Department of Clinical Chemistry and Haematology, University Medical Center, Heidelberglaan 100, Utrecht, The Netherlands.
Blood. 2012 Jul 12;120(2):266-74. doi: 10.1182/blood-2012-03-378646. Epub 2012 May 2.
The antiphospholipid syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies in patients with a history of thrombosis and/or pregnancy morbidity, including fetal loss. APS is an autoimmune disease with a confusing name because the pathologic auto-antibodies are shown to be directed against the plasma protein β(2)-glycoprotein I and not against phospholipids. In fact, auto-antibodies that recognize phospholipids themselves are not associated with thrombosis but with infectious diseases. One of the intriguing questions is why autoantibodies against β(2)-glycoprotein I are so commonly found in both patients and the healthy. Several potential mechanisms have been suggested to explain the increased thrombotic risk in patients with these autoantibodies. In this overview, we will summarize our knowledge on the etiology of the autoantibodies, and we will discuss the evidence that identify autoantibodies against β(2)-glycoprotein I as the culprit of APS.
抗磷脂综合征（APS）定义为有血栓形成和/或妊娠并发症病史（包括胎儿丢失）的患者中持续存在抗磷脂抗体。APS 是一种自身免疫性疾病，其名称令人困惑，因为病理性自身抗体被证明针对血浆蛋白β(2)-糖蛋白 I，而不是针对磷脂。事实上，自身抗体识别自身磷脂与血栓形成无关，而是与传染病有关。一个有趣的问题是，为什么针对β(2)-糖蛋白 I 的自身抗体在患者和健康人群中如此常见。已经提出了几种潜在的机制来解释这些自身抗体患者血栓形成风险增加的原因。在本综述中，我们将总结我们对自身抗体病因的认识，并讨论将针对β(2)-糖蛋白 I 的自身抗体确定为 APS 罪魁祸首的证据。
