Amengual Olga, Atsumi Tatsuya
a Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine , Hokkaido University , Sapporo , Japan.
Mod Rheumatol. 2018 May;28(3):409-416. doi: 10.1080/14397595.2018.1435988. Epub 2018 Feb 21.
The antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the occurrence of venous and arterial thromboses, often multiple, and obstetric-related adverse events in the presence of antiphospholipid antibodies (aPL). APS, first described in 1983, as thrombosis, abortion and cerebral disease, is nowadays recognised as a systemic disease with a wide constellation of clinical manifestations related to acute and chronic vascular lesions. The presence of aPL is the serological hallmark of APS representing a heterogeneous population of autoantibodies with many antigenic specificities directed to phospholipid-binding proteins, either alone or in combination with phospholipids. Many assays have been developed for aPL detection. Particularly, anticardiolipin antibodies, anti-β2-glycoprotein I antibodies and lupus anticoagulant are essential tools for APS diagnosis. The cumulative evidence indicates that aPL are pathogenic autoantibodies binding to target cells and promoting thrombosis and pregnancy complications through a wide range of pathological mechanisms not yet fully understood. Finally, the recognition of the important role of aPL to assess the individual risk of thrombosis or pregnancy complications has expanded the concept of aPL, and currently aPL profile is regarded as a major risk factor for clinical thrombotic events.
抗磷脂综合征（APS）是一种自身免疫性疾病，其特征为在存在抗磷脂抗体（aPL）的情况下出现静脉和动脉血栓形成（通常为多发性）以及与产科相关的不良事件。APS于1983年首次被描述为血栓形成、流产和脑部疾病，如今被认为是一种全身性疾病，具有与急性和慢性血管病变相关的广泛临床表现。aPL的存在是APS的血清学标志，代表了一类异质性自身抗体群体，具有许多针对磷脂结合蛋白的抗原特异性，可单独或与磷脂结合。已经开发了许多用于检测aPL的检测方法。特别是，抗心磷脂抗体、抗β2糖蛋白I抗体和狼疮抗凝物是APS诊断的重要工具。累积证据表明，aPL是致病性自身抗体，可与靶细胞结合，并通过一系列尚未完全了解的病理机制促进血栓形成和妊娠并发症。最后，对aPL在评估个体血栓形成或妊娠并发症风险方面重要作用的认识扩展了aPL的概念，目前aPL谱被视为临床血栓事件的主要危险因素。
