抗磷脂综合征诊疗现状  

Current advances in diagnosis and treatment of antiphospholipid syndrome

作　　者：郑辉[1,2] 杨程德 ZHENG Hui;YANG Chengde(Department of Rheumatology and Immunology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China;不详)

机构地区：[1]上海交通大学医学院附属瑞金医院风湿免疫科,上海200025 [2]山东第一医科大学第二附属医院风湿免疫科,山东泰安271000

出　　处：《中华全科医学》2024年第10期1750-1754,共5页Chinese Journal of General Practice

基　　金：国家自然科学基金项目(82371799)。

摘　　要：抗磷脂综合征(APS)是一种以血清中持续存在高滴度的抗磷脂抗体(aPL)伴反复动/静脉血栓或微血管血栓形成、病理妊娠等临床表现为特征的自身免疫性、炎症性疾病。目前研究认为,APS的发病是由aPL(抗体途径)与天然免疫成分(非抗体途径)共同参与并相互作用所致,多种免疫细胞、凝血系统、补体系统和细胞因子等均参与其中。除了特征性的血栓形成和病理妊娠,APS患者还常伴有未被以往诊断标准纳入的所谓“标准外”临床表现如血小板减少、心脏瓣膜病变、认知功能障碍、网状青斑或其他自身免疫或炎症并发症。随着对APS发病机制和临床特征的深入认识和越来越多的临床实践,旧的分类诊断标准已经不能满足当前的临床和研究需要,因此美国风湿病学会/欧洲抗风湿联盟于2023年共同发布了最新版本的APS分类标准。根据现有的指南及诊疗规范,抗凝和抗血小板治疗是该病治疗的基石,但临床上常面临许多未被满足的治疗需求,近年来APS发病机制的研究进展揭示出了许多新的治疗靶点,为部分难治性患者提供了更多的治疗选择。本文综述了APS的诊疗研究现状,以提高临床医生及相关领域研究者对本病的认识水平,帮助临床医生更好地管理APS患者。Antiphospholipid syndrome(APS)is an autoimmune and inflammatory disease that is characterized by the persistent presence of high titers of antiphospholipid antibodies(aPL)in serum,recurrent arteriovenous or microvascular thrombosis,and pregnancy morbidity.It is currently believed that the aetiology of APS is based on the interaction be-tween aPL(antibody-driven)and the innate immune system(non-antibody-driven).A variety of immune cells,the co-agulation system,the complement system and cytokines are all involved in the pathogenetic process of APS.In addition to the characteristic manifestations of thrombosis and pregnancy morbidity,patients with APS frequently present with so-called"extra-criteria"clinical manifestations that have not been included in previous classification criteria.These may include thrombocytopenia,cardiac valve involvement,cognitive dysfunction,livedo reticularis,or other autoimmune/in-flammatory complications.As our understanding of the pathogenesis and clinical characteristics of APS has deepened,and as our clinical practice has broadened,it has become increasingly clear that the previous classification criteria are un-able to meet the current clinical and research needs.Accordingly,the American College of Rheumatology(ACR)and the European League Against Rheumatism(EULAR)have jointly published the latest iteration of the APS classification criteria,dated 20 August 2023.In accordance with the prevailing management recommendations,anticoagulation and an-tiplatelet therapy represent the fundamental cornerstones of APS treatment.However,there invariably remain numerous unmet clinical needs.The research progress in the pathogenesis of APS has revealed numerous new potential therapeutic targets in recent years,which may provide more treatment options for some refractory patients.The objective of this article is to provide a comprehensive review of the current status of research into the diagnosis and treatment of APS.The objec-tive is to enhance the understanding of this disease among clinicians a

关 键 词：抗磷脂综合征 抗磷脂抗体 诊断 治疗 

分 类 号：R593.2[医药卫生—内科学]