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Polyarteritis nodosa in Adult
Other Resources UpToDate PubMed

Polyarteritis nodosa in Adult

Contributors: Vivian Wong MD, PhD, Samantha R. Pop MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed


Polyarteritis nodosa (PAN) refers to a systemic necrotizing vasculitis of medium-sized and occasionally small arteries. The exact etiology is unknown, but it likely involves immune complex deposition, autoantibodies, inflammatory mediators, and adhesion molecules.

PAN most commonly involves the skin, peripheral nerves, kidneys, joints, and gastrointestinal tract. Any organ may be affected, although the lungs are typically spared. Constitutional symptoms may include malaise, fever, weakness, myalgias, arthralgias, and weight loss in combination with multiorgan symptoms of ischemia or infarction such as abdominal pain, cutaneous ulcers, livedo reticularis, testicular pain, mononeuropathy multiplex, and hypertension. Neurologic manifestations with asymmetric peripheral neuropathy can result in motor and sensory deficits and are the most common presenting organ manifestation. Renal involvement is common, manifested by hypertension and renal impairment, but red cell casts are notably absent; their presence should raise suspicion for an alternative diagnosis. Cardiac involvement presenting with myocardial infarction has been described. Other complications include stroke, gangrene, renal failure (acute, chronic), bowel ischemia and perforation, gastrointestinal bleeding, retinal detachment, and death. Corticosteroids and immunosuppressive medications are the cornerstones of treatment.

PAN usually affects individuals in the fourth to sixth decades of life. It is slightly more common in men, and there is no apparent racial predilection. An isolated cutaneous form also exists (cutaneous polyarteritis nodosa [cPAN]), which is more common in children. PAN has been most commonly associated with infection with hepatitis B (30% of patients are positive for the B antigen). There are reports of associations with hepatitis C, cytomegalovirus (CMV), parvovirus B19, human T-lymphotropic virus (HTLV type 1, type 2), and streptococci. There appears to be an association with inflammatory bowel disease. Minocycline has rarely been reported to induce both systemic PAN and limited cPAN.

Childhood-onset polyarteritis nodosa is a finding in deficiency of adenosine deaminase type 2 (DADA2), a rare multisystem vasculopathic syndrome due to autosomal recessive mutations in the CECR1 gene.


M30.0 – Polyarteritis nodosa

155441006 – Polyarteritis nodosa

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Granulomatosis with polyangiitis
  • Antiphospholipid antibody syndrome
  • Disseminated intravascular coagulation (DIC)
  • Calciphylaxis
  • Microscopic polyangiitis
  • Immunoglobulin A vasculitis (formerly Henoch-Schönlein purpura)
  • Systemic lupus erythematosus
  • Eosinophilic granulomatosis with polyangiitis
  • Thrombotic thrombocytopenic purpura (TTP)
  • Cocaine levamisole toxicity
  • Lymphomatoid granulomatosis
  • Fibromuscular dysplasia
  • Martorell hypertensive leg ulcer
  • Necrotizing Leukocytoclastic vasculitis
  • Cryoglobulinemia
  • Cryofibrinogenemia
  • Cutaneous anthrax
  • Necrotizing fasciitis
  • Acute meningococcemia
  • Sporotrichosis
  • Mucormycosis
  • Erythema nodosum
  • Nodular vasculitis
  • Pyoderma gangrenosum
  • Sarcoidosis
  • VEXAS syndrome

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Management Pearls

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed:06/12/2022
Last Updated:07/12/2022
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Polyarteritis nodosa in Adult
A medical illustration showing key findings of Polyarteritis nodosa : Abdominal pain, Fever, LDH elevated, Malaise, Neuropathy peripheral, Palpable purpura, Proteinuria, Vision loss, Arthralgia, Myalgia, CRP elevated, ESR elevated, RBC decreased
Clinical image of Polyarteritis nodosa - imageId=2732508. Click to open in gallery.  caption: 'Retiform, erythematous plaques with central brown patches on the knees.'
Retiform, erythematous plaques with central brown patches on the knees.
Copyright © 2024 VisualDx®. All rights reserved.