Immunology of the antiphospholipid antibody syndrome.
Immunology of the antiphospholipid antibody syndrome.
复制标题
抗磷脂抗体综合征的免疫学。
DOI：
10.1002/art.1780390903
复制
发表时间：
1996
影响因子：
--
通讯作者：
Roubey,RA
中科院分区：
文献类型：
--
作者：
Roubey,RA
就像披头士乐队的《洛基浣熊》中的蛇蝎美人一样，抗磷脂抗体（aPL）及其相关的综合征（血栓形成、复发性胎儿死亡、血小板减少症）尽管存在严重的命名问题，但仍引起了相当大的关注。沿着对aPL综合征患者的最佳管理经验的增加和自身抗体可能发挥直接病理生理作用的证据的增加，过去几年的研究表明，大部分“抗磷脂”自身抗体实际上不识别磷脂。这些新的数据表明，在常规抗心磷脂和狼疮抗凝剂测定中检测到的抗体的抗原靶标是磷脂结合血浆蛋白，最值得注意的是β-糖蛋白I（&GPI）和凝血酶原或这些蛋白质与磷脂的复合物。此外，其他磷脂结合血浆蛋白和血管内皮上表达的某些分子的自身抗体也可能与抗磷脂抗体综合征（APS）相关，尽管它们在标准aPL测定中不可检测。本文综述了与APS相关的自身抗体的特异性的最新信息，并讨论了这些数据提供的见解到综合征的病理生理学的自身抗体的潜在作用。
Like the femme fatale in The Beatles’ Rocky Raccoon, antiphospholipid antibodies (aPL) and the syndrome with which they are associated (thrombosis, recurrent fetal deaths, thrombocytopenia) have attracted considerable attention despite a serious nomenclature problem. Along with increasing experience in the optimal management of patients with the aPL syndrome and growing evidence that autoantibodies may play a direct pathophysiologic role, research over the past several years demonstrates that a large proportion of “antiphospholipid” autoantibodies do not, in fact, recognize phospholipids. These new data indicate that the antigenic targets of antibodies detected in conventional anticardiolipin and lupus anticoagulant assays are phospholipid-binding plasma proteins, most notably, &-glycoprotein I (&GPI) and prothrombin or complexes of these proteins with phospholipids. Further, autoantibodies to other phospholipid-binding plasma proteins and certain molecules expressed on vascular endothelium may also be associated with the antiphospholipid antibody syndrome (APS) although they are not detectable in standard aPL assays. This review summarizes recent information on the specificities of autoantibodies associated with APS and discusses the insights these data offer into the potential role of autoantibodies in the syndrome’s pathophysiology.