sirolimus and defibrotide

Catastrophic antiphospholipid syndrome (CAPS) is a potentially lethal disease that presents with rapidly progressive multiple organ thromboses. Anticoagulation, corticosteroids, intravenous immunoglobulin, and plasma exchange are the most commonly used treatments for CAPS patients. However, the high mortality despite these medications necessitates new treatment strategies. Following a brief review of current diagnostic and management strategies, we discuss the candidate therapies, i.e., hydroxychloroquine, rituximab, eculizumab, sirolimus, and defibrotide, that can be considered in CAPS patients refractory to traditional treatment.

Topics: Antibodies, Monoclonal, Humanized; Antiphospholipid Syndrome; Antirheumatic Agents; Catastrophic Illness; Humans; Hydroxychloroquine; Immunosuppressive Agents; Patient Care Team; Plasma Exchange; Platelet Aggregation Inhibitors; Polydeoxyribonucleotides; Rituximab; Sirolimus