系统性红斑狼疮的抗磷脂抗体综合征。

S Zmonarski, M Klinger
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引用次数: 0

摘要

抗磷脂抗体(APA)可在明显不相关的疾病过程中出现各种止血异常。在血清中检测到抗体的人群中,近一半患有系统性红斑狼疮(SLE)或将来将诊断出该疾病。考虑到APA不直接与磷脂结合的假说最近越来越流行。它们结合血清β 2-糖蛋白。apa - β 2 GIP复合物加速凝血酶原活化,使β 2 GIP较少用于血清C和s蛋白转化。APA是一种异质抗体。感染后APA不同于自身免疫性疾病。由APA引起的血栓事件是根据一般原则治疗的。类固醇治疗在系统性红斑狼疮合并APA的治疗方案中是必不可少的。在严重的情况下,通过静脉注射环磷酰胺补充。血浆置换和静脉注射免疫球蛋白的作用有限。在严重抵抗性血小板减少症患者中,可以谨慎地尝试引入那那唑。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Antiphospholipid antibody syndrome in systemic lupus erythematosus].

Various hemostatic abnormalities that can be met in the course of apparently unrelated diseases are caused by antiphospholipid antibodies (APA). Nearly on half of the population with antibodies detectable in serum suffer from systemic lupus erythematosus (SLE) or the disease will be diagnosed in the future. The supposition considering that APA do not bind phospholipids directly becomes popular recently. They bind serum beta 2-Glycoprotein. The APA-beta 2 GIP complexes speed up prothrombin activation and make beta 2 GIP less available for serum C- and S-protein transformation. APA are a heterogenous population of antibodies. Postinfectious APA differ from those found in autoimmune diseases. Thrombotic events caused by APA are treated according to general principles. Steroid therapy is essential in treatment programme of SLE with APA. In severe cases it is supplemented by intravenous cyclophosphamide. Plasmapheresis and intravenous immunoglobulins are of limited usefulness. In severe resistant thrombocytopenia one can try to introduce Danazol carefully.

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