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灾难性抗磷脂综合征的治疗与预后考量

Therapeutic and prognostic considerations in catastrophic antiphospholipid syndrome.

作者信息

Erkan Doruk

机构信息

The Barbara Volcker Center for Women and Rheumatic Disease, Division of Rheumatology, Hospital for Special Surgery, Weill Medical College of Cornell University, 535 E70th Street, New York, NY 10021, USA.

出版信息

Autoimmun Rev. 2006 Dec;6(2):98-103. doi: 10.1016/j.autrev.2006.06.013. Epub 2006 Jul 21.

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a rapidly progressive life-threatening disease that causes multiple organ thromboses in the presence of antiphospholipid antibodies. High index of clinical suspicion and careful investigation are required to make an early diagnosis so that treatment with anticoagulation, corticosteroids, and plasma exchange or intravenous immunoglobulins can be initiated. Despite this multi-modal treatment, CAPS is associated with high mortality; evidence-based management recommendations do not exist due to the rarity of the condition and the lack of controlled studies. This article reviews the therapeutic and prognostic controversies that were addressed during the 1st International Symposium on CAPS.

摘要

灾难性抗磷脂综合征(CAPS)是一种迅速进展的危及生命的疾病,在存在抗磷脂抗体的情况下会导致多器官血栓形成。早期诊断需要高度的临床怀疑指数和仔细的检查,以便能够开始使用抗凝剂、皮质类固醇以及血浆置换或静脉注射免疫球蛋白进行治疗。尽管采用了这种多模式治疗,CAPS的死亡率仍然很高;由于该疾病罕见且缺乏对照研究,目前尚无基于证据的管理建议。本文回顾了在第一届CAPS国际研讨会上讨论的治疗和预后争议。

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