title: Catastrophic antiphospholipid syndrome: updated diagnostic algorithms.
abstract: The catastrophic antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. During the 13th International Congress on Antiphospholipid Antibodies (aPL) a "Catastrophic APS Task Force" was developed to discuss the challenges in the diagnosis and management of catastrophic APS. The purpose of this paper is to summarize the diagnostic challenges (false-positive/negative aPL results and overlap with other thrombotic microangiopathies) and propose updated diagnostic algorithms for catastrophic APS. Important steps of the diagnostic algorithms include: a) history of APS or persistent aPL-positivity; b) three or more organ new thrombosis developing in less than a week; c) biopsy diagnosis of microthrombosis; and d) other explanations for multiple organ thromboses and/or microthrombosis.
authors.affiliation: The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY, USA.
authors.forename: Doruk
authors.initials: D
authors.lastname: Erkan
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
mesh_terms: D000465:Algorithms; D017152:Antibodies, Antiphospholipid / Q000097:blood; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D002388:Catastrophic Illness / Q000628:therapy; D006801:Humans
chemical_list: D017152:Antibodies, Antiphospholipid