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.
- systemic lupus erythematosus
- classification criteria
- consensus methods
- multi-criteria decision analysis
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Permission This article is published simultaneously in the September 2019 issue of Arthritis & Rheumatology.
Handling editor David S Pisetsky
Contributors MA, SRJ, TD, KC, DD, RB, MM, RR-G, JSS, DW, DB, DLK, DJ and RN have been involved in the planning and execution of the project and in writing the manuscript. RC, NC-C, BD, DDG, BH, FH, SJ, DK, KL, EM, W. JM, GR-I, JS-G, MS, MU, GB, BFH, NL, CT, SKT, ZT, GS, BA, FA, TMC, AEC, MKC, LC, AD, WG, BH-K, SH, PMI, MJ,GK, XM, IP, JMP-R, JR-D, IR-F, RS, GHS, YT, MGT, CV, EMV, DJW, SY, PLM and MJF have each significantly contributed to the body of work of the project and involved in correcting and finalising the manuscript.
Funding This study was supported by American College of Rheumatology and European League Against Rheumatism.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval Local ethics committees where applicable for chart based review of data.
Provenance and peer review Not commissioned; externally peer reviewed.