2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus

Martin Aringer(University Hospital Carl Gustav Carus), Karen H. Costenbader(Brigham and Women's Hospital), David Daikh(San Francisco VA Medical Center), Ralph Brinks(Heinrich Heine University Düsseldorf), Marta Mosca(University of Pisa), Rosalind Ramsey‐Goldman(Northwestern University), Josef S Smolen(Medical University of Vienna), David Wofsy(University of California, San Francisco), Dimitrios T. Boumpas(National and Kapodistrian University of Athens), Diane L. Kamen(Medical University of South Carolina), David Jayne(University of Cambridge), Ricard Cervera(Universitat de Barcelona), N. Costedoat‐Chalumeau(Hôpital Cochin), Betty Diamond(Feinstein Institute for Medical Research), Dafna D. Gladman(University of Toronto), Bevra Hahn(University of California, Los Angeles), Falk Hiepe(Humboldt-Universität zu Berlin), Søren Jacobsen(Copenhagen University Hospital), Dinesh Khanna(University of Michigan), Kirsten Lerstrøm(South Essex Partnership University Foundation NHS Trust), Elena Massarotti(Brigham and Women's Hospital), Joseph M. McCune(University of Michigan), Guillermo Ruiz‐Irastorza(Hospital de Cruces), Jorge Sánchez‐Guerrero(Mount Sinai Hospital), Matthias Schneider(Heinrich Heine University Düsseldorf), Murray B. Urowitz(University of Toronto), George Βertsias(University of Crete), Bimba F. Hoyer(Humboldt-Universität zu Berlin), Nicolai Leuchten(University Hospital Carl Gustav Carus), Chiara Tani(University of Pisa), Sara K. Tedeschi(Brigham and Women's Hospital), Zahi Touma(University of Toronto), Gabriela Schmajuk(San Francisco VA Medical Center), Branimir Anić(University Hospital Centre Zagreb), Florence Assan(Université Paris-Sud), Tak Mao Chan(University of Hong Kong), Ann E. Clarke(University of Calgary), Mary K. Crow(Hospital for Special Surgery), László Czirják(University of Pecs), Andrea Doria(University of Padua), W. Graninger(Medical University of Graz), Bernadett Halda-Kiss(University of Pecs), Sarfaraz Hasni(National Institute of Arthritis and Musculoskeletal and Skin Diseases), Peter Izmirly(New York University), Michelle Jung(University of Calgary), Gabór Kumánovics(University of Pecs), Xavier Mariette(Université Paris-Sud), Ivan Padjen(University Hospital Centre Zagreb), José María Pego‐Reigosa, Juanita Romero‐Díaz(Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán), Íñigo Rúa‐Figueroa Fernández(Hospital Universitario de Gran Canaria Doctor Negrín), Raphaèle Séror(Université Paris-Sud), Georg Stummvoll(Medical University of Vienna), Yoshiya Tanaka(University of Occupational and Environmental Health Japan), Maria G. Tektonidou(National and Kapodistrian University of Athens), Carlos Vasconcelos(Universidade do Porto), Edward M Vital(University of Leeds), Daniel J. Wallace(Cedars-Sinai Medical Center), Şule Yavuz(Istanbul Bilim University), Pier Luigi Meroni(IRCCS Istituto Auxologico Italiano), Marvin J. Fritzler(University of Calgary), Ray Naden(McMaster University), Thomas Dörner(Humboldt-Universität zu Berlin), Sindhu R. Johnson(Mount Sinai Hospital)
Arthritis & Rheumatology
August 6, 2019
10.1002/art.40930
Cited by 2,473Open Access
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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 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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