This guidance updates and replaces previous BSH guide-lines.Antiphospholipidsyndrome(APS)isanautoimmune disease characterised by thrombosis (venous, arterial and/or microvascular) and/or pregnancy morbidity in association with persistently positive antiphospholipid antibodies (aPL). APS can occur in isolation (primary APS) or in association with other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) and rheumatoid arthritis (sec-ondary APS).The diagnosis of APS requires the presence of at least one clinical event (either an objectively confirmed throm-botic event and/or pregnancy complication) and detection of one or more aPL (lupus anticoagulant [LA], IgG/IgM an-ticardiolipin [aCL] and/or IgG/IgM anti-β₂ glycoprotein-1 [aβ₂GPI]) on two or more occasions at least 12weeks apart.Thrombosis in APS can occur in any organ or tissue. Deep vein thrombosis (DVT) with or without pulmonary embo lism (PE) is the most common venous thrombosis (VTE) while transient ischaemic attack (TIA) and stroke are the most common arterial thromboses. APS may also present with unusual site thrombosis such as portal, renal, mesen teric and cerebral venous sinus thrombosis. Microvascular thrombosis in APS is uncommon but may manifest as the potentially lethal catastrophic antiphospholipid syndrome (CAPS), which develops in <1% of patients with APS and where there is evidence of multiorgan failure commonly af fecting the heart, lungs, brain and/or kidneys. In the updated Sapporo classification criteria, non thrombotic clinical manifestations such as thrombocytope nia and heart valve disease were not included as diagnostic or defining features of definite APS. However, recent American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) Antiphospholipid Syndrome Classification Criteria, designed for uniformity of patient risk profiles in clinical studies, include several fea tures not described in the revised Sapporo criteria. The ACR/ EULAR criteria (Table 1) include a scoring system (score range 1–7 points each) divided into six clinical domains (macrovascu lar venous thromboembolism, macrovascular arterial throm bosis, microvascular thrombosis, obstetric, cardiac valve and haematological) and two laboratory domains similar to the revised Sapporo criteria. Patients are classified as having APS if they score ≥3 points in clinical domains and ≥3 points in lab oratory domains (Table 1). The definition of aPL persistence has not altered, but the maximum time between a clinical event and persistently positive aPL has been shortened from 5 to 3years (Table 1). Diagnosis of APS in routine clinical practice is less re strictive than the ACR/EULAR APS classification criteria; their strict application in routine practice to diagnose individual pa tients should be avoided. The diagnosis of APS should depend on careful clinical assessment, paying attention to alternative causes of thrombosis or pregnancy morbidity and critical eval uation of laboratory results considering the limitations of the laboratory assays. Current clinical practice is based on the evi dence derived from the clinical studies from patients with APS diagnosed as per revised Sapporo criteria.

本指南更新并取代了之前的 BSH 指南。抗磷脂综合征 (APS) 是一种自身免疫性疾病，特征为血栓形成（静脉、动脉和/或微血管）和/或与抗磷脂抗体 (aPL) 持续阳性相关的妊娠。APS 可单独发生（原发性）或与其他自身免疫性疾病有关，最常见的是系统性红斑狼疮 (SLE) 和类风湿性关节炎（继发性APS）。APS 的诊断需要存在至少一种临床事件（客观证实的血栓形成事件和/或妊娠并发症）和检测一种或多种aPL（狼疮抗凝物 [LA]、IgG/IgM抗心磷脂 [aCL] 和/或 IgG/IgM 抗β₂糖蛋白-1）[aβ₂GPI]），至少间隔12周。APS 的血栓形成可发生于任何器官或组织。深静脉血栓形成 (DVT) 伴或不伴肺栓塞肺栓塞 (PE) 是最常见的静脉血栓形成 (VTE)而短暂性脑缺血发作 (TIA) 和卒中是最常见的动脉血栓形成。APS 也可能存在伴有异常部位血栓形成，如门脉、肾、肠系膜动脉和脑静脉窦血栓形成。微血管APS 中的血栓形成并不常见，但可表现为潜在致死性灾难性抗磷脂综合征(CAPS)，在 < 1%的 APS 患者中发生，通常有多器官衰竭的证据影响心脏、肺、脑和/或肾脏。在更新的札幌分类标准中，非血栓性临床表现，如血小板增多nia 和心脏瓣膜疾病不作为诊断或明确 APS 的定义特征。然而，最近美国美国风湿病学会 (ACR)/欧洲风湿病学会风湿病学会 (EULAR) 抗磷脂抗体证候分类标准，统一设计临床研究中的患者风险特征，包括几种 fea修订版 Sapporo 标准中未描述的内容。ACR/EULAR 标准（表1）包括评分系统（评分范围1-7分）分为6个临床领域（macrovascu大血管动脉血栓栓塞血栓形成、微血管血栓形成、产科、心脏瓣膜和血液学）和两个与修订了札幌标准。将患者归类为 APS如果临床领域评分≥3分，实验室评分≥3分口域（表1）。aPL 持久性的定义具有未改变，但临床事件与持续阳性 aPL 已从5年缩短至3年（表1）。在常规临床实践中，APS的诊断较少严格于 ACR/EULAR APS 分类标准；其在常规实践中严格应用于个体 pa 的诊断应避免患者。APS 的诊断应取决于仔细进行临床评估，注意替代方案血栓形成或妊娠发病的原因和关键评价考虑到以下限制的实验室结果的评价实验室分析。目前的临床实践是根据 eviAPS 患者临床研究中的发生率根据修订版 Sapporo 标准诊断。