Epidemiology, pathogenesis, and pathology 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 allergic rhinitis, asthma, and prominent peripheral blood eosinophilia [1-9]. 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 epidemiology, pathogenesis, and pathology of EGPA will be reviewed here. The clinical features, diagnosis, treatment and prognosis of this disorder, as well as the approach to patients with vasculitis and/or eosinophilia are discussed separately. (See "Clinical features and diagnosis 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".)
The epidemiology of EGPA remains unclear because of the uncertainties related to diagnosis . Approximately 10 percent of patients with a major form of vasculitis are recognized to have EGPA. Among the three anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (EGPA, granulomatosis with polyangiitis (Wegener's), and microscopic polyangiitis), EGPA is least common . (See "Overview of and approach to the vasculitides in adults".)
The mean age at diagnosis of EGPA is 40 years . EGPA is an uncommon cause of vasculitis in people older than 65 years, accounting for 5 percent of histologically proven vasculitis among 38 elderly patients with various systemic forms of angiitis [12,13]. EGPA is also rare in children and adolescents; when it does occur in this age group, it appears to follow a more aggressive course with prominent pulmonary and cardiovascular manifestations [14-16].
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