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抗磷脂综合征的新型管理方法。

New approaches for managing antiphospholipid syndrome.

作者信息

Erkan Doruk, Lockshin Michael D

机构信息

The Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, NY, USA.

出版信息

Nat Clin Pract Rheumatol. 2009 Mar;5(3):160-70. doi: 10.1038/ncprheum1017.

Abstract

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.

摘要

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。

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