Please read this first. This guide is a companion to your medical team, not a replacement, and it is
not medical advice.
Everything here is tied to published research. If something you expected is not here, it almost always means we have not mapped a source for it yet,
not that it is unknown to medicine.
polyarteritis nodosa is an early, growing map, so it will look incomplete on purpose: we would rather show less and have every line be something you can check than fill the page with claims we cannot stand behind.
For anything about your own situation, your clinicians hold the full picture.
How this guide is built and why.
What's polyarteritis nodosa?
Polyarteritis nodosa is a necrotizing vasculitis that mainly inflames medium-sized arteries, damaging their walls and reducing blood flow to the organs they supply. It is not associated with ANCA antibodies, which distinguishes it from several related vasculitides, and a minority of cases are linked to hepatitis B infection. It can affect the skin, peripheral nerves, gut, kidneys, and other organs.
| Also indexed as | ORPHA:767, MONDO:0019170 |
|---|
| Features mapped | 11 |
|---|
| Treatments mapped | 3 |
|---|
| Published sources | 14 |
|---|
| Last reviewed | 2026-08-04 |
|---|
Signs and symptoms
Fever
Fever is one of the general, whole-body symptoms that often appears in polyarteritis nodosa, a vasculitis of medium-sized blood vessels.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:41243061, PMID:41128202, ORPHA:767
Notesplain_language confirmed from PMID:41243061 via curation 2026-06-14. plain_language confirmed from PMID:41128202 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:29558355 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Elevated circulating C-reactive protein concentration
C-reactive protein (CRP) is a blood marker of inflammation. It is often raised during active disease, and very high levels have been linked with worse outcomes.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:41280967, ORPHA:767
Notesplain_language confirmed from PMID:41280967 via curation 2026-06-14. | regrounded primary ORPHA:767 -> PMID:41957293 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Peripheral neuropathy
When polyarteritis nodosa reduces blood flow to individual nerves, it can produce a pattern of nerve damage called mononeuritis multiplex, which causes painful loss of sensation and weakness.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:39799509, PMID:39348853, ORPHA:767
Notesplain_language confirmed from PMID:39799509 via curation 2026-06-14. plain_language confirmed from PMID:39348853 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:39316132 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Weight loss
Unintended weight loss is one of the general, whole-body symptoms that often occurs in polyarteritis nodosa.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:41398802, ORPHA:767
Notesplain_language confirmed from PMID:41398802 via curation 2026-06-14. plain_language confirmed from PMID:41128202 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:41128202 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Livedo racemosa
A net-like, mottled discoloration of the skin (livedo) is a main skin sign of polyarteritis nodosa and tends to appear on the legs.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:32044177, PMID:29558355, ORPHA:767
Notesplain_language confirmed from PMID:32044177 via curation 2026-06-14. plain_language confirmed from PMID:29558355 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:31300830 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Hypertension
High blood pressure can develop when the arteries supplying the kidneys are inflamed, and it is one of the more serious manifestations of the disease.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesORPHA:767
Notesplain_language confirmed from PMID:39799509 via curation 2026-06-14. | regrounded primary ORPHA:767 -> PMID:39799509 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Abdominal pain
Abdominal pain occurs when the arteries supplying the gut are inflamed. It can be hard to diagnose because the pain mimics other acute abdominal conditions.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesORPHA:767
Notesplain_language confirmed from PMID:41243061 via curation 2026-06-14. | regrounded primary ORPHA:767 -> PMID:41243061 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Myalgia
Muscle pain (myalgia) is one of the common symptoms of polyarteritis nodosa.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:41615024, PMID:41128202, ORPHA:767
Notesplain_language confirmed from PMID:41615024 via curation 2026-06-14. plain_language confirmed from PMID:41128202 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:29558355 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Skin ulcer
Skin ulcers are a main skin sign of polyarteritis nodosa and tend to appear on the legs.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:36300176, PMID:29558355, ORPHA:767
Notesplain_language confirmed from PMID:36300176 via curation 2026-06-14. plain_language confirmed from PMID:29558355 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:28547523 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Subcutaneous nodule
Lumps under the skin (subcutaneous nodules) are a main skin sign of polyarteritis nodosa and tend to appear on the legs.
Evidence ratingweak
Study designontology_import
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:37325248, PMID:29558355, ORPHA:767
Notesplain_language confirmed from PMID:37325248 via curation 2026-06-14. plain_language confirmed from PMID:29558355 via curation 2026-06-25 [claude-draft]. | regrounded primary ORPHA:767 -> PMID:31300830 on 2026-06-26 [Carrie Schluter, BCPA]
Last reviewed2026-06-26
Mononeuritis multiplex
In polyarteritis nodosa, loss of blood supply to individual peripheral nerves can produce the classic mononeuritis multiplex pattern: a painful loss of sensation and movement affecting separate nerves.
Evidence ratingweak
Study designJournal Article, Review
Confidence (0-1)0.7
Replicationunreplicated
Notesplain_language confirmed from PMID:39348853 via curation 2026-06-25 [claude-draft].
Last reviewed2026-06-25
How it is diagnosed
Polyarteritis nodosa
Diagnosed using: CT angiography (medium-vessel abnormalities).
The source text this rests on
“…contrast-enhanced computed tomography (CECT) and computed tomography angiography (CTA), are vital for evaluating acute presentations and identifying characteristic…”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:41243061 via curation 2026-06-25
Last reviewed2026-06-25
Polyarteritis nodosa
Diagnosed using: ACR 1990 classification criteria.
The source text this rests on
“…fulfilling American College of Rheumatology (ACR) 1990 criteria for…”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:39799509 via curation 2026-06-25
Last reviewed2026-06-25
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
corticosteroids
Steroids are the basis of treatment for polyarteritis nodosa, with more severe disease also needing cyclophosphamide.
Used to help with: Polyarteritis nodosa.
The source text this rests on
“While glucocorticoids remain first-line therapy, emerging evidence supports the use of biologic agents in refractory cases.”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Supporting sourcesPMID:41128202
Notesconfirmed from PMID:41416335 via curation 2026-06-14
Last reviewed2026-06-14
cyclophosphamide
Cyclophosphamide is an immune-suppressing medicine added for severe or organ-threatening disease, usually together with corticosteroids, to control the inflammation.
Used to help with: Polyarteritis nodosa.
The source text this rests on
“Treatment needs steroids, and severe forms need association with cyclophosphamide.”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:41128202 via curation 2026-06-14
Last reviewed2026-06-14
antiviral therapy
When polyarteritis nodosa is linked to hepatitis B infection, treatment centers on antiviral therapy (often with plasma exchange and a short course of steroids) rather than long-term immune suppression, because suppressing the immune system can worsen the viral infection.
Used to help with: Polyarteritis nodosa.
The source text this rests on
“The present index case indicates that antiviral combined with plasma exchange after short-term corticosteroids is an effective therapy for HBV-PAN.”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:37188009 via curation 2026-06-14
Last reviewed2026-06-14
What changes how it shows up
The diagnosis is not the whole story. The factors and open questions below are described in the research mapped here as shaping whether, or how strongly, the condition shows up, or as points the field has not yet settled. They are not, on their own, its cause or its cure.
hepatitis B-associated PAN
Polyarteritis nodosa has historically been linked to hepatitis B virus infection; hepatitis B-associated PAN is now recognized as a separate condition from idiopathic PAN.
Described as modulating: Polyarteritis nodosa.
The source text this rests on
“…hepatitis B associated PAN is a separate…”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:40071425 via curation 2026-06-25
Last reviewed2026-06-25
DADA2 (ADA2 deficiency) mimic
Some inherited conditions, including ADA2 deficiency (DADA2) and VEXAS syndrome, can mimic polyarteritis nodosa.
Described as modulating: Polyarteritis nodosa.
The source text this rests on
“…discovery of genetic syndromes including DADA2 and VEXAS which can mimic…”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:40071425 via curation 2026-06-25
Last reviewed2026-06-25
ANCA-negative medium-vessel vasculitis
Polyarteritis nodosa is set apart from other vasculitis by the absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies (ANCA).
Described as modulating: Polyarteritis nodosa.
The source text this rests on
“The absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies serves to distinguish PAN from other types of vasculitis.”
An excerpt quoted verbatim from the source named above, shown as recorded. The full sentence is in the linked source.
Evidence ratingweak
Confidence (0-1)0.7
Replicationunreplicated
Notesconfirmed from PMID:38068989 via curation 2026-06-25
Last reviewed2026-06-25
How to read the evidence labels
Widely acceptedSpecialists broadly agree on this.
Strong evidenceBacked by solid, repeated research.
Moderate evidenceReasonable evidence, still being confirmed.
Limited evidenceSome evidence, but not yet convincing.
Early evidenceAn early finding that needs more study.
Experts disagreeResearchers actively disagree about this.
No longer supportedLater, stronger evidence or guidance overturned this.
Where this comes from
This guide is built from 14 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
ORPHA:767 · Orphanet/HPO annotations for Polyarteritis nodosa
PMID:28547523 · Cutaneous Manifestations of Medium- and Large-Vessel Vasculitis.
PMID:31300830 · [Cutaneous alterations in vasculitides : Part 2: Special aspects of diseases of large, medium and small vessels].
PMID:37188009 · Interesting rare case of polyarteritis nodosa related to hepatitis B virus and plasma exchange role? - A case report and
PMID:38068989 · Polyarteritis Nodosa: Old Disease, New Etiologies.
PMID:39316132 · [Vasculitic involvement of the skeletal muscle and the peripheral nervous system: clinical and neuropathologic perspective].
PMID:39348853 · Top 10 Clinical Pearls in Vasculitic Neuropathies.
PMID:39799509 · The clinical characteristics and treatment outcomes of patients with systemic polyarteritis nodosa: a single-centre study from India.
PMID:40071425 · Idiopathic polyarteritis nodosa - does it still exist? Viewpoint 1: as our knowledge makes progress, idiopathic polyarte
PMID:41243061 · A visual guide for emergency radiologists navigating the complexity of abdominal vasculitis: part 1 - true vasculitis.
PMID:41416335 · Polyarteritis Nodosa With Mesenteric, Hepatic, and Musculoskeletal Manifestations in a 36-Year-Old Nigerian Male Patient
PMID:41957293 · [18F]FDG PET/CT value in the diagnostic evaluation of patients with polyarteritis nodosa.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.