Thrombosis & Thrombophilia, Guy's and St Thomas' Hospital NHS Foundation Trust, Westminster Bridge Road, London SE1 7EH, UK.
Copenhagen Lupus and Vasculitis Clinic, Center for Rheumatology and Spine Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark.
Nat Rev Dis Primers. 2018 Jan 11;4:17103. doi: 10.1038/nrdp.2017.103.
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin antibodies and anti-β2-glycoprotein 1 antibodies. APS can present with a variety of clinical phenotypes, including thrombosis in the veins, arteries and microvasculature as well as obstetrical complications. The pathophysiological hallmark is thrombosis, but other factors such as complement activation might be important. Prevention of thrombotic manifestations associated with APS includes lifestyle changes and, in individuals at high risk, low-dose aspirin. Prevention and treatment of thrombotic events are dependent mainly on the use of vitamin K antagonists. Immunosuppression and anticomplement therapy have been used anecdotally but have not been adequately tested. Pregnancy morbidity includes unexplained recurrent early miscarriage, fetal death and late obstetrical manifestation such as pre-eclampsia, premature birth or fetal growth restriction associated with placental insufficiency. Current treatment to prevent obstetrical morbidity is based on low-dose aspirin and/or low-molecular-weight heparin and has improved pregnancy outcomes to achieve successful live birth in >70% of pregnancies. Although hydroxychloroquine and pravastatin might further improve pregnancy outcomes, prospective clinical trials are required to confirm these findings.
抗磷脂综合征（APS）是一种自身免疫性疾病，其特征是存在抗磷脂抗体，如狼疮抗凝物、抗心磷脂抗体和抗β2-糖蛋白 1 抗体。APS 可表现出多种临床表型，包括静脉、动脉和微血管血栓形成以及产科并发症。其病理生理学特征是血栓形成，但补体激活等其他因素也可能很重要。预防与 APS 相关的血栓表现包括生活方式改变，以及对高风险个体使用低剂量阿司匹林。预防和治疗血栓事件主要依赖于维生素 K 拮抗剂的使用。免疫抑制和抗补体治疗已被零星使用，但尚未得到充分测试。妊娠发病率包括不明原因的反复早期流产、胎儿死亡和晚期产科表现，如与胎盘功能不全相关的子痫前期、早产或胎儿生长受限。目前预防产科发病率的治疗方法基于低剂量阿司匹林和/或低分子量肝素，已改善了妊娠结局，使超过 70%的妊娠实现成功活产。尽管羟氯喹和普伐他汀可能进一步改善妊娠结局，但仍需要前瞻性临床试验来证实这些发现。
