Joseph R E, Radhakrishnan J, Appel G B
Department of Medicine, Division of Nephrology, Columbia Presbyterian Medical Center, New York, New York, USA.
Curr Opin Nephrol Hypertens. 2001 Mar;10(2):175-81. doi: 10.1097/00041552-200103000-00004.
The antiphospholipid syndrome is a disorder of hypercoagulability in association with circulating antiphospholipid antibodies directed against epitopes on oxidized phospholipids complexed with a glycoprotein, beta 2-glycoprotein I, or against the glycoprotein itself. Disorders associated with antiphospholipid antibodies but not the antiphospholipid syndrome, such as HIV and hepatitis C infection, appear to lack antibodies to beta 2-glycoprotein I. Patients with systemic lupus erythematosus have a high incidence of antiphospholipid antibodies with a high risk of thrombosis, often associated with anticardiolipin antibodies, beta 2-glyocoprotein I antibodies, and the presence of the lupus anticoagulant. Antiphospholipid antibodies are a significant cause of morbidity and mortality in renal patients with and without systemic lupus erythematosus. Renal manifestations include thrombotic microangiopathy and large vessel thrombosis. In patients with end-stage renal disease, antiphospholipid antibodies are prevalent and may increase in frequency with time on dialysis, possibly as a result of oxidative stress incurred during dialysis. The presence of anticardiolipin antibodies have been associated with a high incidence of hemodialysis access clotting. In renal transplant recipients, the incidence of antiphospholipid antibodies is also elevated and may be associated with a higher incidence of primary graft non-function. Although patients with systemic lupus erythematosus have similar renal allograft survival rates to the general population, survival is worse for those patients who are also antiphospholipid antibody positive. Additionally, in hepatitis C positive renal transplant recipients, the presence of anticardiolipin antibodies confers an increased risk of thrombotic complications and the development of thrombotic microangiopathy. Treatment of antiphospholipid antibody syndrome remains centered around anticoagulation with warfarin. The use of immunosuppressive agents has had no dramatic effect on antiphospholipid antibody titers and little clinical effect on thrombotic events.
抗磷脂综合征是一种高凝状态的疾病，与针对氧化磷脂与糖蛋白β2-糖蛋白I形成的表位的循环抗磷脂抗体有关，或与针对该糖蛋白本身的抗体有关。与抗磷脂抗体相关但不属于抗磷脂综合征的疾病，如HIV和丙型肝炎感染，似乎缺乏针对β2-糖蛋白I的抗体。系统性红斑狼疮患者抗磷脂抗体的发生率很高，血栓形成风险也很高，常与抗心磷脂抗体、β2-糖蛋白I抗体以及狼疮抗凝物的存在有关。抗磷脂抗体是有或没有系统性红斑狼疮的肾病患者发病和死亡的重要原因。肾脏表现包括血栓性微血管病和大血管血栓形成。在终末期肾病患者中，抗磷脂抗体很常见，并且可能随着透析时间的延长而增加，这可能是透析过程中产生氧化应激的结果。抗心磷脂抗体的存在与血液透析通路凝血的高发生率有关。在肾移植受者中，抗磷脂抗体的发生率也升高，并且可能与原发性移植无功能的较高发生率有关。虽然系统性红斑狼疮患者的肾移植存活率与一般人群相似，但抗磷脂抗体阳性的患者存活率更差。此外，在丙型肝炎阳性的肾移植受者中，抗心磷脂抗体的存在会增加血栓并发症和血栓性微血管病发生的风险。抗磷脂抗体综合征的治疗仍然以华法林抗凝为中心。免疫抑制剂的使用对抗磷脂抗体滴度没有显著影响，对血栓事件的临床效果也很小。
