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For further information, see CMDT Part 20-19: Polyarteritis Nodosa

Key Features

Essentials of Diagnosis

  • Medium-sized arteries are affected

  • Clinical findings depend on the arteries involved; lungs are spared

  • Common features

    • Fever

    • Abdominal pain

    • Extremity pain

    • Livedo reticularis

    • Mononeuritis multiplex

  • Kidney involvement causes renin-mediated hypertension

  • Associated with hepatitis B (10% of cases)

General Considerations

  • A necrotizing arteritis of medium-sized vessels that has a predilection for involving the skin, peripheral nerves, mesenteric vessels (including renal arteries), heart, and brain but spares the lungs

  • Rare, with a prevalence of 30 per 1 million people

  • Approximately 10% of cases are caused by hepatitis B, with most cases of these occur within 6 months of onset of hepatitis B infection

Clinical Findings

Symptoms and Signs

  • Fever, malaise, weight loss, and other symptoms develop over weeks to months

  • Pain in the extremities

  • Vasculitic neuropathy

  • Livedo reticularis, subcutaneous nodules, and skin ulcers reflect involvement of deeper, medium-sized blood vessels

  • Digital gangrene is not unusual

  • Lower extremity ulcerations, usually occurring near the malleoli

  • Renin-mediated hypertension due to involvement of renal arteries

  • Acalculous cholecystitis or appendicitis caused by compromised function of major viscera

  • Dramatic presentation in some cases

    • Acute abdomen caused by mesenteric vasculitis and gut perforation

    • Hypotension resulting from rupture of a microaneurysm in the liver, kidney, or bowel

  • Subclinical cardiac involvement is common; overt cardiac dysfunction occurs occasionally

  • Lungs are seldom (if ever) involved

Differential Diagnosis

  • Microscopic polyangiitis

  • Granulomatosis with polyangiitis

  • Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome)

  • Endocarditis

  • Cryoglobulinemia

  • Cholesterol atheroembolic disease

  • Other systemic causes of peripheral neuropathy

    • Rheumatoid arthritis

    • Diabetes mellitus

    • Amyloidosis

    • Sarcoidosis

    • Plasma cell myeloma

  • Other causes of mesenteric ischemia, eg, embolism, atherosclerosis


Laboratory Tests

  • Slight anemia and leukocytosis are common

  • Acute-phase reactants (ESR, CRP) are often (but not always) strikingly elevated

  • Patients are ANCA-negative

  • Rheumatoid factor or antinuclear antibodies may be present in low titers

  • Appropriate tests for active hepatitis B infection (HBsAg, HBeAg, hepatitis B viral load) should be performed

Diagnostic Procedures

  • Requires confirmation with either a tissue biopsy or vascular imaging

  • Biopsies of symptomatic sites such as skin (from the edge of an ulcer or the center of a nodule) and nerve and muscle are essential for diagnosis

    • A deep biopsy, not a punch biopsy, of skin ulcers or nodules should be made to ensure a medium-size vessel is included in the sample

    • Biopsy of the nerve and muscle (instead of the nerve alone) is recommended in patients with neuropathic symptoms

    • If performed by experienced physicians, tissue biopsies normally have high benefit-risk ratios because of the importance of establishing the diagnosis

  • Angiographic finding of aneurysmal dilations in the renal, mesenteric, or hepatic arteries is ...

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