title: Diagnosis and management of catastrophic antiphospholipid syndrome.
abstract: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening disease. In 1992, Asherson defined it as a widespread coagulopathy related to the antiphospholipid antibodies (aPL). CAPS requires rapid diagnosis and prompt initiation of treatment. Areas covered: This paper discusses all aspects of CAPS, including its pathophysiology, clinical manifestations, diagnostic approaches, differential diagnoses, management and treatment of relapsing CAPS, and its prognosis. To obtain the information used in this review, scientific databases were searched using the key words antiphospholipid antibodies, catastrophic antiphospholipid syndrome, hemolytic anemia, lupus anticoagulant, and thrombotic microangiopathic hemolytic anemia. Expert commentary: CAPS is a rare variant of the antiphospholipid syndrome (APS). It is characterized by thrombosis in multiple organs and a cytokine storm developing over a short period, with histopathologic evidence of multiple microthromboses, and laboratory confirmation of high aPL titers. This review discusses the diagnostic challenges and current approaches to the treatment of CAPS.
authors.affiliation: a Department of Medicine E , Meir Medical Center , Kfar Saba , Israel.|b Sackler Faculty of Medicine , Tel Aviv University , Ramat Aviv , Israel.
authors.forename: Or
authors.initials: O
authors.lastname: Carmi
authors.affiliation: a Department of Medicine E , Meir Medical Center , Kfar Saba , Israel.|b Sackler Faculty of Medicine , Tel Aviv University , Ramat Aviv , Israel.
authors.forename: Maya
authors.initials: M
authors.lastname: Berla
authors.affiliation: b Sackler Faculty of Medicine , Tel Aviv University , Ramat Aviv , Israel.|c The Zabludowicz Center for Autoimmune Diseases , Sheba Medical Center , Tel Hashomer , Israel.
authors.forename: Yehuda
authors.initials: Y
authors.lastname: Shoenfeld
authors.affiliation: a Department of Medicine E , Meir Medical Center , Kfar Saba , Israel.|b Sackler Faculty of Medicine , Tel Aviv University , Ramat Aviv , Israel.
authors.forename: Yair
authors.initials: Y
authors.lastname: Levy
mesh_terms: D000465:Algorithms; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D019468:Disease Management; D006801:Humans; D010641:Phenotype; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome
keywords: Antiphospholipid antibodies; catastrophic antiphospholipid syndrome; hemolytic anemia; lupus anticoagulant; thrombotic microangiopathic hemolytic anemia