University Medical Centre, Department of Clinical Chemistry and Haematology, Utrecht, the Netherlands.
Thromb Res. 2011 Feb;127 Suppl 3:S40-2. doi: 10.1016/S0049-3848(11)70011-1.
The antiphospholipid syndrome is an autoimmune disease characterised by the clinical features of recurrent thrombosis in the venous or arterial circulation and foetal losses in combination with circulating anti-phospholipid antibodies in the blood of the afflicted patients. Over the last 25 years numerous studies have established the correlation between the presence of antibodies against anionic phospholipids and thrombo-embolic manifestations but how these antibodies cause thrombosis is still unclear. Most scientists now accept the fact that only a subset of the antiphospholipid antibodies has clinical relevance. Not antibodies to anionic phospholipids but rather antibodies to β2-glycoprotein I are thought to be the major cause for the pathological manifestations. β2-Glycoprotein I is a plasma protein without a known function and persons lacking β2-Glycoprotein I are completely healthy. Our challenge is to understand why auto-antibodies against such a dispensable protein are so common and how antibodies directed against a protein without obvious function can induce the severe clinical manifestations observed in this syndrome.
抗磷脂综合征是一种自身免疫性疾病，其特征是静脉或动脉循环中反复发生血栓形成和胎儿丢失，同时伴有受累患者血液中循环抗磷脂抗体。在过去的 25 年中，许多研究已经确立了抗阴离子磷脂抗体的存在与血栓栓塞表现之间的相关性，但这些抗体如何引起血栓形成仍不清楚。大多数科学家现在接受这样一个事实，即只有抗磷脂抗体的一个子集具有临床相关性。引起病理表现的主要原因不是抗阴离子磷脂抗体，而是抗β2-糖蛋白 I 抗体。β2-糖蛋白 I 是一种血浆蛋白，没有已知的功能，缺乏β2-糖蛋白 I 的人完全健康。我们的挑战是要了解为什么针对如此可有可无的蛋白质的自身抗体如此普遍，以及针对没有明显功能的蛋白质的抗体如何能引起这种综合征中观察到的严重临床表现。
