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

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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Affiliated Institutions

• IRCCS Istituto Auxologico Italiano IT
• Harvard University US
• Hospital de Cruces ES
• Copenhagen University Hospital DK
• University of California, Los Angeles US
• University of Padua IT
• San Francisco VA Medical Center US
• Hospital for Special Surgery US
• University of Toronto CA
• Charité - Universitätsmedizin Berlin DE
• Istituti di Ricovero e Cura a Carattere Scientifico IT
• University Hospital Centre Zagreb HR
• Technische Universität Dresden DE
• Leeds Teaching Hospitals NHS Trust GB
• Medical University of Graz AT
• Cedars-Sinai Medical Center US
• Hôpital Cochin FR
• Freie Universität Berlin DE
• Rigshospitalet DK
• Heinrich Heine University Düsseldorf DE
• Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán MX
• Northwestern University US
• Humboldt-Universität zu Berlin DE
• Feinstein Institute for Medical Research US
• University of Zagreb HR
• Biomedical Research Foundation of the Academy of Athens GR
• University of Pisa IT
• Universitat de Barcelona ES
• Université Paris-Saclay FR
• University of Crete GR
• Centro Hospitalar do Porto PT
• Medical University of South Carolina US
• Brigham and Women's Hospital US
• Hôpitaux Universitaires Paris-Ouest FR
• University of Cyprus CY
• Istanbul Bilim University TR
• University of Cambridge GB
• University of Pecs HU
• Université Paris-Sud FR
• University Health Network CA
• National Institute of Arthritis and Musculoskeletal and Skin Diseases US
• Azienda Ospedaliera Universitaria Pisana IT
• Universidade do Porto PT
• Medical University of Vienna AT
• Toronto Western Hospital CA
• Inserm FR
• University Hospital Carl Gustav Carus DE
• University of Hong Kong HK
• Hospital Universitario de Gran Canaria Doctor Negrín ES
• University of Michigan US
• University of Calgary CA
• Mount Sinai Hospital CA
• New York University US
• University of Leeds GB
• McMaster University CA
• University of California, San Francisco US
• National and Kapodistrian University of Athens GR
• University of Occupational and Environmental Health Japan JP

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