Erkan Doruk, Lockshin Michael D
The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY, USA.
Nat Clin Pract Rheumatol. 2009 Mar;5(3):160-70. doi: 10.1038/ncprheum1017.
Antiphospholipid antibodies (aPL) are a family of autoantibodies directed against phospholipid-binding plasma proteins, most commonly beta(2)-glycoprotein-I. Primary thrombosis prevention in persistently aPL-positive individuals requires a risk-stratified approach; elimination of reversible thrombosis risk factors and aggressive prophylaxis during high-risk periods are crucial. The effectiveness of aspirin in persistently aPL-positive patients without vascular involvement is not supported by data from prospective, controlled studies. For the secondary prevention of thrombosis in persistently aPL-positive individuals, the current recommendation is life-long warfarin; however, determining the intensity and duration of warfarin treatment, as well as the role of alternative anticoagulants, requires further research. The effectiveness of high-intensity anticoagulation in patients with antiphospholipid syndrome (APS) and vascular involvement is not supported by data from prospective, controlled studies. Patients with catastrophic APS usually receive a combination of anticoagulants, corticosteroids, intravenous immunoglobulin and plasma exchange; however, despite this aggressive approach, the mortality rate remains high. Potential new approaches for the management of persistently aPL-positive patients include hydroxychloroquine, statins, rituximab, complement inhibition, and other targeted therapies that have been effective in experimental APS models.
抗磷脂抗体(aPL)是一类针对磷脂结合血浆蛋白的自身抗体,最常见的是β2糖蛋白I。对于持续aPL阳性个体的原发性血栓形成预防,需要采用风险分层方法;消除可逆性血栓形成危险因素以及在高危期进行积极预防至关重要。前瞻性对照研究的数据不支持阿司匹林对无血管受累的持续aPL阳性患者的有效性。对于持续aPL阳性个体血栓形成的二级预防,目前的建议是终身服用华法林;然而,确定华法林治疗的强度和持续时间以及替代抗凝剂的作用,还需要进一步研究。前瞻性对照研究的数据不支持高强度抗凝对患有抗磷脂综合征(APS)和血管受累患者的有效性。灾难性APS患者通常接受抗凝剂、皮质类固醇、静脉注射免疫球蛋白和血浆置换的联合治疗;然而,尽管采取了这种积极的治疗方法,死亡率仍然很高。对于持续aPL阳性患者的潜在新治疗方法包括羟氯喹、他汀类药物、利妥昔单抗、补体抑制以及其他在实验性APS模型中有效的靶向治疗。