Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Summary
Definition
History and exam
Key diagnostic factors
- history of asthma, allergic rhinitis, or sinusitis
- focal numbness or weakness
- nasal discharge or stuffiness, or facial pain
- palpable purpura and petechiae
- wheeze
- hemoptysis
- skin nodules
Other diagnostic factors
- use of certain medications
- fatigue, arthralgias, myalgias
- shortness of breath or cough
- abdominal pain
- sensory or motor deficits
- tachypnea
- rales
- peripheral edema
- orthopnea
- hepatojugular reflux
Risk factors
- history of asthma, allergic rhinitis, or sinusitis
- use of certain medications
Diagnostic tests
1st tests to order
- CBC with differential
- serum antineutrophil cytoplasmic antibodies (ANCA)
- serum CRP
- erythrocyte sedimentation rate
- serum BUN and creatinine
- urinalysis
- pulmonary function test
- chest x-ray
- echocardiography
Tests to consider
- flow cytometry of peripheral blood
- molecular testing for FIP1L1/PDGFR alpha mutation
- serum IgE
- serum-specific IgE and IgG to Aspergillus fumigatus
- stool culture for ova and parasites
- Toxocara serology
- CT chest
- electromyogram
- bronchoscopy with bronchoalveolar lavage (BAL)
- biopsy of affected tissue
- lower extremity ultrasound
- CT angiography
- bone marrow biopsy
- thiopurine methyltransferase level
- HIV testing
Emerging tests
- cardiac MRI
- eotaxin-3
Treatment algorithm
nonsevere EGPA
severe EGPA
relapse following successful remission
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
Divulgaciones
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
Divulgaciones
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
Divulgaciones
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
Divulgaciones
RAO declares that he has no competing interests.
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Referencias
Artículos principales
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.Texto completo Resumen
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.Texto completo Resumen
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.Texto completo Resumen
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.Texto completo Resumen
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- Granulomatosis with polyangiitis (GPA) (formerly known as Wegener granulomatosis)
- Microscopic polyangiitis (MPA)
- Hypereosinophilic syndrome (HES)
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