Arachchillage Deepa J, Laffan Mike
Centre for Haematology, Department of Immunology and Inflammation, Imperial College London, London, United Kingdom.
Department of Haematology, Imperial College Healthcare NHS Trust, London, United Kingdom.
Res Pract Thromb Haemost. 2025 Mar 7;9(2):102724. doi: 10.1016/j.rpth.2025.102724. eCollection 2025 Feb.
Antiphospholipid syndrome (APS) is a highly prothrombotic autoimmune disease characterized by the persistent presence of antiphospholipid autoantibodies (aPL) in association with thrombotic or nonthrombotic macro- and microvascular manifestations and/or pregnancy complications. This review is restricted to thrombotic APS. Since the publication of the American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for APS, several authors have emphasized the difference between "classification" and "diagnosis" as a potential pitfall for clinicians. In addition to challenges associated with the diagnosis of APS, there are many unresolved areas in understanding pathogenesis and in the management of both thrombotic and obstetric APS. Although APS is an antibody-mediated autoimmune disease, secondary thrombosis prevention is achieved by anticoagulation, mainly with vitamin K antagonists, such as warfarin, rather than immunomodulation. Evidence is convincing for the use of vitamin K antagonists in triple-positive APS with venous thromboembolism. However, the best anticoagulant approach in the management of venous thromboembolism patients with single or dual positive aPL is not clear. Management of patients with stroke or arterial thrombosis with aPL remains a major unresolved issue, although some guidelines recommend the use of warfarin rather than antiplatelet therapy as the first-line treatment of stroke in APS. Recurrent thrombosis, despite therapeutic anticoagulation, remains a frequent problem and may be explained by the contribution of thrombo-inflammation in patients with thrombotic APS. In this narrative review, we discuss some of the unresolved issues in the diagnosis and management of thrombotic APS.
抗磷脂综合征（APS）是一种具有高度血栓形成倾向的自身免疫性疾病，其特征是抗磷脂自身抗体（aPL）持续存在，并伴有血栓形成或非血栓形成的大、微血管表现和/或妊娠并发症。本综述仅限于血栓形成性APS。自从美国风湿病学会/欧洲风湿病联盟协会发布APS分类标准以来，一些作者强调了“分类”和“诊断”之间的差异，认为这是临床医生可能面临的一个陷阱。除了与APS诊断相关的挑战外，在理解发病机制以及血栓形成性和产科APS的管理方面，仍有许多未解决的问题。虽然APS是一种抗体介导的自身免疫性疾病，但二级血栓预防主要通过抗凝来实现，主要使用维生素K拮抗剂，如华法林，而不是免疫调节。有令人信服的证据支持在伴有静脉血栓栓塞的三阳性APS中使用维生素K拮抗剂。然而，对于单阳性或双阳性aPL的静脉血栓栓塞患者，最佳的抗凝方法尚不清楚。对于伴有aPL的中风或动脉血栓形成患者的管理仍然是一个主要的未解决问题，尽管一些指南建议使用华法林而非抗血小板治疗作为APS中风的一线治疗方法。尽管进行了治疗性抗凝，复发性血栓形成仍然是一个常见问题，这可能是由于血栓形成性APS患者的血栓炎症所致。在这篇叙述性综述中，我们讨论了血栓形成性APS诊断和管理中的一些未解决问题。
