POS1295 PERFORMANCE OF 2019 EULAR/ACR CLASSIFICATION CRITERIA FOR SYSTEMIC LUPUS ERYTHEMATOSUS IN A PEDIATRIC POPULATION – A MULTICENTER STUDY
Authors: Y. Levinsky, M. Broide, S. Kagan, O. Goldberg, O. Scheuerman, R. Tal, I. Tirosh, Y. Butbul, L. Harel, G. Amarilyo
Journal: Annals of the Rheumatic Diseases (2021)

Background: The “European League Against Rheumatism” and “American College of Rheumatology” 2019 (EULAR/ACR-19) criteria for the diagnosis of Systemic Lupus Erythematosus (SLE) were recently published, with the stated goal of maintaining the level of sensitivity and raising the level of specificity for classification of SLE in adults. Objectives: We aimed to examine the function of the new EULAR/ACR-19 criteria in a population of children and compare them to the SLICC-12 and ACR-97 criteria. Methods: In this multicenter study the charts of jSLE patients from three tertiary medical centers were reviewed and compared to patients with non-jSLE diagnosis. Pediatric rheumatologists, blinded to the original diagnosis, reviewed and diagnosed all cases. Pediatric patients’ clinical and laboratory data were retrospectively extracted and then examined with regard to how they met the new and old criteria. Results: Included were 225 patients (112 jSLE, 113 non-SLE). When applied to juvenile SLE classification, the sensitivity of the new EULAR/ACR-19 criteria was 0.96 (0.9-.0.99) and the specificity was 0.89 (0.82-0.94). These were comparable to the Systemic Lupus International Collaborating Clinics (SLICC) criteria. The sensitivity of the EULAR/ACR-19 criteria improves over time and was 0.83 twelve months following disease onset, reaching 0.96 after longer than 24 months. Conclusion: Among a cohort of jSLE patients, sensitivity of the new EULAR/ACR-19 criteria was found to be high and specificity may have improved slightly compared to the SLICC-12 criteria. We support the use of the new classification criteria for pediatric patients in future jSLE studies, but it should be noted that its specificity is lower than for adults. ACR-97 SLICC-12 EULAR/ACR-19 Sensitivity (95% CI) 0.79 (0.70-0.86) 0.96 (0.9-0.99) 0.96 (0.9-.0.99) Specificity (95% CI) 0.94 (0.88-0.97) 0.85 (0.77-0.91) 0.89 (0.82-0.94) Accuracy (95% CI) 0.86 (0.81-0.9) 0.9 (0.86-0.94) 0.92 (0.88-.0.96) Positive Likelihood Ratio (95% CI) 12.7(6.1-26.2) 6.35(4.1-9.9) 9.0 (5.3-15.4) Negative Likelihood Ratio (95% CI) 0.23(0.16-0.33) 0.05(0.02-0.12) 0.05(0.02-0.12) Diagnostic odds ratio (95% CI) 55.5(22.80-135.0) 120.85(43.0-340.0) 180.1(61.3-529.4) References: [1]Smith EMD, Lythgoe H, Midgley A, Beresford MW, Hedrich CM. Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options. Clin Immunol 2019 published on December 2019. doi: 10.1016/j.clim.2019.108274 [2]Massias JS, Smith EMD, Al-Abadi E, Armon K, Bailey K, Ciurtin C, et al. Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups. Lupus 2020;29(5):474–81. [3]Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997 Sep;40(9):1725 [4]Petri M, Orbai AM, Alarcõn GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum 2012 Aug;64(8):2677-86 [5]Hartman EAR, van Royen-Kerkhof A, Jacobs JWG, Welsing PMJ, Fritsch-Stork RDE. Performance of the 2012 Systemic Lupus International Collaborating Clinics classification criteria versus the 1997 American College of Rheumatology classification criteria in adult and juvenile systemic lupus erythematosus. A systematic review and meta-an. Autoimmun Rev. 2018;17(3):316–22. [6]Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Arthritis Rheumatol 2019;71(9):1400–12. Disclosure of Interests: None declared