European League against Rheumatism (EULAR)/American College of Rheumatology (ACR) SLE classification criteria item performance

Martin Aringer*, Ralph Brinks, Thomas Dörner, David Daikh, Marta Mosca, Rosalind Ramsey-Goldman, Josef S. Smolen, David Wofsy, Dimitrios T. Boumpas, Diane L. Kamen, David Jayne, R. Cervera, Nathalie Costedoat-Chalumeau, Betty Diamond, Dafna D. Gladman, Bevra Hahn, Falk Hiepe, Søren Jacobsen, Dinesh Khanna, Kirsten LerstrømElena Massarotti, Joseph McCune, Guillermo Ruiz-Irastorza, Jorge Sanchez-Guerrero, Matthias Schneider, Murray Urowitz, George Bertsias, Bimba F. Hoyer, Nicolai Leuchten, Gabriela Schmajuk, Chiara Tani, Sara K. Tedeschi, Zahi Touma, Branimir Anic, Florence Assan, Tak Mao Chan, Ann Elaine Clarke, Mary K. Crow, László Czirják, Andrea Doria, Winfried Graninger, Bernadett Halda-Kiss, Sarfaraz Hasni, Peter M. Izmirly, Michelle Jung, Gábor Kumánovics, Xavier Mariette, Ivan Padjen, José M. Pego-Reigosa, Juanita Romero-Diaz, Íñigo Rúa-Figueroa, Raphaèle Seror, Georg H. Stummvoll, Yoshiya Tanaka, Maria G. Tektonidou, Carlos Vasconcelos, Edward M. Vital, Daniel J. Wallace, Sule Yavuz, Pier Luigi Meroni, Marvin J. Fritzler, Ray Naden, Karen Costenbader, Sindhu R. Johnson

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

30 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)775-781
Number of pages7
JournalAnnals of the rheumatic diseases
Issue number6
StatePublished - Jun 1 2021


  • antibodies
  • antiphospholipid
  • autoantibodies
  • lupus erythematosus
  • synovitis
  • systemic

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Rheumatology
  • Immunology and Allergy
  • Immunology


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