Churg-Strauss Syndrome
Keywords:
Churg-Strauss syndrome, ANCA, vasculitisAbstract
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).
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