Pathophysiology of the catastrophic antiphospholipid syndrome (CAPS)

Antiphospholipid antibodies (APA) are a common cause of acquired thrombophilia. APA recognize plasma phospholipid-binding proteins (e. g., beta(2)-glycoprotein I, prothrombin, annexin V, etc.). Catastrophic antiphospholipid syndrome (CAPS) is an uncommon variant of the antiphospholipid syndrome. CAPS patients often present with multiorgan failure. Precipitating factors include surgical procedures, drugs, and discontinuation of anticoagulant therapy. Increasingly, infections are recognized as a major precipitating condition. The majority of patients present with renal involvement as well as evidence of acute respiratory distress syndrome (ARDS). This review discusses the clinical and pathophysiologic aspects of CAPS as well as the differenital diagnosis.