Catastrophic antiphospholipid syndrome

Abstract

The catastrophic antiphospholipid syndrome is a potentially life-threatening condition with a high mortality, which requires a high degree of clinical awareness on the part of attending physicians. Patients with this syndrome have in common: a) clinical evidence of multiple organ involvement developed over a very short time period; b) histopathological evidence of multiple small vessel occlusions, and c) laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titre. The combination of high doses of intravenous (iv) heparin, iv steroids, iv gamma globulins and/or repeated plasma exchanges is the basic treatment of choice for all patients with this severe condition.