formation → tissue ischemia/hemorrhage in affected vascular territories → organ-specific manifestations (neuropathy, GI ischemia, renal ischemia/hypertension, skin ulcers/nodules, coronary events). (chung20212021americancollegea pages 1-2, wolff2023polyarteritisnodosaold pages 1-2)
GO biological process (suggested): - GO:0006954 inflammatory response - GO:0002682 regulation of immune system process - GO:0001525 angiogenesis (aneurysm/repair context) - GO:0007596 blood coagulation (thrombosis complications)
Cell Ontology (CL) (suggested): - Macrophage (CL:0000235) - T cell (CL:0000084) - Endothelial cell (CL:0000115) - Vascular smooth muscle cell (CL:0000192)
UBERON anatomy (suggested): - Medium-sized artery / muscular artery (e.g., UBERON:0001637 artery; more specific vessel terms should be selected per organ) - Mesenteric artery, renal artery, hepatic artery
(These ontology mappings are proposed based on described biology; no ontology IDs were explicitly enumerated in the retrieved texts.)
Primary: medium-sized arteries, especially visceral arterial branches (mesenteric, hepatic, renal) with aneurysms/stenoses; skin and peripheral nerve involvement are common. (chung20212021americancollegea pages 1-2, wolff2023polyarteritisnodosaold pages 1-2)
Secondary/complications: mesenteric infarction/hemorrhage, cardiac ischemia, cerebrovascular events, renal ischemia/hypertension. (wolff2023polyarteritisnodosaold pages 1-2, walter2024shiftingperspectivesin pages 7-9)
Typical presentation is adult-onset in many cohorts (mean ~early 50s) with male predominance, though monogenic mimics (DADA2) often present in childhood. (wolff2023polyarteritisnodosaold pages 1-2, wolff2023polyarteritisnodosaold pages 7-8)
Disease course may be relapsing, with relapse estimates around ~27% in pooled data. (taprantzis2026clinicalmanifestationsprognostic pages 1-2)
Reported European prevalence range in modern reviews is ~2–31 per million. (wolff2023polyarteritisnodosaold pages 1-2)
Demographics in a contemporary cohort cited in a 2023 review: mean age 53.6 ± 18 years, male predominance with sex ratio ~1.5. (wolff2023polyarteritisnodosaold pages 2-3)
ACR 1990 PAN classification criteria: ≥3 of 10 items yields sensitivity 82.2% and specificity 86.6% for classification; limitations recognized because criteria predate separation of microscopic polyangiitis. (walter2024shiftingperspectivesin pages 7-9, puechal2022polyarteritisnodosastate pages 2-3)
CHCC definition (quoted in a 2022 state-of-the-art review): “Necrotising inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules.” (puechal2022polyarteritisnodosastate pages 1-2)
There is no specific serologic biomarker for primary PAN; ANCA negativity supports PAN classification, and ANCA positivity should raise suspicion for AAV/MPA rather than PAN. (lin2021polyarteritisnodosaa pages 1-2, puechal2022polyarteritisnodosastate pages 1-2)
Angiography may show saccular/fusiform aneurysms and stenoses in mesenteric/hepatic/renal arteries. Conventional angiography remains highest resolution; CTA and MRA are useful alternatives in specific contexts. (chung20212021americancollegea pages 5-6, chung20212021americancollegea pages 1-2)
Biopsy remains the diagnostic gold standard when feasible; deep skin biopsy is recommended over superficial punch when evaluating cutaneous disease, and combined nerve+muscle biopsy is recommended over nerve alone for suspected vasculitic neuropathy. (chung20212021americancollegea pages 5-6)
A treatment algorithm and classification/criteria table are shown in Puéchal (2022), including ACR criteria, CHCC definition, and the EMA classification approach, plus a clinical treatment flowchart stratified by subtype and severity. (puechal2022polyarteritisnodosastate media 85b1bd4a, puechal2022polyarteritisnodosastate media 858e4031)
The Five-Factor Score (FFS) assigns 1 point each for proteinuria >1 g/day, creatinine >140 µmol/L, cardiomyopathy, severe GI involvement, and CNS involvement to stratify risk and guide treatment intensity. (wolff2023polyarteritisnodosareviewed pages 7-8)
A recent meta-analysis estimated relapse ~27%, mortality ~13%, and remission ~65% across included PAN studies (heterogeneous definitions and eras). (taprantzis2026clinicalmanifestationsprognostic pages 1-2)
ACR/VF 2021 guideline (real-world implementation in rheumatology practice): - Severe PAN: cyclophosphamide + glucocorticoids preferred over glucocorticoids alone and over rituximab + glucocorticoids; IV pulse glucocorticoids are conditionally preferred over high-dose oral glucocorticoids for newly diagnosed severe disease. (chung20212021americancollegea pages 5-6) - Nonsevere PAN: glucocorticoids plus a non-glucocorticoid immunosuppressant (e.g., azathioprine or methotrexate) are conditionally preferred over glucocorticoids alone. (chung20212021americancollegea pages 5-6, walter2024shiftingperspectivesin pages 7-9)
Dose/duration patterns reported in a 2023 review: induction for moderate–severe PAN commonly uses prednisone ~1 mg/kg/day (max 60 mg/day) and cyclophosphamide for ~3–6 months, with cyclophosphamide generally limited to ≤6 months, followed by maintenance azathioprine or methotrexate for ~12–18 months. (wolff2023polyarteritisnodosareviewed pages 7-8)
Evidence remains limited (mostly retrospective cohorts/case series). A European retrospective series cited in a 2023 review reported complete remission rates of 50% (tocilizumab), 40% (TNF inhibitors), and 33% (rituximab) in relapsing/refractory PAN. (wolff2023polyarteritisnodosareviewed pages 7-8)
MAXO suggestions (treatments/actions; illustrative): - Glucocorticoid therapy (MAXO:0000747) - Cyclophosphamide therapy (MAXO:0001024) - Azathioprine therapy (MAXO:0000724) - Methotrexate therapy (MAXO:0001008) - Plasma exchange (MAXO:0000411)
Primary prevention of idiopathic PAN is not established. For secondary PAN risk reduction, HBV vaccination and HBV control plausibly reduce HBV-associated PAN burden; this is consistent with the reported historical decline in HBV-PAN. (lin2021polyarteritisnodosaa pages 1-2)
No naturally occurring PAN analogs in other species were identified in the retrieved evidence.
No validated animal models were identified in the retrieved evidence set.
References
(wolff2023polyarteritisnodosaold pages 1-2): Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, and Denis Comte. Polyarteritis nodosa: old disease, new etiologies. International Journal of Molecular Sciences, 24:16668, Nov 2023. URL: https://doi.org/10.3390/ijms242316668, doi:10.3390/ijms242316668. This article has 50 citations.
(chung20212021americancollegea pages 1-2): Sharon A. Chung, Mark Gorelik, Carol A. Langford, Mehrdad Maz, Andy Abril, Gordon Guyatt, Amy M. Archer, Doyt L. Conn, Kathy A. Full, Peter C. Grayson, Maria F. Ibarra, Lisa F. Imundo, Susan Kim, Peter A. Merkel, Rennie L. Rhee, Philip Seo, John H. Stone, Sangeeta Sule, Robert P. Sundel, Omar I. Vitobaldi, Ann Warner, Kevin Byram, Anisha B. Dua, Nedaa Husainat, Karen E. James, Mohamad Kalot, Yih Chang Lin, Jason M. Springer, Marat Turgunbaev, Alexandra Villa‐Forte, Amy S. Turner, and Reem A. Mustafa. 2021 american college of rheumatology/vasculitis foundation guideline for the management of polyarteritis nodosa. Arthritis Care & Research, 73:1061-1070, Jul 2021. URL: https://doi.org/10.1002/acr.24633, doi:10.1002/acr.24633. This article has 137 citations and is from a domain leading peer-reviewed journal.
(puechal2022polyarteritisnodosastate pages 1-2): Xavier Puéchal. Polyarteritis nodosa: state of the art. Joint Bone Spine, 89:105320, Jul 2022. URL: https://doi.org/10.1016/j.jbspin.2021.105320, doi:10.1016/j.jbspin.2021.105320. This article has 16 citations and is from a peer-reviewed journal.
(wolff2023polyarteritisnodosaold pages 2-3): Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, and Denis Comte. Polyarteritis nodosa: old disease, new etiologies. International Journal of Molecular Sciences, 24:16668, Nov 2023. URL: https://doi.org/10.3390/ijms242316668, doi:10.3390/ijms242316668. This article has 50 citations.
(wolff2023polyarteritisnodosareviewed pages 8-9): Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, and Denis Comte. Polyarteritis nodosa reviewed in 2023: old disease, new etiologies. Oct 2023. URL: https://doi.org/10.20944/preprints202310.0949.v1, doi:10.20944/preprints202310.0949.v1.
(hocevar2021clinicalapproachto pages 1-2): Alojzija Hočevar, Matija Tomšič, and Katja Perdan Pirkmajer. Clinical approach to diagnosis and therapy of polyarteritis nodosa. Current Rheumatology Reports, Feb 2021. URL: https://doi.org/10.1007/s11926-021-00983-2, doi:10.1007/s11926-021-00983-2. This article has 58 citations and is from a peer-reviewed journal.
(hocevar2021clinicalapproachto pages 5-6): Alojzija Hočevar, Matija Tomšič, and Katja Perdan Pirkmajer. Clinical approach to diagnosis and therapy of polyarteritis nodosa. Current Rheumatology Reports, Feb 2021. URL: https://doi.org/10.1007/s11926-021-00983-2, doi:10.1007/s11926-021-00983-2. This article has 58 citations and is from a peer-reviewed journal.
(walter2024shiftingperspectivesin pages 7-9): Dylan J. Walter, Grace E. Bigham, Steven Lahti, and Syed W. Haider. Shifting perspectives in coronary involvement of polyarteritis nodosa: case of 3-vessel occlusion treated with 4-vessel cabg and review of literature. BMC Cardiovascular Disorders, Apr 2024. URL: https://doi.org/10.1186/s12872-024-03841-y, doi:10.1186/s12872-024-03841-y. This article has 3 citations and is from a peer-reviewed journal.
(wolff2023polyarteritisnodosaold pages 7-8): Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, and Denis Comte. Polyarteritis nodosa: old disease, new etiologies. International Journal of Molecular Sciences, 24:16668, Nov 2023. URL: https://doi.org/10.3390/ijms242316668, doi:10.3390/ijms242316668. This article has 50 citations.
(wolff2023polyarteritisnodosareviewed pages 7-8): Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, and Denis Comte. Polyarteritis nodosa reviewed in 2023: old disease, new etiologies. Oct 2023. URL: https://doi.org/10.20944/preprints202310.0949.v1, doi:10.20944/preprints202310.0949.v1.
(taprantzis2026clinicalmanifestationsprognostic pages 1-2): Nikolaos Taprantzis, Maria Eleni Kasimeri, Dimosthenis Chrysikos, Amir Shihada, Alexandros Samolis, George Tsakotos, Martina Liga, and Theodore Troupis. Clinical manifestations, prognostic impact, and relapse in polyarteritis nodosa: a systematic review and meta-analysis. Rheumatology International, Feb 2026. URL: https://doi.org/10.1007/s00296-026-06082-8, doi:10.1007/s00296-026-06082-8. This article has 0 citations and is from a peer-reviewed journal.
(chung20212021americancollegea pages 5-6): Sharon A. Chung, Mark Gorelik, Carol A. Langford, Mehrdad Maz, Andy Abril, Gordon Guyatt, Amy M. Archer, Doyt L. Conn, Kathy A. Full, Peter C. Grayson, Maria F. Ibarra, Lisa F. Imundo, Susan Kim, Peter A. Merkel, Rennie L. Rhee, Philip Seo, John H. Stone, Sangeeta Sule, Robert P. Sundel, Omar I. Vitobaldi, Ann Warner, Kevin Byram, Anisha B. Dua, Nedaa Husainat, Karen E. James, Mohamad Kalot, Yih Chang Lin, Jason M. Springer, Marat Turgunbaev, Alexandra Villa‐Forte, Amy S. Turner, and Reem A. Mustafa. 2021 american college of rheumatology/vasculitis foundation guideline for the management of polyarteritis nodosa. Arthritis Care & Research, 73:1061-1070, Jul 2021. URL: https://doi.org/10.1002/acr.24633, doi:10.1002/acr.24633. This article has 137 citations and is from a domain leading peer-reviewed journal.
(lin2021polyarteritisnodosaa pages 1-2): Yih Chang Lin, Mohamad A. Kalot, Nedaa M. Husainat, Kevin Byram, Anisha B. Dua, Karen E. James, Jason M. Springer, Marat Turgunbaev, Alexandra Villa‐Forte, Andy Abril, Carol Langford, Mehrdad Maz, Sharon A. Chung, and Reem A. Mustafa. Polyarteritis nodosa: a systematic review of test accuracy and benefits and harms of common treatments. ACR Open Rheumatology, 3:91-100, Jan 2021. URL: https://doi.org/10.1002/acr2.11189, doi:10.1002/acr2.11189. This article has 14 citations and is from a peer-reviewed journal.
(puechal2022polyarteritisnodosastate pages 2-3): Xavier Puéchal. Polyarteritis nodosa: state of the art. Joint Bone Spine, 89:105320, Jul 2022. URL: https://doi.org/10.1016/j.jbspin.2021.105320, doi:10.1016/j.jbspin.2021.105320. This article has 16 citations and is from a peer-reviewed journal.
(puechal2022polyarteritisnodosastate media 85b1bd4a): Xavier Puéchal. Polyarteritis nodosa: state of the art. Joint Bone Spine, 89:105320, Jul 2022. URL: https://doi.org/10.1016/j.jbspin.2021.105320, doi:10.1016/j.jbspin.2021.105320. This article has 16 citations and is from a peer-reviewed journal.
(puechal2022polyarteritisnodosastate media 858e4031): Xavier Puéchal. Polyarteritis nodosa: state of the art. Joint Bone Spine, 89:105320, Jul 2022. URL: https://doi.org/10.1016/j.jbspin.2021.105320, doi:10.1016/j.jbspin.2021.105320. This article has 16 citations and is from a peer-reviewed journal.
(wolff2023polyarteritisnodosaold pages 8-9): Louis Wolff, Alice Horisberger, Laura Moi, Maria P. Karampetsou, and Denis Comte. Polyarteritis nodosa: old disease, new etiologies. International Journal of Molecular Sciences, 24:16668, Nov 2023. URL: https://doi.org/10.3390/ijms242316668, doi:10.3390/ijms242316668. This article has 50 citations.
(NCT00315406 chunk 1): Peter Merkel. Determining Disease Activity Biomarkers in Individuals With Polyarteritis Nodosa. University of Pennsylvania. 2006. ClinicalTrials.gov Identifier: NCT00315406
(NCT00647166 chunk 1): Association Corticosteroid/Azathioprine in Microscopic Polyangiitis/ Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome). Assistance Publique - Hôpitaux de Paris. 2008. ClinicalTrials.gov Identifier: NCT00647166
(NCT00647166 chunk 2): Association Corticosteroid/Azathioprine in Microscopic Polyangiitis/ Polyarteritis Nodosa or Eosinophilic Granulomatosis With Polyangiitis (Churg Strauss Syndrome). Assistance Publique - Hôpitaux de Paris. 2008. ClinicalTrials.gov Identifier: NCT00647166