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. 2023 Oct;75(10):1687-1702.
doi: 10.1002/art.42624. Epub 2023 Aug 28.

The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Medha Barbhaiya #  1 , Stephane Zuily #  2 , Ray Naden  3 , Alison Hendry  4 , Florian Manneville  5 , Mary-Carmen Amigo  6 , Zahir Amoura  7 , Danieli Andrade  8 , Laura Andreoli  9 , Bahar Artim-Esen  10 , Tatsuya Atsumi  11 , Tadej Avcin  12 , H Michael Belmont  13 , Maria Laura Bertolaccini  14 , D Ware Branch  15 , Graziela Carvalheiras  16 , Alessandro Casini  17 , Ricard Cervera  18 , Hannah Cohen  19 , Nathalie Costedoat-Chalumeau  20 , Mark Crowther  21 , Guilherme de Jesus  22 , Aurelien Delluc  23 , Sheetal Desai  24 , Maria De Sancho  25 , Katrien M Devreese  26 , Reyhan Diz-Kucukkaya  27 , Ali Duarte-Garcia  28 , Camille Frances  29 , David Garcia  30 , Jean-Christophe Gris  31 , Natasha Jordan  32 , Rebecca K Leaf  33 , Nina Kello  34 , Jason S Knight  35 , Carl Laskin  36 , Alfred I Lee  37 , Kimberly Legault  38 , Steve R Levine  39 , Roger A Levy  40 , Maarten Limper  41 , Michael D Lockshin  1 , Karoline Mayer-Pickel  42 , Jack Musial  43 , Pier Luigi Meroni  44 , Giovanni Orsolini  45 , Thomas L Ortel  46 , Vittorio Pengo  47 , Michelle Petri  48 , Guillermo Pons-Estel  49 , Jose A Gomez-Puerta  50 , Quentin Raimboug  51 , Robert Roubey  52 , Giovanni Sanna  53 , Surya V Seshan  54 , Savino Sciascia  55 , Maria G Tektonidou  56 , Angela Tincani  10 , Denis Wahl  2 , Rohan Willis  57 , Cecile Yelnik  58 , Catherine Zuily  59 , Francis Guillemin  5 , Karen Costenbader  60 , Doruk Erkan  1 ; ACR/EULAR APS Classification Criteria Collaborators
Collaborators, Affiliations
Free article

The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Medha Barbhaiya et al. Arthritis Rheumatol. 2023 Oct.
Free article

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.

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References

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