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Polyarteritis nodosa 

Polyarteritis nodosa
Chapter:
Polyarteritis nodosa
Author(s):

Loïc Guillevin

DOI:
10.1093/med/9780198746690.003.0460
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date: 27 February 2021

Polyarteritis nodosa (PAN) is a necrotizing angiitis, often associated with hepatitis B infection, that predominantly affects medium-sized arteries. Patients usually have systemic features; specific manifestations include peripheral neuropathy, mononeuritis multiplex, skin lesions (subcutaneous nodules, purpura), hypertension, renal insufficiency, gastrointestinal haemorrhage/perforation, and orchitis. Elevated markers of inflammation are found in most patients, but serum antineutrophil cytoplasmic antibodies are not present. Standard treatment of hepatitis B negative cases is with corticosteroids (typically initiated with intravenous methylprednisolone) and cyclophosphamide; hepatitis B positive cases are treated with an antiviral agent, corticosteroid and plasma exchange, with corticosteroid discontinued as soon as disease comes under control. Severe gastrointestinal involvement is the primary cause of early death; later deaths may result from treatment side effects.

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