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Epidemiology, pathogenesis, and pathology of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author
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

INTRODUCTION

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".)

EPIDEMIOLOGY

The epidemiology of EGPA remains unclear because of the uncertainties related to diagnosis [10]. 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 [5]. (See "Overview of and approach to the vasculitides in adults".)

The mean age at diagnosis of EGPA is 40 years [11]. 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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Literature review current through: Nov 2016. | This topic last updated: Mon Jul 11 00:00:00 GMT 2016.
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