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Systemic capillary leak syndrome (SCLS) was first described in 1960, but fewer than 130 cases have been reported worldwide. Acute attacks typically develop within a few hours and result in severe hypotension, edema, hemoconcentration, and hypoalbuminemia. Researchers from Paris present a case series of 28 SCLS patients (seen from 1997 through 2010).
Median age at disease onset was 49, and median delay to diagnosis was 7 months. The median frequency of attacks was 1.2 annually, and the most common trigger was infection (74% of events). Patients received many prophylactic treatments, including intravenous immunoglobulin and β2-agonists. Eight patients (29%) died during a median follow-up of 55 months. Projected 5-year survival was 73%; it was …