2023 ACR/EULAR antiphospholipid syndrome classification criteria

• Published in: Annals of the rheumatic diseases Vol. 82; no. 10; pp. 1258 - 1270
• Main Authors: Barbhaiya, Medha, Zuily, Stephane, Naden, Ray, Hendry, Alison, Manneville, Florian, Amigo, Mary-Carmen, Amoura, Zahir, Andrade, Danieli, Andreoli, Laura, Artim-Esen, Bahar, Atsumi, Tatsuya, Avcin, Tadej, Belmont, H Michael, Bertolaccini, Maria Laura, Branch, D Ware, Carvalheiras, Graziela, Casini, Alessandro, Cervera, Ricard, Cohen, Hannah, Costedoat-Chalumeau, Nathalie, Crowther, Mark, de Jesús, Guilherme, Delluc, Aurelien, Desai, Sheetal, Sancho, Maria De, Devreese, Katrien M, Diz-Kucukkaya, Reyhan, Duarte-García, Ali, Frances, Camille, Garcia, David, Gris, Jean-Christophe, Jordan, Natasha, Leaf, Rebecca K, Kello, Nina, Knight, Jason S, Laskin, Carl, Lee, Alfred I, Legault, Kimberly, Levine, Steve R, Levy, Roger A, Limper, Maarten, Lockshin, Michael D, Mayer-Pickel, Karoline, Musial, Jack, Meroni, Pier Luigi, Orsolini, Giovanni, Ortel, Thomas L, Pengo, Vittorio, Petri, Michelle, Pons-Estel, Guillermo, Gomez-Puerta, Jose A, Raimboug, Quentin, Roubey, Robert, Sanna, Giovanni, Seshan, Surya V, Sciascia, Savino, Tektonidou, Maria G, Tincani, Angela, Wahl, Denis, Willis, Rohan, Yelnik, Cécile, Zuily, Catherine, Guillemin, Francis, Costenbader, Karen, Erkan, Doruk
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 01.10.2023; BMJ Publishing Group
• Subjects: Antibodies; Antiphospholipid antibodies; Antiphospholipid syndrome; Antiphospholipid Syndrome - diagnosis; Autoantibodies; Autoimmune diseases; Cardiolipin; Cardiology and cardiovascular system; Classification; Clinical trials; Decades; Disease; Female; Glycoprotein I; Human health and pathology; Humans; Immunoglobulin G; Immunoglobulin M; Laboratories; Life Sciences; Literature reviews; Microvasculature; Morbidity; Patients; Pregnancy; Rheumatology; Rhumatology and musculoskeletal system; Thromboembolism; Thrombosis; Veins & arteries; North America; Europe; antiphospholipid syndrome; antibodies, antiphospholipid; thrombosis; Antibodies; Antiphospholipid; Thrombosis; Antiphospholipid syndrome
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/ard-2023-224609

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.