Review article The significance of autoantibodies against 2-glycoprotein I

The antiphospholipid syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies in patients with a history of thrombosis and/or preg-nancy morbidity, including fetal loss. APS is an autoimmune disease with a confus-ing name because the pathologic auto-antibodies are shown to be directed against the plasma protein 2-glycoprotein I and not against phospholipids. In fact, auto-antibodies that recognize phospholipids themselves are not associated with throm-bosis but with infectious diseases. One of the intriguing questions is why autoanti-bodies against 2-glycoprotein I are so commonly found in both patients and the healthy. Several potential mechanisms have been suggested to explain the in-creased 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 2-glycoprotein I as the culprit of APS. (Blood. 2012; 120(2):266-274