The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria
- PMID: 37635643
- DOI: 10.1002/art.42624
The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria
Abstract
Objective: 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.
Methods: This international multidisciplinary initiative included 4 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.
Results: 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 6 clinical domains (macrovascular venous thromboembolism, macrovascular arterial thrombosis, microvascular, obstetric, cardiac valve, and hematologic) and 2 laboratory domains (lupus anticoagulant functional coagulation assays, and solid-phase enzyme-linked immunosorbent assays for IgG/IgM anticardiolipin and/or IgG/IgM anti-β2 -glycoprotein I antibodies). Patients accumulating at least 3 points each from the clinical and laboratory domains are classified as having APS. In the validation cohort, the new APS criteria versus the 2006 revised Sapporo classification criteria had a specificity of 99% versus 86%, and a sensitivity of 84% versus 99%.
Conclusion: 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.
© 2023 American College of Rheumatology.
Similar articles
-
2023 ACR/EULAR antiphospholipid syndrome classification criteria.Ann Rheum Dis. 2023 Oct;82(10):1258-1270. doi: 10.1136/ard-2023-224609. Epub 2023 Aug 28. Ann Rheum Dis. 2023. PMID: 37640450
-
Assessment of the 2023 ACR/EULAR antiphospholipid syndrome classification criteria in a Chinese cohort: Impact on clinical practice.J Autoimmun. 2024 Jun;146:103237. doi: 10.1016/j.jaut.2024.103237. Epub 2024 May 14. J Autoimmun. 2024. PMID: 38749076
-
Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.Int J Rheum Dis. 2024 May;27(5):e15175. doi: 10.1111/1756-185X.15175. Int J Rheum Dis. 2024. PMID: 38720575
-
2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus.Ann Rheum Dis. 2019 Sep;78(9):1151-1159. doi: 10.1136/annrheumdis-2018-214819. Epub 2019 Aug 5. Ann Rheum Dis. 2019. PMID: 31383717 Review.
-
Development of a New International Antiphospholipid Syndrome Classification Criteria Phase I/II Report: Generation and Reduction of Candidate Criteria.Arthritis Care Res (Hoboken). 2021 Oct;73(10):1490-1501. doi: 10.1002/acr.24520. Epub 2021 Sep 2. Arthritis Care Res (Hoboken). 2021. PMID: 33253499 Free PMC article. Review.
Cited by
-
Neurovascular Issues in Antiphospholipid Syndrome: Arterial Vasculopathy from Small to Large Vessels in a Neuroradiological Perspective.J Clin Med. 2024 Jun 24;13(13):3667. doi: 10.3390/jcm13133667. J Clin Med. 2024. PMID: 38999233 Free PMC article. Review.
-
Persistently High Platelet Factor 4 Levels in an Adolescent with Recurrent Late Thrombotic Complications after SARS-CoV-2 mRNA Vaccination.Hematol Rep. 2024 Jul 29;16(3):504-511. doi: 10.3390/hematolrep16030048. Hematol Rep. 2024. PMID: 39189244 Free PMC article.
-
Clinical Features and Survival Analysis of Lupus Nephritis among Patients with Systemic Lupus Erythematosus: A Three-Decade-Long Retrospective Cohort Study.Biomedicines. 2024 Sep 18;12(9):2117. doi: 10.3390/biomedicines12092117. Biomedicines. 2024. PMID: 39335630 Free PMC article.
-
Incidence and prevalence of antiphospholipid syndrome (APS) in the USA (2016-2019): a retrospective database study.BMJ Open. 2024 Dec 11;14(12):e084563. doi: 10.1136/bmjopen-2024-084563. BMJ Open. 2024. PMID: 39663168 Free PMC article.
-
Rare connective tissue diseases in patients with C1-inhibitor deficiency hereditary angioedema: first evidence on prevalence and distribution from a large Italian cohort study.Front Immunol. 2024 Oct 18;15:1461407. doi: 10.3389/fimmu.2024.1461407. eCollection 2024. Front Immunol. 2024. PMID: 39493762 Free PMC article.
References
REFERENCES
-
- Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheumatol 1999;42:1309-11.
-
- Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome. J Thromb Haemost 2006;4:295-306.
-
- Garcia D, Erkan D. Diagnosis and management of the antiphospholipid syndrome. N Engl J Med 2018;378:2010-21.
-
- Sciascia S, Radin M, Cecchi I, et al. 16th International congress on antiphospholipid antibodies task force report on clinical manifestations of antiphospholipid syndrome. Lupus 2021;30:1314-26.
-
- Erkan D, Derksen R, Levy R, et al. Antiphospholipid Syndrome Clinical Research Task Force report. Lupus 2011;20:219-24.
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Miscellaneous