Barbara Volcker Center for Women and Rheumatic Diseases, Hospital for Special Surgery, Weill Cornell Medicine, New York, New York, USA.
Vascular Medicine Division, French National Referral Center for Systemic and Autoimmune Diseases, Université de Lorraine, Inserm, DCAC, and CHRU-Nancy, Nancy, France.
Ann Rheum Dis. 2023 Oct;82(10):1258-1270. doi: 10.1136/ard-2023-224609. Epub 2023 Aug 28.
To develop new antiphospholipid syndrome (APS) classification criteria with high specificity for use in observational studies and trials, jointly supported by the American College of Rheumatology (ACR) and EULAR.
This international multidisciplinary initiative included four phases: (1) Phase I, criteria generation by surveys and literature review; (2) Phase II, criteria reduction by modified Delphi and nominal group technique exercises; (3) Phase III, criteria definition, further reduction with the guidance of real-world patient scenarios, and weighting via consensus-based multicriteria decision analysis, and threshold identification; and (4) Phase IV, validation using independent adjudicators' consensus as the gold standard.
The 2023 ACR/EULAR APS classification criteria include an entry criterion of at least one positive antiphospholipid antibody (aPL) test within 3 years of identification of an aPL-associated clinical criterion, followed by additive weighted criteria (score range 1-7 points each) clustered into six clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and two laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β-glycoprotein I antibodies). Patients accumulating at least three points each from the clinical and laboratory domains are classified as having APS. In the validation cohort, the new APS criteria vs the 2006 revised Sapporo classification criteria had a specificity of 99% vs 86%, and a sensitivity of 84% vs 99%.
These new ACR/EULAR APS classification criteria were developed using rigorous methodology with multidisciplinary international input. Hierarchically clustered, weighted, and risk-stratified criteria reflect the current thinking about APS, providing high specificity and a strong foundation for future APS research.
与美国风湿病学会 (ACR) 和欧洲抗风湿病联盟 (EULAR) 共同制定具有高特异性的新抗磷脂综合征 (APS) 分类标准，以便于在观察性研究和试验中使用。
这项国际性多学科倡议包括四个阶段：(1) 第 I 阶段，通过调查和文献回顾生成标准；(2) 第 II 阶段，通过修改后的德尔菲法和名义群体技术练习进行标准缩减；(3) 第 III 阶段，通过基于共识的多准则决策分析和阈值识别对标准进行定义、进一步缩减，并结合现实患者情况进行指导；(4) 第 IV 阶段，使用独立裁决者的共识作为金标准进行验证。
2023 年 ACR/EULAR APS 分类标准包括一个纳入标准，即在确定与 aPL 相关的临床标准后 3 年内，至少有一项阳性抗磷脂抗体 (aPL) 检测结果，随后是附加加权标准（每项标准的评分范围为 1-7 分），分为六个临床领域（大血管静脉血栓形成、大血管动脉血栓形成、微血管、产科、心脏瓣膜和血液）和两个实验室领域（狼疮抗凝物功能凝血试验和固相酶联免疫吸附试验检测 IgG/IgM 抗心磷脂抗体和/或 IgG/IgM 抗β-糖蛋白 I 抗体）。患者在临床和实验室领域各累积至少三分，即可被诊断为 APS。在验证队列中，新 APS 标准与 2006 年修订的 Sapporo 分类标准相比，特异性分别为 99%和 86%，敏感性分别为 84%和 99%。
这些新的 ACR/EULAR APS 分类标准是使用多学科国际投入的严格方法制定的。分层聚类、加权和风险分层标准反映了当前对抗磷脂综合征的认识，为未来的 APS 研究提供了高特异性和坚实的基础。
