@article{3000380, title = "2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus", author = "Aringer, M. and Costenbader, K. and Daikh, D. and Brinks, R. and Mosca, M. and Ramsey-Goldman, R. and Smolen, J.S. and Wofsy, D. and Boumpas, D.T. and Kamen, D.L. and Jayne, D. and Cervera, R. and Costedoat-Chalumeau, N. and Diamond, B. and Gladman, D.D. and Hahn, B. and Hiepe, F. and Jacobsen, S. and Khanna, D. and Lerstrøm, K. and Massarotti, E. and McCune, J. and Ruiz-Irastorza, G. and Sanchez-Guerrero, J. and Schneider, M. and Urowitz, M. and Bertsias, G. and Hoyer, B.F. and Leuchten, N. and Tani, C. and Tedeschi, S.K. and Touma, Z. and Schmajuk, G. and Anic, B. and Assan, F. and Chan, T.M. and Clarke, A.E. and Crow, M.K. and Czirják, L. and Doria, A. and Graninger, W. and Halda-Kiss, B. and Hasni, S. and Izmirly, P.M. and Jung, M. and Kumánovics, G. and Mariette, X. and Padjen, I. and Pego-Reigosa, J.M. and Romero-Diaz, J. and Rúa-Figueroa Fernández, Í. and Seror, R. and Stummvoll, G.H. and Tanaka, Y. and Tektonidou, M.G. and Vasconcelos, C. and Vital, E.M. and Wallace, D.J. and Yavuz, S. and Meroni, P.L. and Fritzler, M.J. and Naden, R. and Dörner, T. and Johnson, S.R.", journal = "Arthritis and Rheumatology", year = "2019", volume = "71", number = "9", pages = "1400-1412", publisher = "John Wiley and Sons Inc", issn = "2326-5191, 2326-5205", doi = "10.1002/art.40930", keywords = "antinuclear antibody; autoantibody; antinuclear antibody; autoantibody; complement; phospholipid antibody, arthritis; Article; cranial neuropathy; cytopenia; Delphi study; disease classification; European League Against Rheumatism/American College of Rheumatology classification criteria; fever; health care survey; human; inflammation; leukopenia; lupus erythematosus nephritis; mental disease; mononeuropathy; morning stiffness; pericarditis; priority journal; sensitivity and specificity; synovitis; systemic lupus erythematosus; adult; blood; classification; cohort analysis; decision support system; Europe; female; immunology; international cooperation; male; medical society; middle aged; rheumatology; systemic lupus erythematosus; United States, Adult; Antibodies, Antinuclear; Antibodies, Antiphospholipid; Autoantibodies; Cohort Studies; Complement System Proteins; Decision Support Techniques; Delphi Technique; Europe; Female; Humans; International Cooperation; Lupus Erythematosus, Systemic; Male; Middle Aged; Rheumatology; Sensitivity and Specificity; Societies, Medical; United States", abstract = "Objective: To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). Methods: This international initiative had four phases. 1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort, and a patient survey. 2) Criteria reduction by Delphi and nominal group technique exercises. 3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. 4) Refinement of weights and threshold scores in a new derivation cohort of 1,001 subjects and validation compared with previous criteria in a new validation cohort of 1,270 subjects. Results: The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in 7 clinical (constitutional, hematologic, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and 3 immunologic (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria. Conclusion: These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered, and weighted criteria reflects current thinking about SLE and provides an improved foundation for SLE research. © 2019, American College of Rheumatology" }