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产科抗磷脂综合征:一种旧定义疾病的新分类。

Obstetric antiphospholipid syndrome: a recent classification for an old defined disorder.

机构信息

Department of Obstetrics and Gynecology, Università Cattolica del Sacro Cuore, Largo A. Gemelli 8, 00168 Rome, Italy.

Department of Clinical Sciences and Community Health, University of Milan, Istituto Auxologico Italiano, Milan, Italy.

出版信息

Autoimmun Rev. 2014 Sep;13(9):901-8. doi: 10.1016/j.autrev.2014.05.004. Epub 2014 May 10.

Abstract

Obstetric antiphospholipid syndrome (APS) is now being recognized as a distinct entity from vascular APS. Pregnancy morbidity includes >3 consecutive and spontaneous early miscarriages before 10weeks of gestation; at least one unexplained fetal death after the 10th week of gestation of a morphologically normal fetus; a premature birth before the 34th week of gestation of a normal neonate due to eclampsia or severe pre-eclampsia or placental insufficiency. It is not well understood how antiphospholipid antibodies (aPLs), beyond their diagnostic and prognostic role, contribute to pregnancy manifestations. Indeed aPL-mediated thrombotic events cannot explain the obstetric manifestations and additional pathogenic mechanisms, such as a placental aPL mediated complement activation and a direct effect of aPLs on placental development, have been reported. Still debated is the possible association between aPLs and infertility and the effect of maternal autoantibodies on non-vascular manifestations in the babies. Combination of low dose aspirin and unfractionated or low molecular weight heparin is the effective treatment in most of the cases. However, pregnancy complications, in spite of this therapy, can occur in up to 20% of the patients. Novel alternative therapies able to abrogate the aPL pathogenic action either by interfering with aPL binding at the placental level or by inhibiting the aPL-mediated detrimental effect are under active investigation.

摘要

产科抗磷脂综合征(APS)现在被认为是一种与血管 APS 不同的实体。妊娠发病率包括>3 次连续和自发性早期流产,发生在 10 周妊娠之前;至少有一次不明原因的胎儿死亡发生在 10 周妊娠之后的形态正常胎儿;由于子痫或严重子痫前期或胎盘功能不全,导致正常新生儿早产发生在 34 周妊娠之前。目前尚不清楚抗磷脂抗体(aPL)除了其诊断和预后作用外,如何导致妊娠表现。实际上,aPL 介导的血栓事件不能解释产科表现,并且已经报道了其他发病机制,例如胎盘 aPL 介导的补体激活和 aPL 对胎盘发育的直接作用。aPL 与不孕的可能相关性以及母体自身抗体对婴儿非血管表现的影响仍存在争议。低剂量阿司匹林与未分级或低分子肝素的联合是大多数情况下的有效治疗方法。然而,尽管进行了这种治疗,妊娠并发症仍可能发生在多达 20%的患者中。能够通过干扰胎盘水平的 aPL 结合或通过抑制 aPL 介导的有害作用来消除 aPL 致病作用的新型替代疗法正在积极研究中。

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