DOI: 10.1002/art.40930 ISSN:

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 Lerstrøm, Elena 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
  • Immunology
  • Rheumatology
  • Immunology and Allergy
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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.

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