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

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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References

Aringer M, Dorner T, Leuchten N, Johnson S . Toward new criteria for systemic lupus erythematosus-a standpoint. Lupus. 2016; 25(8):805-11. DOI: 10.1177/0961203316644338. View

Felson D, Anderson J . Methodological and statistical approaches to criteria development in rheumatic diseases. Baillieres Clin Rheumatol. 1995; 9(2):253-66. DOI: 10.1016/s0950-3579(05)80189-x. View

Johnson S, Naden R, Fransen J, van den Hoogen F, Pope J, Baron M . Multicriteria decision analysis methods with 1000Minds for developing systemic sclerosis classification criteria. J Clin Epidemiol. 2014; 67(6):706-14. PMC: 4134523. DOI: 10.1016/j.jclinepi.2013.12.009. View

Johnson S, Goek O, Singh-Grewal D, Vlad S, Feldman B, Felson D . Classification criteria in rheumatic diseases: a review of methodologic properties. Arthritis Rheum. 2007; 57(7):1119-33. DOI: 10.1002/art.23018. View

Tsokos G . Systemic lupus erythematosus. N Engl J Med. 2011; 365(22):2110-21. DOI: 10.1056/NEJMra1100359. View

Schmajuk G, Hoyer B, Aringer M, Johnson S, Daikh D, Dorner T . Multicenter Delphi Exercise to Identify Important Key Items for Classifying Systemic Lupus Erythematosus. Arthritis Care Res (Hoboken). 2017; 70(10):1488-1494. PMC: 6026087. DOI: 10.1002/acr.23503. View

Bertsias G, Pamfil C, Fanouriakis A, Boumpas D . Diagnostic criteria for systemic lupus erythematosus: has the time come?. Nat Rev Rheumatol. 2013; 9(11):687-94. DOI: 10.1038/nrrheum.2013.103. View

Touma Z, Cervera R, Brinks R, Lorenzoni V, Tani C, Hoyer B . Associations Between Classification Criteria Items in Systemic Lupus Erythematosus. Arthritis Care Res (Hoboken). 2019; 72(12):1820-1826. DOI: 10.1002/acr.24078. View

Wallace D, Stohl W, Furie R, Lisse J, McKay J, Merrill J . A phase II, randomized, double-blind, placebo-controlled, dose-ranging study of belimumab in patients with active systemic lupus erythematosus. Arthritis Rheum. 2009; 61(9):1168-78. PMC: 2758229. DOI: 10.1002/art.24699. View

10.

Ines L, Silva C, Galindo M, Lopez-Longo F, Terroso G, Romao V . Classification of Systemic Lupus Erythematosus: Systemic Lupus International Collaborating Clinics Versus American College of Rheumatology Criteria. A Comparative Study of 2,055 Patients From a Real-Life, International Systemic Lupus Erythematosus.... Arthritis Care Res (Hoboken). 2015; 67(8):1180-5. DOI: 10.1002/acr.22539. View

11.

Littlejohn E, Marder W, Lewis E, Francis S, Jackish J, McCune W . The ratio of erythrocyte sedimentation rate to C-reactive protein is useful in distinguishing infection from flare in systemic lupus erythematosus patients presenting with fever. Lupus. 2018; 27(7):1123-1129. PMC: 5938146. DOI: 10.1177/0961203318763732. View

12.

Tedeschi S, Johnson S, Boumpas D, Daikh D, Dorner T, Diamond B . Multicriteria decision analysis process to develop new classification criteria for systemic lupus erythematosus. Ann Rheum Dis. 2019; 78(5):634-640. PMC: 7057251. DOI: 10.1136/annrheumdis-2018-214685. View

13.

Hsieh M, Everhart J, Byrd-Holt D, Tisdale J, Rodgers G . Prevalence of neutropenia in the U.S. population: age, sex, smoking status, and ethnic differences. Ann Intern Med. 2007; 146(7):486-92. DOI: 10.7326/0003-4819-146-7-200704030-00004. View

14.

Ganczarczyk L, Urowitz M, Gladman D . "Latent lupus". J Rheumatol. 1989; 16(4):475-8. View

15.

Petri M, Orbai A, Alarcon G, Gordon C, Merrill J, Fortin P . Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012; 64(8):2677-86. PMC: 3409311. DOI: 10.1002/art.34473. View

16.

Rahman A, Isenberg D . Systemic lupus erythematosus. N Engl J Med. 2008; 358(9):929-39. DOI: 10.1056/NEJMra071297. View

17.

Costenbader K, Schur P . We need better classification and terminology for "people at high risk of or in the process of developing lupus". Arthritis Care Res (Hoboken). 2014; 67(5):593-6. PMC: 4391980. DOI: 10.1002/acr.22484. View

18.

Johnson S, Khanna D, Daikh D, Cervera R, Costedoat-Chalumeau N, Gladman D . Use of Consensus Methodology to Determine Candidate Items for Systemic Lupus Erythematosus Classification Criteria. J Rheumatol. 2018; 46(7):721-726. DOI: 10.3899/jrheum.180478. View

19.

Nair R, Aggarwal R, Khanna D . Methods of formal consensus in classification/diagnostic criteria and guideline development. Semin Arthritis Rheum. 2011; 41(2):95-105. PMC: 3131416. DOI: 10.1016/j.semarthrit.2010.12.001. View

20.

Singh J, Solomon D, Dougados M, Felson D, Hawker G, Katz P . Development of classification and response criteria for rheumatic diseases. Arthritis Rheum. 2006; 55(3):348-52. DOI: 10.1002/art.22003. View