Diagnosis and management of the antiphospholipid syndrome.
Diagnosis and management of the antiphospholipid syndrome.
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DOI：
10.1016/j.blre.2017.07.006
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发表时间：
2017-11
期刊：
影响因子：
7.4
通讯作者：
McCrae KR
中科院分区：
文献类型：
--
作者：
Chaturvedi S;McCrae KR
抗磷脂综合征（APS）的特点是在持续存在抗磷脂抗体（APLA）的情况下形成血栓和/或妊娠并发症。 APLA 的实验室诊断取决于狼疮抗凝剂的检测（可延长磷脂依赖性抗凝试验）和/或抗心磷脂 (aCL) 和抗 β2-糖蛋白-1 (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 诱导的细胞激活、天然抗凝和纤溶系统的抑制以及补体激活等。 APS 的血栓复发率很高，尤其是三阳性患者（狼疮抗凝剂、aCL 和抗 β2GPI 抗体的患者），用维生素 K 拮抗剂无限期抗凝是血栓性 APS 的标准治疗方法。目前没有足够的证据推荐在血栓性 APS 中常规使用直接口服抗凝剂 (DOAC)。低分子阿司匹林或普通肝素可能会降低产科 APS 流产的发生率。最近对 APS 发病机制的深入了解导致了新的潜在治疗干预措施的确定，包括抗炎和免疫调节疗法。需要进行更多研究来更好地了解 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来定义首次或复发 APLA 相关临床事件风险最大的患者，并确定 DOAC 和新型抗炎和免疫调节疗法治疗难治性 APS 的安全性和有效性。
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-1 (β2GPI) antibodies. APLA are primarily directed towards 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 anticoagulants, 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.
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DOI：
10.1182/asheducation-2015.1.53
发表时间：
2015-12-01
影响因子：
3
作者：
Chaturvedi, Shruti;McCrae, Keith R.
通讯作者：
McCrae, Keith R.
DOI：
10.1111/j.1471-0528.1999.tb08208.x
发表时间：
1999-02-01
期刊：
BRITISH JOURNAL OF OBSTETRICS AND GYNAECOLOGY
影响因子：
--
作者：
Backos, M;Rai, R;Regan, L
通讯作者：
Regan, L
影响因子：
2.6
作者：
Betancur, J. F.;Bonilla-Abadia, F.;Tobon, G. J.
通讯作者：
Tobon, G. J.
影响因子：
9.6
作者：
Bates, Shannon M.;Greer, Ian A.;Vandvik, Per Olav
通讯作者：
Vandvik, Per Olav
影响因子：
2.6
作者：
Asherson, RA
通讯作者：
Asherson, RA