2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus

Martin Aringer*, Karen Costenbader, David Daikh, Ralph Brinks, Marta Mosca, Rosalind Ramsey-Goldman, Josef S. Smolen, David Wofsy, Dimitrios T. Boumpas, Diane L. Kamen, David Jayne, Ricard 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, Chiara Tani, Sara K. Tedeschi, Zahi Touma, Gabriela Schmajuk, 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 Fernández, 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, Thomas Dörner, Sindhu R. Johnson

*Corresponding author for this work

Research output: Contribution to journalArticle

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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 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 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 seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (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 reflect current thinking about SLE and provide an improved foundation for SLE research.

Original languageEnglish (US)
Pages (from-to)1151-1159
Number of pages9
JournalAnnals of the Rheumatic Diseases
Volume78
Issue number9
DOIs
StatePublished - Sep 1 2019

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Keywords

  • classification criteria
  • consensus methods
  • lupus
  • multi-criteria decision analysis
  • systemic lupus erythematosus
  • validation

ASJC Scopus subject areas

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

Cite this

Aringer, M., Costenbader, K., Daikh, D., Brinks, R., Mosca, M., Ramsey-Goldman, R., Smolen, J. S., Wofsy, D., Boumpas, D. T., Kamen, D. L., Jayne, D., Cervera, R., Costedoat-Chalumeau, N., Diamond, B., Gladman, D. D., Hahn, B., Hiepe, F., Jacobsen, S., Khanna, D., ... Johnson, S. R. (2019). 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Annals of the Rheumatic Diseases, 78(9), 1151-1159. https://doi.org/10.1136/annrheumdis-2018-214819