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Clinical features and diagnosis 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 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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Literature review current through: Nov 2017. | This topic last updated: Sep 12, 2017.
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