Antiphospholipid Syndrome and Pregnancy Pathogenesis to Translation
Antiphospholipid Syndrome and Pregnancy Pathogenesis to Translation
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DOI：
10.1002/art.40136
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发表时间：
2017-09-01
影响因子：
13.3
通讯作者：
Salmon, Jane E.
中科院分区：
文献类型：
--
作者：
Abrahams, Vikki M.;Chamley, Lawrence W.;Salmon, Jane E.
抗磷脂综合征（APS）是一种与血栓形成（静脉或动脉）和不良妊娠结局相关的自身免疫性疾病。APS的研究始于1906年在梅毒患者中发现自身抗体，其特征在于1952年由于与抗心磷脂反应而导致的生物假阳性，随后不久在具有不良妊娠结局的女性中鉴定出“循环抗凝剂”。在20世纪80年代，血栓形成、流产和狼疮抗凝剂的关联被发现，并且术语“抗磷脂综合征”被创造。APS的诊断基于临床产科和/或血栓形成史以及抗磷脂抗体（aPL）持续阳性（两次至少间隔12周）的实验室发现（表1）。抗磷脂抗体是针对由带负电荷的磷脂和磷脂结合蛋白组成的复合抗原的自身抗体的异质组。其中公认最好的，以及诊断APS所需的aPL是狼疮抗凝剂（LAC）、抗心磷脂抗体（aCL）和抗β2-糖蛋白-I（aβ2GPI）抗体。1 aCL和aβ2GPI aPL免疫测定的性能特征以及aPL结果中公认的实验室间变异性要求可靠的实验室鉴定中等或高滴度结果（表1）。此外，aCL和α β2GPI抗体对APS的特异性随着更高滴度和IgG同种型而增加。存在重复阳性结果的要求，因为其他条件可能导致一过性阳性aPL结果。LAC的存在，以及在某些系列中aβ2GPI抗体的存在，是比aCL抗体更好的妊娠发病率预测因子。2，3 aPL“三重”阳性（LAC、aCL和α β2GPI）比双重或单一aPL阳性具有更大的临床意义。4
The antiphospholipid syndrome (APS) is an autoimmune condition associated with thrombosis (venous or arterial) and adverse pregnancy outcomes. Investigation of APS began with the discovery of autoantibodies in patients with syphilis in 1906, characterized as biologic false-positives due to reactivity with anticardiolipin in 1952, and followed soon after by the identification of “circulating anticoagulants” in women with adverse pregnancy outcomes. In the 1980’s, the association of thrombosis, miscarriages, and lupus anticoagulant was discovered, and the term “antiphospholipid syndrome” was coined.The diagnosis of APS is based on clinical obstetric and/or thrombotic history and laboratory findings of persistently-positive (on two occasions at least 12 weeks apart) antiphospholipid antibodies (aPL)(Table 1). Antiphospholipid antibodies are a heterogeneous group of autoantibodies directed against a complex antigen consisting of negatively-charged phospholipids and phospholipid binding proteins. The best recognized of these, and the aPL required to make a diagnosis of APS, are lupus anticoagulant (LAC), anticardiolipin antibodies (aCL) and anti-β2-glycoprotein-I (aβ2GPI) antibodies. 1 The performance characteristics of the aPL immunoassays for aCL and aβ2GPI and the widely-recognized inter-laboratory variability in aPL results demands that reliable laboratories identify medium-or high-titer results (Table 1). Furthermore, the specificity of aCL and aβ2GPI antibodies for APS increases with higher titers and with IgG isotype. The requirement for repeatedly positive results exists because other conditions can result in transiently positive aPL results. The presence of LAC, and in some series aβ2GPI antibodies, are better predictors of pregnancy morbidity than aCL antibodies. 2, 3 “Triple” aPL positivity (LAC, aCL, and aβ2GPI) is of greater clinical significance than double or single aPL positivity. 4