15th International Congress on Antiphospholipid Antibodies Task Force on Catastrophic Antiphospholipid Syndrome Report
Catastrophic antiphospholipid syndrome (CAPS) is a systemic disorder, resulting in both thrombotic and non-thrombotic events. Thrombosis manifests mainly as thrombotic microangiopathy (TMA), although large-vessel thrombosis occurs in up to one third of patients. Non-thrombotic responses include activation of inflammatory processes, such as systemic inflammatory response syndrome or acute respiratory distress syndrome. Specific conditions may trigger these cascades in susceptible individuals who have acquired antiphospholipid antibodies (aPL). Several inflammatory cascades are hypothesized, including NF-kB activation by Toll-like receptor and the complement system, but so far no specific mechanism or defect has been identified as the main cause, and no common molecular pathway has been found specifically to cause the syndrome. During the 15th International Congress on aPL in 2016, the “Task Force on CAPS ” members presented updated evidence and relevant literature in their areas of expertise.