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Granulomatose eosinofílica com poliangiite (Síndrome de Churg-Strauss)

  • Visão geral
  • Teoria
  • Diagnóstico
  • Tratamento
  • ACOMPANHAMENTO
  • Recursos
Última revisão: 20 Sep 2025
Última atualização: 16 Jan 2024

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
Full details

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
Full details

Risk factors

  • história de asma, rinite alérgica ou sinusite
  • uso de determinados medicamentos
Full details

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
Full details

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)
Full details

Emerging tests

  • ressonância nuclear magnética (RNM) cardíaca
  • eotaxina-3

Treatment algorithm

ACUTE

GEPA não grave

GEPA grave

ONGOING

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

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

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.
  • Granulomatose eosinofílica com poliangiite (Síndrome de Churg-Strauss) images

  • Differentials

    • Granulomatose com poliangiite (GPA) (anteriormente conhecida como granulomatose de Wegener)
    • Poliangiíte microscópica (PAM)
    • Síndrome hipereosinofílica (SHE)
    More Differentials

  • Guidelines

    • Guideline for the prevention and treatment of glucocorticoid-induced osteoporosis
    • Recommendations for the management of ANCA-associated vasculitis
    More Guidelines

  • Patient information

    Asma em adultos: perguntas a fazer ao seu médico

    Asma em adultos: o que é?

    More Patient information
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