Islam Md Asiful, Alam Fahmida, Sasongko Teguh Haryo, Gan Siew Hua
Human Genome Centre, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia.
Curr Pharm Des. 2016;22(28):4451-69. doi: 10.2174/1381612822666160527160029.
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by a persistently high titer of antiphospholipid antibodies (aPLs). In addition to pregnancy morbidity, arterial and/or venous thrombosis is another clinical feature of APS. Regardless of the type of APS, the thrombi formed by the induction of aPLs can lead to deep vein thrombosis, pulmonary embolism, myocardial infarction, stroke and gangrene. Although the concept of APS was introduced approximately 32 years ago, its thrombogenic pathophysiology is still unclear. Therefore, patients are treated with anticoagulant and/or antiplatelet regimens just as in other thrombotic disorders even though the thrombotic pathophysiology is mainly aPLs-mediated. In this review, we provided an update of the cellular, auto-immune and genetic factors known to play important roles in the generation of thrombi. Current successful regimens are also outlined along with potential emerging treatment strategies that may lead to the optimum management of thrombotic APS patients.
抗磷脂综合征（APS）是一种系统性自身免疫性疾病，其特征为抗磷脂抗体（aPLs）持续高滴度存在。除了妊娠并发症外，动脉和/或静脉血栓形成是APS的另一临床特征。无论APS的类型如何，由aPLs诱导形成的血栓可导致深静脉血栓形成、肺栓塞、心肌梗死、中风和坏疽。尽管APS的概念大约在32年前就已提出，但其血栓形成的病理生理学仍不清楚。因此，尽管血栓形成的病理生理学主要由aPLs介导，但患者仍像其他血栓性疾病一样接受抗凝和/或抗血小板治疗方案。在本综述中，我们提供了已知在血栓形成中起重要作用的细胞、自身免疫和遗传因素的最新信息。还概述了当前成功的治疗方案以及可能导致血栓性APS患者最佳管理的潜在新兴治疗策略。
