Catastrophic Anti-Phospholipid Syndrome (CAPS) is a rare autoimmune disorder characterized by widespread small vessel thromboembolic events in multiple organs. There are four diagnostic criteria:
1) presence of antiphospholipid antibodies
2) histopathological evidence of small vessel occlusion
3) involvement of 3 or more organ systems
4) development of manifestations in <1 week
A triple therapy approach of anticoagulation, corticosteroids, and therapeutic plasma exchange (TPE) or IVIG has been shown to decrease mortality. However, there are no prospective trials exist to guide chronic management, and the optimal timing and frequency of these treatments is unknown. We present the case of a patient with definitive and recurrent CAPS, and our experience with the chronic management of this rare condition.
