Summary
Definition
History and exam
Key diagnostic factors
- idade entre 40 e 60 anos
- febre
- perda de peso
- mialgia ou artralgia
- mononeurite múltipla
- parestesia
- Sensibilidade muscular
- dor abdominal
- manifestações cutâneas
- pressão arterial diastólica >90 mmHg
- história de transfusão de sangue anterior à introdução do rastreamento de rotina do HBV
- abuso de substâncias por via intravenosa prévio ou atual
- infecção recente pelo HBV
- dor testicular
Other diagnostic factors
- sexo masculino
- leucemia de células pilosas
- convulsão
- fraqueza do neurônio motor superior
- sangramento gastrointestinal agudo
- peritonite
- cegueira monocular
- dor torácica
- insuficiência cardíaca congestiva
- nódulos mamários sensíveis
Risk factors
- infecção pelo vírus da hepatite B (HBV)
- idade entre 40 e 60 anos
- leucemia de células pilosas
- história de transfusão de sangue anterior à introdução do rastreamento de rotina do HBV
- infecção pelo vírus da hepatite C (HCV)
- sexo masculino
Diagnostic investigations
1st investigations to order
- proteína C-reativa
- velocidade de hemossedimentação (VHS)
- Hemograma completo
- complemento
- creatinina sérica
- análise da urina de jato médio
- testes da função hepática
- sorologia para HBV
- sorologia para o vírus da hepatite C (HCV)
- crioglobulinas
- hemocultura
- creatina quinase
- anticorpo anticitoplasma de neutrófilo (ANCA)
- fatores antinucleares (FANs)
- anticorpos antiácido desoxirribonucleico (DNA) de fita dupla (anti-dsDNA)
- fator reumatoide
- anticorpos antipeptídeos citrulinados cíclicos (anticorpos anti-CCP)
- anticoagulante lúpico
- anticorpos antifosfolipídeos do tipo imunoglobulina G
- glicoproteína B2
- fibrinogênio
- angiografia digital por subtração convencional
- ecocardiografia
Investigations to consider
- angiografia por ressonância magnética (ARM)
- Angiotomografia
- biópsia do tecido afetado
- sorologia do HIV
- teste genético para deficiência de adenosina desaminase 2 (ADA2)
Treatment algorithm
poliarterite nodosa (PAN) não relacionada ao vírus da hepatite B (HBV)
PAN relacionada ao HBV
recidiva da doença
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
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;53:2306-2309.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
Mukhtyar C, Guillevin L, Cid MC, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310-317.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
- Granulomatose com poliangiite (anteriormente conhecida como granulomatose de Wegener)
- Poliangiite microscópica
- Síndrome de Churg-Strauss
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- Guideline for vaccinations in patients with rheumatic and musculoskeletal diseases
- BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis
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