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Churg-Strauss Syndrome

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Resumo:Churg-Strauss syndrome is a systemic ANCA-associated small- to medium-sized vessels vasculitis. Common clinical manifestations are marked blood eosinophilia, asthma, chronic sinusitis, cardiomyopathy, pulmonary infiltrates, gastrointestinal complaints and a multiplex neuropathy. Anti-MPO (antimyeloperoxidase) pANCA (ANCA with a perinuclear fluorescence pattern) is present in 38-59% of cases. Cardiac involvement is an important cause of morbidity and the leading cause of mortality in Churg-Strauss syndrome. The morphological substrate is an eosinophilic necrotizing vasculitis. Treatment is based on corticosteroid therapy and immunosuppressive drugs (cyclophosphamide and azathioprine) and is determined according to prognostic criteria. Complete remission occurs in almost 90% of cases and relapses are frequent (25% of cases).
Autores principais:Rodrigues, B. F.
Outros Autores:Araújo, J. M.
Assunto:síndrome de Churg-Strauss ANCA vasculite Churg-Strauss syndrome ANCA vasculitis
Ano:2011
País:Portugal
Tipo de documento:artigo
Instituição associada:Sociedade Portuguesa de Medicina Interna
Idioma:português
Origem:Revista Portuguesa de Medicina Interna

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