@article{3003233,
    title = "European League against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance",
    author = "Aringer, M. and Brinks, R. and Dörner, T. and Daikh, D. 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 Schmajuk, G. and Tani, C. and Tedeschi, S.K. and Touma, Z. 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, I. 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 Costenbader, K. and Johnson, S.R.",
    journal = "Annals of the Rheumatic Diseases",
    year = "2021",
    volume = "80",
    number = "6",
    pages = "775-781",
    publisher = "BMJ Publishing Group",
    issn = "0003-4967, 1468-2060",
    doi = "10.1136/annrheumdis-2020-219373",
    keywords = "antinuclear antibody;  complement component C3;  complement component C4;  double stranded DNA, Article;  cohort analysis;  controlled study;  delirium;  diagnostic test accuracy study;  disease association;  disease classification;  disease course;  Europe;  fever;  human;  infection;  leukopenia;  major clinical study;  malignant neoplasm;  metabolic disorder;  priority journal;  psychosis;  rheumatic disease;  rheumatology;  sensitivity and specificity;  systemic lupus erythematosus",
    abstract = "Background/objectives The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019 classification criteria for systemic lupus erythematosus system showed high specificity, while attaining also high sensitivity. We hereby analysed the performance of the individual criteria items and their contribution to the overall performance of the criteria. Methods We combined the EULAR/ACR derivation and validation cohorts for a total of 1197 systemic lupus erythematosus (SLE) and n=1074 non-SLE patients with a variety of conditions mimicking SLE, such as other autoimmune diseases, and calculated the sensitivity and specificity for antinuclear antibodies (ANA) and the 23 specific criteria items. We also tested performance omitting the EULAR/ACR criteria attribution rule, which defines that items are only counted if not more likely explained by a cause other than SLE. Results Positive ANA, the new entry criterion, was 99.5% sensitive, but only 19.4% specific, against a non-SLE population that included other inflammatory rheumatic, infectious, malignant and metabolic diseases. The specific criteria items were highly variable in sensitivity (from 0.42% for delirium and 1.84% for psychosis to 75.6% for antibodies to double-stranded DNA), but their specificity was uniformly high, with low C3 or C4 (83.0%) and leucopenia <4.000/mm³ (83.8%) at the lowest end. Unexplained fever was 95.3% specific in this cohort. Applying the attribution rule improved specificity, particularly for joint involvement. Conclusions Changing the position of the highly sensitive, non-specific ANA to an entry criterion and the attribution rule resulted in a specificity of >80% for all items, explaining the higher overall specificity of the criteria set. © Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ."
}