Alijotas-Reig Jaume, Miro-Mur Francesc, Hoxha Ariela, Khamashta Munther A, Shoenfeld Yehuda
Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain
Department of Medicine, Vall d'Hebron University Hospital, Barcelona, Spain.
RMD Open. 2025 Aug 25;11(3):e006014. doi: 10.1136/rmdopen-2025-006014.
Antiphospholipid syndrome (APS) is an autoimmune disorder for which there are no universally accepted diagnostic criteria, although classification criteria do exist, as is the case with most autoimmune diseases. Until 2023, the 2006 Sydney classification criteria were in use. Although originally intended for research purposes, these criteria have often been employed in clinical practice as a substitute for diagnostic guidelines, thereby conflating classification with diagnosis. In July 2023, ACR and European Alliance of Associations for Rheumatology convened a panel of experts to revise these criteria. The newly published classification criteria are explicitly intended for research use only. They place a strong emphasis on specificity-99%-but this comes at the expense of sensitivity-84%. The updated criteria encompass six clinical domains and two laboratory domains. Notably, the inclusion of new clinical features, such as thrombocytopenia, cardiac valve involvement and microvascular thrombosis, has broadened patient inclusion and, indirectly, aided the diagnostic process. However, a significant proportion of patients with suspected antiphospholipid antibody-related conditions may no longer meet the criteria for APS classification. In real-world settings, this could result in these individuals being denied appropriate management, thereby increasing their risk of subsequent thrombotic or obstetric events, as has already been demonstrated.This manuscript examines the advantages and limitations of the new clinical and laboratory domains, considering their implications not only from a research but also from a clinical perspective, APS.
抗磷脂综合征(APS)是一种自身免疫性疾病,尽管存在分类标准(大多数自身免疫性疾病都是如此),但尚无普遍接受的诊断标准。直到2023年,一直使用的是2006年悉尼分类标准。尽管这些标准最初是用于研究目的,但在临床实践中经常被用作诊断指南的替代品,从而将分类与诊断混为一谈。2023年7月,美国风湿病学会(ACR)和欧洲风湿病协会联盟召集了一个专家小组来修订这些标准。新发布的分类标准明确仅用于研究。它们非常强调特异性(99%),但这是以敏感性(84%)为代价的。更新后的标准涵盖六个临床领域和两个实验室领域。值得注意的是,纳入血小板减少症、心脏瓣膜受累和微血管血栓形成等新的临床特征扩大了患者范围,并且间接地有助于诊断过程。然而,相当一部分疑似抗磷脂抗体相关疾病的患者可能不再符合APS分类标准。在实际临床环境中,这可能导致这些患者得不到适当的治疗,从而增加他们随后发生血栓形成或产科事件的风险,正如已经证明的那样。本手稿探讨了新的临床和实验室领域的优点和局限性,不仅从研究角度,而且从临床角度考虑它们对APS的影响。