Treatment and prognosis 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 [1-6]. 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 diagnosis of EGPA is typically suspected based on the clinical findings (ie, eosinophilia ≥1500/microL, asthma, and allergic rhinitis). A positive antineutrophil cytoplasmic antibody test is present in 40 to 60 percent, but is neither sensitive nor specific. The diagnosis is generally confirmed by a biopsy of an affected tissue, such as lung, skin, or peripheral nerve [1-4] (table 1).
The treatment and prognosis of EGPA will be reviewed here. The epidemiology, pathogenesis, clinical features, and diagnosis of this disorder are discussed separately. (See "Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Clinical features and diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)".)
ASSESSING VASCULITIS SEVERITY
Two scoring systems have been developed to assess vasculitis disease activity in patients with eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) and other vasculitides: the "five-factors score" and the Birmingham Vasculitis Activity Score (BVAS). These scoring systems are used to guide initial therapy.
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- ASSESSING VASCULITIS SEVERITY
- INITIATING THERAPY
- Systemic glucocorticoids
- MAINTENANCE AND GLUCOCORTICOID SPARING THERAPY
- Inhaled glucocorticoids
- OTHER THERAPIES
- Mycophenolate mofetil
- Intravenous immune globulin
- Anti-IgE therapy
- Anti-IL-5 antibodies
- Plasma exchange
- PREVENTION OF TREATMENT RELATED COMPLICATIONS
- SPECIAL CONSIDERATIONS
- Upper airway involvement
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS