2023 ACR/EULAR antiphospholipid syndrome classification criteria
Open accessAbstract
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 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. 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 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 2-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%. 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.
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The major novelty in the new criteria is the scoring system for APS classification: classify if at least 3 points from clinical domains and at least 3 points from laboratory domains.
“The major novelty in the new criteria is the scoring system for APS classification: we can classify a patient as having APS if there are at least 3 points from clinical domains and at least 3 points from laboratory domains [1].”
From Insights into the 2023 ACR/EULAR antiphospholipid syndrome classification criteria: findings from a cohort of 205 patients with primary APS (2025) · cites this paper as [1]
The Abstract states that patients with at least three points each from the clinical and laboratory domains are classified as having APS, and the Discussion describes separate point requirements as a refinement of earlier criteria. But the Abstract also specifies an entry criterion—a positive antiphospholipid antibody test within three years of an associated clinical criterion—so the two scores alone are not sufficient as the citing sentence implies. (LLM, conf=0.86)
Experimental — accuracy not yet measuredThe 2023 ACR/EULAR criteria define aCL and aβ2GPI thresholds as moderate (40-79 units) and high positivity (>80 units).
“Moreover, the aCL and aβ2GPI thresholds were defined as moderate (40–79 units) and high positivity (>80 units)”
From Insights into the 2023 ACR/EULAR antiphospholipid syndrome classification criteria: findings from a cohort of 205 patients with primary APS (2025) · cites this paper as [1]
The criteria figure (Discussion) states moderate positivity as 40–79 units and high positivity as >80 units for aCL and anti-β2GPI. However, the final definitions (Phase III-C) specify high positivity as ≥80 ELISA units, which includes exactly 80; the cited paper is inconsistent on that boundary. The thresholds also apply to standardized ELISA results, a qualification absent from the claim. (LLM, conf=0.85)
Experimental — accuracy not yet measuredThe 2023 ACR/EULAR criteria improved specificity in classifying patients with APS compared with Sydney criteria.
“In fact, the 2023 ACR/EULAR criteria improved specificity in classifying patients with APS compared with Sidney criteria [1].”
From Insights into the 2023 ACR/EULAR antiphospholipid syndrome classification criteria: findings from a cohort of 205 patients with primary APS (2025) · cites this paper as [1]
The 2023 ACR/EULAR criteria give isolated IgM positivity a low weight, insufficient for APS classification.
“a low weight was given to isolated IgM positivity, leading to an insufficient score for APS classification even when clinical criteria are met.”
From Insights into the 2023 ACR/EULAR antiphospholipid syndrome classification criteria: findings from a cohort of 205 patients with primary APS (2025) · cites this paper as [1]
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- Authors
- Barbhaiya, M., Zuily, S., Naden, R., Hendry, A., Manneville, F., Amigo, M.-C., Amoura, Z., Andrade, D., Andreoli, L., Artim-Esen, B., Atsumi, T., Avcin, T., Belmont, M. H., Bertolaccini, M. L., Branch, D. W., Carvalheiras, G., Casini, A., Cervera, R., Cohen, H., Costedoat-Chalumeau, N., Crowther, M., de Jesús, G., Delluc, A., Desai, S., Sancho, M. D., Devreese, K. M., Diz-Kucukkaya, R., Duarte-García, A., Frances, C., Garcia, D., Gris, J.-C., Jordan, N., Leaf, R. K., Kello, N., Knight, J. S., Laskin, C., Lee, A. I., Legault, K., Levine, S. R., Levy, R. A., Limper, M., Lockshin, M. D., Mayer-Pickel, K., Musial, J., Meroni, P. L., Orsolini, G., Ortel, T. L., Pengo, V., Petri, M., Pons-Estel, G., Gomez-Puerta, J. A., Raimboug, Q., Roubey, R., Sanna, G., Seshan, S. V., Sciascia, S., Tektonidou, M. G., Tincani, A., Wahl, D., Willis, R., Yelnik, C., Zuily, C., Guillemin, F., Costenbader, K., Erkan, D., Agmon-Levin, N., Aguilar, C., Alba, P., Alpan, O., Ambrozic, A., Andrade, L., Appenzeller, S., Berkun, Y., Cabral, A., Canaud, G., Chen, P., Chighizola, C., Cimaz, R., Cuadrado, M. J., Groot, P. G. D., Moerloose, P. D., Derksen, R., Dörner, T., Fortin, P., Giannakopoulos, B., Gonzalez, E. B., Inanc, M., Kenet, G., Khamashta, M., Kriegel, M., Krilis, S., Ladha, D., Massicotte, P., McCarty, G., Mikdashi, J., Myones, B., Sammaritano, L., Signorelli, F., Soybilgic, A., Woller, S., & Zuo, R.
- Affiliations
- Hospital for Special Surgery (US)
- Auckland City Hospital (NZ)
- Middlemore Hospital (NZ)
- Inserm (FR)
- Centro Médico ABC (MX)
- Sorbonne Université (FR)
- Universidade de São Paulo (BR)
- Hokkaido University (JP)
- NYU Langone Health (US)
- University of Utah (US)
- Hospital de Santo António (PT)
- University Hospital of Geneva (CH)
- Hospital Clínic de Barcelona (ES)
- University College London (GB)
- Université Paris Cité (FR)
- McMaster University (CA)
- University of California, Irvine (US)
- Weill Cornell Medicine (US)
- Ghent University Hospital (BE)
- University of Washington (US)
- Addenbrooke's Hospital (GB)
- Massachusetts General Hospital (US)
- Northwell Health (US)
- University of Michigan (US)
- University of Toronto (CA)
- Yale University (US)
- Maimonides Medical Center (US)
- Universidade do Estado do Rio de Janeiro (BR)
- Utrecht University (NL)
- University of Graz (AT)
- Jagiellonian University (PL)
- IRCCS Istituto Auxologico Italiano (IT)
- Azienda Ospedaliera Universitaria Integrata Verona (IT)
- Duke Medical Center (US)
- University of Padua (IT)
- Johns Hopkins University (US)
- Hôpital Bichat-Claude-Bernard (FR)
- University of North Carolina at Chapel Hill (US)
- Guy's Hospital (GB)
- Ospedale San Giovanni Bosco (IT)
- National and Kapodistrian University of Athens (GR)
- The University of Texas Medical Branch at Galveston (US)
- Brigham and Women's Hospital (US)
- Corresponding author
- Erkan, Doruk
- ORCID
- Barbhaiya, Medha
- Zuily, Stephane
- Manneville, Florian
- Amigo, Mary-Carmen
- Amoura, Zahir
- Andrade, Danieli
- Andreoli, Laura
- Atsumi, Tatsuya
- Belmont, Michael H
- Branch, D Ware
- Carvalheiras, Graziela
- Casini, Alessandro
- Cervera, Ricard
- Cohen, Hannah
- Costedoat-Chalumeau, Nathalie
- Crowther, Mark
- de Jesús, Guilherme
- Desai, Sheetal
- Devreese, Katrien M
- Duarte-García, Ali
- Garcia, David
- Gris, Jean-Christophe
- Leaf, Rebecca K
- Kello, Nina
- Knight, Jason S
- Laskin, Carl
- Lee, Alfred I
- Levy, Roger A
- Limper, Maarten
- Lockshin, Michael D
- Mayer-Pickel, Karoline
- Meroni, Pier Luigi
- Orsolini, Giovanni
- Ortel, Thomas L
- Pengo, Vittorio
- Petri, Michelle
- Pons-Estel, Guillermo
- Sanna, Giovanni
- Seshan, Surya V
- Sciascia, Savino
- Tektonidou, Maria G
- Wahl, Denis
- Willis, Rohan
- Yelnik, Cécile
- Costenbader, Karen
- Erkan, Doruk
- Alba, Paula
- Alpan, Oral
- Appenzeller, Simone
- Berkun, Yackov
- Cabral, Antonio
- Chighizola, Cecilia
- Cimaz, Rolando
- Groot, Philip G de
- Moerloose, Philippe de
- Dörner, Thomas
- Fortin, Paul
- Giannakopoulos, Bill
- Khamashta, Munther
- Kriegel, Martin
- Krilis, Steven
- Mikdashi, Jamal
- Sammaritano, Lisa
- Soybilgic, Arzu
- Woller, Scott
- Venue
- Annals of the Rheumatic Diseases
- Publisher
- Elsevier BV
- Published
- 2023-10
- Volume
- 82
- Issue
- 10
- Pages
- 1258-1270
- ISSN
- 0003-4967 (ISSN-L)
- OpenAlex
- W4386220262
- Cited by
- 493
- References
- 5
- Citations by year
- 2026: 149 · 2025: 167 · 2024: 148 · 2023: 27 · 2014: 1
- FWCI
- 103.55
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Annals of the Rheumatic Diseases (publishedVersion)
- PubMed (publishedVersion)
- Ghent University Academic Bibliography (Ghent University) (publishedVersion)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion, other-oa)
- Institutional Research Information System University of Turin (University of Turin) (submittedVersion, other-oa)
- Archive ouverte UNIGE (University of Geneva) (submittedVersion, cc-by)
- Homo Politicus (Academy of Humanities and Economics in Lodz) (submittedVersion, cc-by-nc-nd)
- HAL (submittedVersion)
- Ghent University Academic Bibliography (Ghent University) (submittedVersion)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- LillOA (Université de Lille (University Of Lille)) (submittedVersion, other-oa)
- HighWire Press Open Archive (submittedVersion)
- Funders
- Brigham and Women's Hospital
- Hospital for Special Surgery
- Weill Cornell Medical College
- Topics
- Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Keywords
- antiphospholipid syndrome, classification criteria, antiphospholipid antibodies, anticardiolipin antibodies, anti-beta2-glycoprotein I antibodies, lupus anticoagulant, thrombosis, venous thromboembolism, arterial thrombosis, pregnancy complications, valvular heart disease, hematologic manifestations, multi-criteria decision making, diagnostic specificity
- MeSH terms
- Autoantibodies, Female, Humans, Immunoglobulin G, Immunoglobulin M, Pregnancy, Rheumatology, Antiphospholipid Syndrome
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