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;10(2):74-9. doi: 10.1016/j.autrev.2010.08.005. Epub 2010 Aug 7.
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.
灾难性抗磷脂综合征 (APS) 是 APS 中最严重的形式，具有急性多器官受累和小血管血栓形成。在第 13 届国际抗磷脂抗体大会 (aPL) 上，成立了一个“灾难性 APS 工作组”，以讨论灾难性 APS 的诊断和管理挑战。本文的目的是总结诊断挑战（假阳性/阴性 aPL 结果和与其他血栓性微血管病的重叠），并提出灾难性 APS 的更新诊断算法。诊断算法的重要步骤包括：a）APS 病史或持续的 aPL 阳性；b）不到一周内出现三个或更多新器官血栓形成；c）微血栓的活检诊断；d）对多个器官血栓形成和/或微血栓形成的其他解释。
