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Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Talmadge E King, Jr, MD
Section Editors
Kevin R Flaherty, MD, MS
Richard J Glassock, MD, MACP
Bruce S Bochner, MD
Deputy Editor
Helen Hollingsworth, MD


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)".)


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-factor score" and the Birmingham Vasculitis Activity Score (BVAS). These scoring systems are used to guide initial therapy.

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Literature review current through: Nov 2017. | This topic last updated: Jul 18, 2017.
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