Antiphospholipid syndrome
Antiphospholipid syndrome
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DOI：
10.1182/asheducation-2013.1.675
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发表时间：
2013-12-01
影响因子：
3
通讯作者：
Lim, Wendy
中科院分区：
文献类型：
--
作者：
Lim, Wendy
抗磷脂综合征（APS）定义为持续存在抗磷脂抗体（apl）的患者静脉或动脉血栓形成和/或妊娠发病率。灾难性APS是APS最严重的形式，它与微血管血栓形成的快速发展相关，导致APS患者多器官功能衰竭。APS和灾难性APS患者被认为有很高的血栓复发风险，尽管抗凝治疗仍可能发生。虽然抗血栓治疗仍然是主要的治疗方法，但出血表现可能使治疗复杂化，并导致发病率增加。aPL水平持续升高的患者，特别是那些狼疮抗凝血剂、抗心磷脂抗体和抗β (2)GPI抗体检测呈三阳性的患者，出现血栓形成和妊娠并发症的风险增加，而孤立的aPL阳性似乎与低风险相关。认识到APS患者具有不同的血栓形成风险特征可以帮助临床医生评估抗凝治疗的风险和益处。APS治疗中抗凝治疗的最佳类型、强度和持续时间仍然存在争议，特别是对于动脉血栓形成和复发性血栓形成。未来的研究需要描述APS的血栓形成风险，并评估当前和新的抗凝剂以及非抗凝治疗。
The antiphospholipid syndrome (APS) is defined by venous or arteria thrombosis and/or pregnancy morbidity in patients with persistent presence of antiphospholipid antibodies (aPLs). Catastrophic APS is the most severe form of APS, which is associated with rapid development of microvascular thrombosis resulting in multiorgan failure in patients with aPLs. Patients with APS and catastrophic APS are recognized to have a high risk of recurrent thrombosis that can occur despite anticoagulant therapy. Although antithrombotic therapy remains the mainstay of treatment, bleeding manifestations can complicate management and contribute to increased morbidity. Patients with persistently elevated aPL levels, particularly those who exhibit positive testing for lupus anticoagulant, anticardiolipin antibodies, and anti-beta(2)GPI antibodies triple positivity, appear to be at increased risk for thrombosis and pregnancy complications, whereas isolated positivity for aPLs appears to be associated with low risk. Recognizing that patients with APS have different thrombotic risk profiles may assist clinicians in assessing the risks and benefits of anticoagulation The optimal type intensity and duration of anticoagulation in the treatment of APS remain controversial particularly for arterial thrombosis and recurrent thrombosis. Future studies that delineate thrombotic risk in APS and evaluate current and novel anticoagulants as well as nonanticoagulant therapies are required.