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Presse Med. 2012 Oct;41(10):1004-13. doi: 10.1016/j.lpm.2012.07.008. Epub 2012 Aug 23.

[Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)].

[Article in French]

Author information

1
Assistance publique-Hôpitaux de Paris, hôpital Cochin, université Paris-Descartes, service de médecine interne, Paris, France. loic.guillevin@cch.aphp.fr

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), is a rare necrotizing vasculitis of small-sized vessels, associated to antimyeloperoxydase ANCA in 40% of patients. EGPA occurs in patients with asthma. Asthma is sever, associated with eosinophilia and extrapulmonary symptoms. Among them, mononeuritis multiplex is the most frequent symptom. When cardiac involvement is present, prognosis is poor. Despite a good overall prognosis, deaths are caused by vasculitis activity, gastrointestinal and cardiac involvement. Treatment is well codified based on steroids, which are quickly effective. Immunosuppressants combined with corticosteroids are compulsory to treat the most sever forms, mainly when cardiac and gastrointestinal or renal symptoms are present.

PMID:
22921086
DOI:
10.1016/j.lpm.2012.07.008
[Indexed for MEDLINE]
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