文献简介

出版社:Blood Reviews

作  者:Shruti Chaturvedi, Keith R. McCrae

编  号:

关键字:antiphospholipid syndrome

年  份:2018   点击量:51

文献摘要 全文翻译

Antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy complications in the presence of persistent antiphospholipid antibodies (APLA). Laboratory diagnosis of APLA depends upon the detection of a lupus anticoagulant, which prolongs phospholipid-dependent anticoagulation tests, and/or anticardiolipin (aCL) and anti-β2-glycoprotein-I (β2GPI) antibodies. APLA are primarily directed toward phospholipid binding proteins. Pathophysiologic mechanisms underlying thrombosis and pregnancy loss in APS include APLA induced cellular activation, inhibition of natural anticoagulant and fibrinolytic systems, and complement activation, among others. There is a high rate of recurrent thrombosis in APS, especially in triple positive patients (patients with lupus anticoagulant, aCL and anti-β2GPI antibodies), and indefinite anticoagulation with a vitamin K antagonist is the standard of care for thrombotic APS. There is currently insufficient evidence to recommend the routine use of direct oral anticoagulants (DOAC) in thrombotic APS. Aspirin with low molecular weight or unfractionated heparin may reduce the incidence of pregnancy loss in obstetric APS. Recent insights into the pathogenesis of APS have led to the identification of new potential therapeutic interventions, including anti-inflammatory and immunomodulatory therapies. Additional research is needed to better understand the effects of APLA on activation of signaling pathways in vascular cells, to identify more predictive biomarkers that define patients at greatest risk for a first or recurrent APLA-related clinical event, and to determine the safety and efficacy of DOACs and novel anti-inflammatory and immune-modulatory therapies for refractory APS.

抗磷脂综合征(APS)以血栓形成和/或妊娠并发症,以及持续性抗磷脂抗体(APLA)的存在为特征。 APLA的实验室诊断基于检测到狼疮抗凝血物(其延长了磷脂依赖性抗凝试验),和/或抗心磷脂(aCL)和抗 β2糖蛋白I(β2GPI)抗体。APLA主要针对磷脂结合蛋白。APS中血栓形成和妊娠丢失的病理生理机制包括APLA诱导的细胞活化,天然抗凝物和纤维蛋白溶解系统受抑制以及补体激活等。APS患者中血栓复发率高,尤其是在三重阳性患者中(狼疮抗凝体、aCL和抗β2GPI抗体阳性),且无限期抗凝伴维生素K拮抗剂是治疗血栓性APS的标准疗法。目前无足够证据推荐血栓性APS患者日常直接口服抗凝剂(DOAC)。阿司匹林伴低分子量或普通肝素可降低APS患者流产率。近期对APS发病机制的深入了解确定了新的潜在的治疗干预措施,包括抗炎和免疫调节治疗。需要进一步研究以更好地理解APLA对血管细胞信号通路激活的影响,确定更具预测性的生物标志物从而确定首次或反复发生APLA相关临床事件风险最高的患者,并确定DOACs和新的抗炎和免疫调节疗法治疗难治性APS的安全性和有效性。