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深入了解“非标准”产科抗磷脂综合征的异质性临床特征:两例病例报告及文献复习。

An advanced understanding of the heterogeneous clinical features of "non-criteria" obstetric antiphospholipid syndrome: Two case reports and a literature review.

机构信息

Department of Obstetrics and Gynecology, West China Second University Hospital, Sichuan University, Chengdu, China.

Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, China.

出版信息

Front Immunol. 2023 Feb 14;14:1122127. doi: 10.3389/fimmu.2023.1122127. eCollection 2023.

Abstract

Antiphospholipid syndrome (APS) is an acquired autoimmune disorder characterized by recurrent venous and/or arterial thrombosis and/or pregnancy complications, in the presence of elevated antiphospholipid (aPL) antibodies. APS in pregnant women is referred to as "obstetrical" APS (OAPS). The diagnosis of definite OAPS requires the presence of one or more typical clinical criteria and persistent aPL antibodies at least 12 weeks apart. However, the classification criteria for OAPS have generated wide discussion, with a growing impression that certain patients not fully meeting these criteria might be inappropriately excluded from the classification, which is known as "non-criteria" OAPS. We present here two unique cases of potentially lethal "non-criteria" OAPS, complicating severe preeclampsia, fetal growth restriction (FGR), liver rupture, preterm birth, refractory recurrent miscarriages, or even stillbirth. We further share our diagnostic search and analysis, treatment adjustment, and prognosis for this unusual antenatal event. We will also present a short review of an advanced understanding of the pathogenetic mechanisms of this disease, heterogeneous clinical features, and potential significance.

摘要

抗磷脂综合征(APS)是一种获得性自身免疫性疾病,其特征是存在升高的抗磷脂(aPL)抗体时,反复发生静脉和/或动脉血栓形成和/或妊娠并发症。孕妇中的 APS 称为“产科”APS(OAPS)。明确的 OAPS 诊断需要存在一个或多个典型的临床标准和至少相隔 12 周的持续存在 aPL 抗体。然而,OAPS 的分类标准引起了广泛的讨论,越来越多的人认为,某些不完全符合这些标准的患者可能被不恰当地排除在分类之外,这被称为“非标准”OAPS。我们在此介绍了两种可能致命的“非标准”OAPS 的独特病例,它们与严重子痫前期、胎儿生长受限(FGR)、肝破裂、早产、难治性反复流产甚至死胎有关。我们进一步分享了我们对这种不寻常的产前事件的诊断搜索和分析、治疗调整和预后。我们还将简要回顾对这种疾病的发病机制、异质性临床特征和潜在意义的深入了解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a923/9971720/ed9d05620357/fimmu-14-1122127-g001.jpg

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