抗磷脂综合征诊治及发病机制

Diagnosis,treatment, and pathogenesis of antiphospholipid syndrome

抗磷脂综合征(APS)是以血栓形成和(或)病理性妊娠为主要临床特征、实验室检查为持续性抗磷脂抗体(a PL)阳性的疾病谱。近年来关于APS的研究取得了较多新的进展,主要涉及APS的发病机制、APS的诊断标准、APS "诊断标准外"临床表现、a PL类型和治疗推荐等,这些研究进展为APS研究提供了最新的知识和观点,同时为APS治疗提供了新的靶点,并可改善APS的治疗结局。本文就APS "诊断标准外"临床表现及a PL类型、治疗进展包括直接口服抗凝药(DOACs)的应用以及发病机制进展作一综述。

Antiphospholipid syndrome （APS） is a clinical spectrum which characterized by thrombosis and/or pregnancy morbidity,and persistently positive antiphospholipid antibodies （aPL）. Recently, more progresses have been made in the studies on pathogenesis, diagnosis criteria, ‘extra-criteria’ clinical manifestations, aPL typing and treatment recommendation of APS. These researches brings up to the update knowledge and ideas. They also provide new targets for the management of APS, which will improve the outcome of APS patients. This review introduces ‘extra-criteria＇ clinical manifestations of APS, aPL typing, latest published treatment recommendations including direct oral anticoagulants （DOACs）, and new mechanisms in APS pathogenesis.