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Highlights & Basics
- Polyarteritis nodosa (PAN) is a rare form of systemic vasculitis that affects only medium-sized vessels (i.e., small and medium-sized arteries).
- Hepatitis B virus (HBV)-related PAN has become very rare since the introduction of effective immunization programs against the virus.
- Both non HBV-related PAN and HBV-related PAN are differentiated from the other small- and medium-vessel vasculitides by the absence of antineutrophil cytoplasmic antibodies, and by confirmation that small vessels (i.e., arterioles, capillaries, venules) are not involved.
- Angiography typically demonstrates microaneurysms and focal narrowing in medium-sized blood vessels.
- Pathology is characterized by focal and segmental transmural necrotizing inflammation with fibrinoid necrosis in medium-sized vessels.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
American College of Rheumatology 1990 criteria for PAN
Renal angiogram showing aneurysms, a classical feature of PAN
Renal angiogram showing aneurysms and microaneurysms
A purpuric rash is a common feature
Skin ulcers in the lower limbs are a common manifestation of PAN
Digital gangrene from medium vessel vasculitis
Large skin ulcer from a patient with PAN
Vesiculo-bullous skin rash with necrosis
Mesenteric angiogram showing small and micro aneurysms
Pathology specimen of a medium-sized muscular artery showing transmural inflammation
Biopsy specimen showing florid transmural inflammation of a small artery
Citations
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Colmegna I, Maldonado-Cocco JA. Polyarteritis nodosa revisited. Curr Rheumatol Rep. 2005;7:288-296.[Abstract]
Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009 Mar;68(3):310-7[Abstract][Full Text]
Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.[Abstract][Full Text]
Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003 Jul 3;349(1):36-44.[Abstract][Full Text]
De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005 Aug;52(8):2461-9.[Abstract][Full Text]
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