Pathophysiology of the catastrophic antiphospholipid syndrome (CAPS)

Antiphospholipid antibodies (APA) are a common cause of acquired thrombophi lia. APA recognize plasma phospholipid-binding proteins (e.g., beta(2)-glyc oprotein I, prothrombin, annexin V, etc.). Catastrophic antiphospholipid sy ndrome (CAPS) is an uncommon variant of the antiphospholipid syndrome. CAPS patients often present with multiorgan failure. Precipitating factors incl ude surgical procedures, drugs, and discontinuation of anticoagulant therap y. Increasingly, infections are recognized as a major precipitating conditi on. The majority of patients present with renal involvement as well as evid ence of acute respiratory distress syndrome (ARDS), This review discusses t he clinical and pathophysiologic aspects of CAPS as well as the differentia l diagnosis. (C) 2000 Wiley-Liss, Inc.