Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.
Department of Clinical and Experimental Sciences, University of Brescia, Rheumatology and Clinical Immunology, Spedali Civili, Brescia, Italy.
J Autoimmun. 2017 Jan;76:10-20. doi: 10.1016/j.jaut.2016.10.004. Epub 2016 Oct 21.
The antiphospholipid syndrome (APS) is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis and pregnancy morbidity in the presence of pathogenic autoantibodies known as antiphospholipid antibodies (aPL). APS may be associated with other diseases, mainly systemic lupus erythematosus (SLE). The presence or absence of SLE might modify the clinical or serological expression of APS. Apart from the classical manifestations, APS patients with associated SLE more frequently display a clinical profile with arthralgias, arthritis, autoimmune hemolytic anemia, livedo reticularis, epilepsy, glomerular thrombosis, and myocardial infarction. The management of patients with SLE and APS/aPL should include an accurate stratification of vascular risk factors. Low dose aspirin and hydroxychloroquine should be considered as primary prophylaxis. In high risk situations, such as surgery, prolonged immobilization, and puerperium, the prophylaxis should be potentiated with low molecular weight heparin. The challenge of treating patients with a previous vascular event (secondary prophylaxis) is the choice of treatment (anti-platelet agents, anticoagulation with vitamin K antagonists or combined therapy) and its duration, based on individual risk stratification and the site of vascular presentation. The role of novel anticoagulants in APS patients is still to be clearly defined. Novel approaches are needed since the prognosis of SLE patients with APS/aPL is still worse than that of SLE patients with negative aPL. The goal for the future is to improve the outcome of these patients by means of early recognition and optimal preventative treatment.
抗磷脂综合征（APS）是一种自身免疫性疾病，其特征是存在致病性自身抗体（称为抗磷脂抗体[aPL]）时发生静脉和/或动脉血栓形成和妊娠并发症。APS 可能与其他疾病相关，主要是系统性红斑狼疮（SLE）。SLE 的存在或缺失可能会改变 APS 的临床或血清学表现。除了经典表现外，伴有 SLE 的 APS 患者更频繁地表现出关节痛、关节炎、自身免疫性溶血性贫血、网状青斑、癫痫、肾小球血栓形成和心肌梗死的临床特征。SLE 和 APS/aPL 患者的管理应包括对血管危险因素进行准确分层。应考虑小剂量阿司匹林和羟氯喹作为初级预防。在手术、长时间固定和产褥期等高危情况下，应使用低分子量肝素增强预防。治疗有先前血管事件（二级预防）的患者的挑战是根据个体风险分层和血管表现部位选择治疗（抗血小板药物、维生素 K 拮抗剂抗凝或联合治疗）及其持续时间。新型抗凝剂在 APS 患者中的作用仍有待明确。需要新的方法，因为伴有 APS/aPL 的 SLE 患者的预后仍比 aPL 阴性的 SLE 患者差。未来的目标是通过早期识别和最佳预防治疗来改善这些患者的预后。
