Update on the Diagnosis, Treatment, and Prognosis of the Catastrophic Antiphospholipid Syndrome

The catastrophic antiphospholipid syndrome (APS) is a potentially life-threatening condition, the diagnosis of which requires a high degree of clinical awareness on the part of attending physicians. Patients with APS present with 1) clinical evidence of multiple organ involvement developed over a very short time; 2) histopathologic evidence of multiple small-vessel occlusions; and 3) laboratory confirmation of the presence of antiphospholipid antibodies, usually in high titer. A combination of anticoagulants, corticosteroids, intravenous immunoglobulins, and plasma exchanges is the basic treatment for all patients with this severe condition. Unfortunately, despite current therapies, the mortality rate is still high (around 30%). However, once patients with catastrophic APS have recovered, they usually follow a stable course with continued anticoagulation and few patients present with a relapse of the catastrophic episode.