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 immunisation programmes 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 anti-neutrophil 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 characterised by focal and segmental transmural necrotising inflammation with fibrinoid necrosis in medium-sized vessels.
Treatment for non-HBV-related PAN is based on immunosuppression with corticosteroids and cyclophosphamide.
Treatment for HBV-related PAN utilises a short course of high-dose corticosteroids, followed by a combination of antiviral therapy and plasma exchange.
Prognosis can be determined by use of the 5-factor score.
The term 'periarteritis nodosa' was used in the 19th century to describe any form of systemic vasculitis without a known cause. Since then, the name has changed to polyarteritis nodosa (PAN) and the definition has been refined to: "Necrotising inflammation of medium-sized or small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules." This definition was proposed at the Chapel Hill Consensus Conference (CHCC) in 1992 and published in 1994.
History and exam
- age 40 to 60 years
- weight loss
- myalgia or arthralgia
- mononeuritis multiplex
- muscle tenderness
- abdominal pain
- skin manifestations
- diastolic blood pressure >90 mmHg
- history of blood transfusion predating introduction of routine HBV screening
- previous or current intravenous drug abuse
- recent hepatitis B virus (HBV) infection
- testicular pain
- erythrocyte sedimentation rate (ESR)
- serum creatinine
- midstream urine analysis
- liver function tests
- HBV serology
- hepatitis C virus (HCV) serology
- blood culture
- creatine kinase
- anti-neutrophil cytoplasmic antibodies (ANCA)
- antinuclear antibodies (ANA)
- anti-double-stranded DNA antibodies (anti-dsDNA)
- rheumatoid factor
- antibodies to cyclic citrullinated peptides (anti-CCP antibodies)
- lupus anticoagulant
- immunoglobulin G antiphospholipid antibodies
- B2 glycoprotein
- conventional digital subtraction angiography
Ravi Suppiah, MBChB, PGDipSportMed, MD, FRACP
Auckland and Counties Manukau District Health Boards
RS declares that he has no competing interests.
Joanna Robson, MBBS, PhD, MRCP
Nuffield Orthopaedic Centre
JR declares that she has no competing interests.
Raashid Luqmani, DM, FRCP, FRCP(E)
Professor of Rheumatology
NIHR Musculoskeletal Biomedical Research Unit
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science
University of Oxford
RL is an author of a number of references cited in this topic.
Dr Ravi Suppiah, Dr Joanna Robson and Dr Raashid Luqmani would like to gratefully acknowledge Dr Loic Guillevin, the previous contributor to this topic. LG is an author of a number of references cited in this topic.
Alan Bridges, MD
Professor and Vice Chair
Department of Medicine
University of Wisconsin Hospital
AB declares that he has no competing interests.
Richard Watts, MA, DM, FRCP
RW has received fees for consulting from Roche Pharmaceuticals, manufacturer of rituximab. His department has received financial support from Wyeth Pharmaceuticals, manufacturer of etanercept, and from Schering-Plough, manufacturer of infliximab. RW is an author of a number of references cited in this topic.
Ellen C. Ebert, MD
Professor of Medicine
Department of Medicine
UMDNJ-Robert Wood Johnson Medical School
ECE declares that she has no competing interests.
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