Rand Jacob H
Department of Medicine, Mount Sinai School of Medicine, New York, New York 10029, USA.
Annu Rev Med. 2003;54:409-24. doi: 10.1146/annurev.med.54.101601.152412. Epub 2001 Dec 3.
The antiphospholipid (aPL) antibody syndrome is an autoimmune condition in which vascular thrombosis and/or recurrent pregnancy losses occur in patients with laboratory evidence for antibodies that bind to phospholipids. There have been significant advances in the recognition of the role of phospholipid-binding cofactors, primarily beta2GPI, as the true immunologic targets of the antibodies. Recent evidence suggests that the antibodies disrupt phospholipid-dependent anticoagulant mechanisms and/or that aPL antibodies induce the expression of procoagulant and proadhesive molecules on endothelial cells. Current diagnosis is based on clinical findings and empirically derived tests, such as assays for antibodies that bind to phospholipids or putative cofactors and coagulation assays that detect inhibition of phospholipid-dependent coagulation reactions. Current treatment relies primarily on anticoagulant therapy. Research advances are expected to bring mechanistically based diagnostic tests and improved therapy that target the roots of the disease process.
抗磷脂(aPL)抗体综合征是一种自身免疫性疾病,在有实验室证据表明存在与磷脂结合的抗体的患者中,会出现血管血栓形成和/或反复流产。在认识磷脂结合辅助因子(主要是β2糖蛋白I)作为抗体真正的免疫靶点的作用方面取得了重大进展。最近的证据表明,这些抗体破坏了磷脂依赖性抗凝机制,和/或aPL抗体诱导内皮细胞上促凝和促黏附分子的表达。目前的诊断基于临床发现和经验性检测,如检测与磷脂或假定辅助因子结合的抗体的检测方法,以及检测磷脂依赖性凝血反应抑制作用的凝血检测方法。目前的治疗主要依赖抗凝治疗。预计研究进展将带来基于发病机制的诊断检测方法和针对疾病根源的改进治疗方法。