title: Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease.
abstract: 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.
authors.affiliation: Hospital for Special Surgery, New York, NY 10021; email: unluo@hss.edu.
authors.forename: Ozan
authors.initials: O
authors.lastname: Unlu
authors.affiliation: Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY 10021.
authors.forename: Doruk
authors.initials: D
authors.lastname: Erkan
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000188:drug therapy* / Q000628:therapy; D018501:Antirheumatic Agents / Q000627:therapeutic use; D002388:Catastrophic Illness / Q000628:therapy; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010348:Patient Care Team; D010951:Plasma Exchange; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011089:Polydeoxyribonucleotides / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use; D020123:Sirolimus / Q000627:therapeutic use
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D018501:Antirheumatic Agents; D007166:Immunosuppressive Agents; D010975:Platelet Aggregation Inhibitors; D011089:Polydeoxyribonucleotides; C036901:defibrotide; D000069283:Rituximab; D006886:Hydroxychloroquine; C481642:eculizumab; D020123:Sirolimus
keywords: CAPS; antiphospholipid syndrome; defibrotide; eculizumab; hydroxychloroquine; rituximab; sirolimus