• Doruk Erkan, Gerard Espinosa, and Ricard Cervera.
• The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY, USA.
• Autoimmun Rev. 2010 Dec 1; 10 (2): 74-9.

AbstractThe 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.Copyright © 2010 Elsevier B.V. All rights reserved.

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