Resumo
Definition
History and exam
Key diagnostic factors
- história de asma, rinite alérgica ou sinusite
- fraqueza ou dormência focal
- obstrução ou secreção nasal, ou dor facial
- púrpura palpável e petéquias
- sibilo
- hemoptise
- nódulos cutâneos
Other diagnostic factors
- uso de determinados medicamentos
- fadiga, artralgias, mialgias
- dispneia ou tosse
- dor abdominal
- deficits sensoriais ou motores
- taquipneia
- estertores
- edema periférico
- ortopneia
- refluxo hepatojugular
Risk factors
- história de asma, rinite alérgica ou sinusite
- uso de determinados medicamentos
Diagnostic investigations
1st investigations to order
- Hemograma completo com diferencial
- anticorpos anticitoplasma de neutrófilo (ANCA) séricos
- proteína C-reativa sérica
- velocidade de hemossedimentação
- ureia e creatinina séricas
- urinálise
- teste de função pulmonar
- radiografia torácica
- ecocardiograma
Investigations to consider
- citometria de fluxo de sangue periférico
- testes moleculares para mutação FIP1L1/PDGFR alfa
- imunoglobulina E (IgE) sérica
- IgE e IgG séricas específicas para Aspergillus fumigatus
- coprocultura para ovos e parasitas
- Sorologia para Toxocara
- tomografia computadorizada (TC) do tórax
- eletromiografia
- broncoscopia com lavagem broncoalveolar (LBA)
- biópsia do tecido afetado
- ultrassonografia de membro inferior
- Angiotomografia
- biópsia da medula óssea
- nível da tiopurina metiltransferase
- teste de vírus da imunodeficiência humana (HIV)
Emerging tests
- ressonância nuclear magnética (RNM) cardíaca
- eotaxina-3
Treatment algorithm
GEPA não grave
GEPA grave
recidiva após remissão bem-sucedida
Contributors
Authors
Karina Keogh, MBBCh
Assistant Professor
Division of Pulmonary and Critical Care Medicine
Mayo Clinic
Rochester
MN
Disclosures
KK is an author of a number of references cited in this topic and has developed educational material on Churg-Strauss syndrome for First Consult (Elsevier), and received an education grant from GlaxoSmithKline for lectures given on EGPA at Boston University. KK has undertaken contractual research for GlaxoSmithKline (utilizing mepolizumab in asthma trials) and AstraZeneca (asthma trial), but received no personal financial gain for this work.
Acknowledgements
Dr Karina Keogh would like to gratefully acknowledge Dr Garvan Kane, a previous contributor to this topic.
Disclosures
GK is an author of some references cited in this topic.
Peer reviewers
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 has organisational financial interests with funds received for consultation and training on disease assessment in vasculitis for GlaxoSmithKline, Nordic, and Chemocentryx. RL has received personal funds for lectures on vasculitis from Roche and Nippon Kayaku. RL is an author of a reference cited in this topic.
Matthew D. Morgan, MB ChB, MRCP, PhD
Clinical Senior Lecturer in Renal Medicine
Centre for Translational Inflammation Research
University of Birmingham Research Laboratories
Queen Elizabeth Hospital Birmingham
Birmingham
UK
Disclosures
MDM has been a paid consultant for Sphere Medical Ltd, and has received research grants and funding from GlaxoSmithKline and Novartis.
Philip Seo, MD, MHS
Assistant Professor of Medicine
Division of Rheumatology
Johns Hopkins University School of Medicine
Co-Director
Johns Hopkins Vasculitis Center
Baltimore
MD
Disclosures
PS declares that he has no competing interests.
Jaap Van Laar, MD, PhD
Professor of Clinical Rheumatology
Institute of Cellular Medicine
Newcastle University
Newcastle upon Tyne
Honorary Consultant Rheumatologist
The James Cook University Hospital
Middlesbrough
UK
Disclosures
JVL is consultant for Roche, the manufacturer of rituximab, and has received speaker fees and reimbursements for attending several conferences.
Robert A. Ortmann, MD
Associate Professor and Director
Division of Rheumatology
University of Arkansas for Medical Sciences
Little Rock
AR
Disclosures
RAO declares that he 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
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
Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015 Sep;26(7):545-53.Full text Abstract
Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021 Aug;73(8):1366-83.Full text Abstract
Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16:ard-2022-223764.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 (GPA) (anteriormente conhecida como granulomatose de Wegener)
- Poliangiíte microscópica (PAM)
- Síndrome hipereosinofílica (SHE)
More DifferentialsGuidelines
- Guideline for the prevention and treatment of glucocorticoid-induced osteoporosis
- Recommendations for the management of ANCA-associated vasculitis
More GuidelinesPatient information
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