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灾难性抗磷脂综合征:一种潜在致命疾病的候选治疗方法。

Catastrophic Antiphospholipid Syndrome: Candidate Therapies for a Potentially Lethal Disease.

机构信息

Hospital for Special Surgery, New York, NY 10021; email:

Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, NY 10021.

出版信息

Annu Rev Med. 2017 Jan 14;68:287-296. doi: 10.1146/annurev-med-042915-102529.

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a potentially lethal disease that presents with rapidly progressive multiple organ thromboses. Anticoagulation, corticosteroids, intravenous immunoglobulin, and plasma exchange are the most commonly used treatments for CAPS patients. However, the high mortality despite these medications necessitates new treatment strategies. Following a brief review of current diagnostic and management strategies, we discuss the candidate therapies, i.e., hydroxychloroquine, rituximab, eculizumab, sirolimus, and defibrotide, that can be considered in CAPS patients refractory to traditional treatment.

摘要

灾难性抗磷脂综合征(CAPS)是一种潜在致命的疾病,表现为快速进展的多器官血栓形成。抗凝、皮质类固醇、静脉注射免疫球蛋白和血浆置换是 CAPS 患者最常用的治疗方法。然而,尽管使用了这些药物,死亡率仍然很高,这就需要新的治疗策略。在简要回顾了目前的诊断和治疗策略之后,我们讨论了候选治疗方法,即在传统治疗无效的 CAPS 患者中可以考虑使用羟氯喹、利妥昔单抗、依库珠单抗、西罗莫司和地昔单抗。

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