Classic pathologic triad of tissue eosinophilia, granulomatous inflammation, and vasculitis.
The cornerstone of treatment is corticosteroids. Severe disease is treated in a similar manner to other antineutrophil cytoplasmic antibody associated vasculitides, with additional immunosuppressive agents.
Cardiac involvement causes significant morbidity and mortality.
Typically patients are initially diagnosed with asthma, and then months to years later develop manifestations due to tissue eosinophilia, followed by vasculitis.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of primary systemic autoimmune vasculitis characterized by inflammation of blood vessels. In EGPA, vasculitis is associated with asthma and eosinophilia. EGPA is also known as Churg-Strauss syndrome (CSS). Affected people may have perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) directed against myeloperoxidase (MPO). These autoantibodies are more common in patients with more vasculitic manifestations, such as glomerulonephritis.
History and exam
- 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
- bronchoscopy with bronchoalveolar lavage (BAL)
- biopsy of affected tissue
- lower extremity ultrasound
- CT angiography
- bone marrow biopsy
- thiopurine methyltransferase level
- HIV testing
Karina Keogh, MBBCh
Division of Pulmonary and Critical Care Medicine
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). KK has undertaken contractual research for GlaxoSmithKline (utilizing mepolizumab and sirukumab in asthma trials), but received no personal financial gain for this work.
Dr Karina Keogh would like to gratefully acknowledge Dr Garvan Kane, a previous contributor to this topic. GK is an author of a number of references cited in this topic.
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
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
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
Johns Hopkins Vasculitis Center
PS declares that he has no competing interests.
Jaap Van Laar, MD, PhD
Professor of Clinical Rheumatology
Institute of Cellular Medicine
Newcastle upon Tyne
Honorary Consultant Rheumatologist
The James Cook University Hospital
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
RAO declares that he has no competing interests.
- More guidelines
Use of this content is subject to our disclaimer