Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors.

In order to know the causes of death and the prognostic factors, our group analyzed 250 patients included until February 2005 in the web-site based international registry of patients with catastrophic antiphospholipid syndrome (APS) ("CAPS Registry") (http://www.med.ub.es/MIMMUN/FORUM/CAPS.HTM). Cerebral involvement, mainly consisting of stroke, followed by cardiac involvement and infections were considered the main causes of death in patients with catastrophic APS. The presence of systemic lupus erythematosus was related with higher mortality. According to the results of this analysis, anticoagulation plus steroids plus plasma exchange should be the first line of therapy in patients with catastrophic APS.