Harper Brock E, Wills Rohan, Pierangeli Silvia S
Department of Internal Medicine, Division of Rheumatology, University of texas medical branch, Galveston, TX, USA.
Int J Clin Rheumtol. 2011 Apr 1;6(2):157-171. doi: 10.2217/ijr.11.9.
Antiphospholipid syndrome is a systemic autoimmune disease associated with thrombosis and recurrent fetal loss in the setting of detectable antiphospholipid (aPL) antibodies. The major antigenic target has been identifed as β-glycoprotein I (βGPI), which mediates binding of aPL antibodies to target cells including endothelial cells, monocytes, platelets and trophoblasts, leading to prothrombotic and proinfammatory changes that ultimately result in thrombosis and fetal loss. This article summarizes recent insights into the role of βGPI in normal hemostasis, interactions between aPL antibodies, βGPI and cell-surface molecules, molecular prothrombotic and proinfammatory changes induced by aPL antibodies and pathogenic changes leading to fetal loss in antiphospholipid syndrome. New directions in therapy using these insights are examined.
抗磷脂综合征是一种全身性自身免疫性疾病，在可检测到抗磷脂（aPL）抗体的情况下，与血栓形成和反复流产有关。主要抗原靶点已被确定为β-糖蛋白I（βGPI），它介导aPL抗体与包括内皮细胞、单核细胞、血小板和滋养层细胞在内的靶细胞结合，导致促血栓形成和促炎变化，最终导致血栓形成和流产。本文总结了关于βGPI在正常止血中的作用、aPL抗体、βGPI与细胞表面分子之间的相互作用、aPL抗体诱导的分子促血栓形成和促炎变化以及抗磷脂综合征中导致流产的致病变化的最新见解。还探讨了利用这些见解进行治疗的新方向。
