Summary
Definition
History and exam
Key diagnostic factors
- age 40 to 60 years
- fever
- weight loss
- myalgia or arthralgia
- mononeuritis multiplex
- paresthesia
- 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
Other diagnostic factors
- male sex
- hairy cell leukemia
- seizure
- upper motor neuron weakness
- gastrointestinal (GI) bleeding
- peritonitis
- monocular blindness
- chest pain
- congestive cardiac failure
- tender breast lumps
Risk factors
- hepatitis B virus (HBV) infection
- age 40 to 60 years
- hairy cell leukemia
- history of blood transfusion predating introduction of routine HBV screening
- hepatitis C virus (HCV) infection
- male sex
Diagnostic tests
1st tests to order
- CRP
- erythrocyte sedimentation rate (ESR)
- CBC
- complement
- serum creatinine
- midstream urine analysis
- liver function tests
- hepatitis B virus (HBV) serology
- hepatitis C virus (HCV) serology
- cryoglobulins
- blood culture
- creatine kinase
- antineutrophil 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
- fibrinogen
- conventional digital subtraction angiography
- echocardiography
Tests to consider
- MR angiography (MRA)
- CT angiography
- biopsy of affected tissue
- HIV serology
- genetic test for adenosine deaminase 2 (ADA2) deficiency
Treatment algorithm
non HBV-related PAN
HBV-related PAN
relapse of disease
Contributors
Authors
Ravi Suppiah, MBChB, PGDipSportMed, MD, FRACP
Consultant Rheumatologist
Auckland and Counties Manukau District Health Boards
Auckland
New Zealand
Disclosures
RS declares that he has no competing interests.
Joanna Robson, MBBS, PhD, MRCP
Rheumatology Registrar
Nuffield Orthopaedic Centre
Oxford
UK
Disclosures
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
Oxford
UK
Disclosures
RL is an author of a number of references cited in this topic.
Acknowledgements
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.
Peer reviewers
Alan Bridges, MD
Professor and Vice Chair
Department of Medicine
University of Wisconsin Hospital
Madison
WI
Disclosures
AB declares that he has no competing interests.
Richard Watts, MA, DM, FRCP
Consultant Rheumatologist
Ipswich Hospital
Ipswich
UK
Disclosures
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
New Brunswick
NJ
Disclosures
ECE declares that she has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094-1100. Abstract
Mahr A, Guillevin L, Poissonnet M, et al. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51:92-99. Abstract
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):310-7Full text Abstract
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.Full text Abstract
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.Full text Abstract
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.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis)
- Microscopic polyangiitis
- Churg-Strauss syndrome
More DifferentialsGuidelines
- Guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
- BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis
More GuidelinesPatient information
Pneumonia: do I need a vaccine?
More Patient information
Log in or subscribe to access all of BMJ Best Practice
Use of this content is subject to our disclaimer