Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Talmadge E King, Jr, MD
Talmadge E King, Jr, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Interstitial Lung Disease
- Dean, School of Medicine
- Vice Chancellor, Medical Affairs
- University of California San Francisco
- Section Editors
- Kevin R Flaherty, MD, MS
Kevin R Flaherty, MD, MS
- Section Editor — Interstitial Lung Disease
- Associate Professor of Medicine
- University of Michigan Health System
- Richard J Glassock, MD, MACP
Richard J Glassock, MD, MACP
- Editor-in-Chief — Nephrology
- Section Editor — Glomerular Diseases
- Emeritus Professor
- The David Geffen School of Medicine at UCLA
- Bruce S Bochner, MD
Bruce S Bochner, MD
- Editor-in-Chief — Allergy and Immunology
- Section Editor — Adult Allergy; Asthma
- Samuel M Feinberg Professor of Medicine
- Northwestern University Feinberg School of Medicine
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss), abbreviated EGPA, which was previously called the Churg-Strauss syndrome (CSS) or allergic granulomatosis and angiitis, is a multisystem disorder characterized by chronic rhinosinusitis, asthma, and prominent peripheral blood eosinophilia [1-7]. EGPA is classified as a vasculitis of the small and medium sized arteries, although the vasculitis is often not apparent in the initial phases of the disease.
The most commonly involved organ is the lung, followed by the skin. EGPA, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems. Vasculitis of extrapulmonary organs is largely responsible for the morbidity and mortality associated with EGPA.
The clinical features and diagnosis of EGPA will be reviewed here. The epidemiology, pathogenesis, treatment, and prognosis of this disorder, as well as the approach to patients with vasculitis and/or eosinophilia are discussed separately. (See "Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Overview of and approach to the vasculitides in adults" and "Approach to the patient with unexplained eosinophilia".)
Phases of disease — The clinical features of EGPA typically develop in several sequential phases, although these phases are not always clearly distinguishable [8,9]:
●Prodromal phase – The prodromal phase occurs among individuals in the second and third decades of life and is characterized by atopic disease, allergic rhinitis, and asthma.
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- CLINICAL FEATURES
- Phases of disease
- Asthma and lung disease
- Upper airway and ear disease
- Thromboembolic disease
- Gastrointestinal tract
- Complications in pregnancy
- EVALUATION AND DIAGNOSIS
- Laboratory tests
- - Eosinophilia
- - Antineutrophil cytoplasmic antibodies
- - Acute phase reactants
- - Other laboratory tests
- Pulmonary function tests
- Bronchoalveolar lavage
- Cardiovascular tests
- DIAGNOSTIC CRITERIA
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS