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抗磷脂综合征(APS)是一种以动静脉血栓反复形成、病态妊娠及抗磷脂抗体持续阳性为特征的疾病。APS的发病与多种自身抗体相关,如抗心磷脂抗体、抗β-2糖蛋白-1抗体、抗凝血酶原抗体等。临床上根据有无合并其他自身免疫性疾病,分为原发性APS和继发性APS。APS可累及全身各个器官,无论是原发性还是继发性APS,肾脏均是其损害的主要靶器官,肾内各级血管,包括肾动脉主干及其分支,肾小球毛细血管及肾静脉均可出现血栓。APS肾损害的临床表现复杂多样,但缺乏特异性,因此临床诊断比较困难,误诊、漏诊率高,也给治疗带来很大麻烦。APS可导致终末期肾病及移植肾功能不全,一旦诊断明确需要长期抗凝治疗以预防血栓事件,一些新的药物如利妥昔单抗、雷帕霉素等被发现可能对APS有效。
Antiphospholipid syndrome (APS) is a disease characterized by the repeated formation of arteriovenous thrombosis, morbid pregnancy, and persistent antiphospholipid antibodies. The incidence of APS and a variety of autoantibodies, such as anticardiolipin antibodies, anti-β-2 glycoprotein-1 antibodies, antithrombin antibodies. Clinically based on the availability of other autoimmune diseases, divided into primary APS and secondary APS. APS can affect all organs of the body, whether primary or secondary APS, the kidneys are the main target organ damage, all levels of blood vessels in the kidney, including the renal artery and its branches, glomerular capillary and renal vein Thrombosis can occur. The clinical manifestations of APS nephropathy are complex and diverse, but they lack specificity. Therefore, clinical diagnosis is more difficult, misdiagnosis and misdiagnosis rate also bring great cumbersome treatment. APS can lead to end-stage renal disease and graft insufficiency. Once diagnosed with long-term anticoagulant therapy to prevent thrombotic events, some new drugs such as rituximab and rapamycin are found to be potentially effective against APS.