Background/Purpose: Different classification criteria for systemic lupus erythematosus (SLE) have been proposed over many years. The most widely used and accepted criteria have been the 1997 ACR criteria. In 2012, the SLICC criteria were published in an attempt to improve clinical relevance of SLE criteria and address concerns with the 1997 ACR criteria. In 2017, a weighted criteria were proposed at the ACR meeting. Our aim was to validate the 2017 weighted criteria for pediatric-onset SLE.

Methods: Retrospective chart review of patients with a clinical diagnosis of SLE diagnosed before the age of 19 years at our tertiary care center between 2003 and 2018. The 2017 weighted, 2012 SLICC, and 1997 ACR classification criteria were applied to these patients, and compared against a gold standard of physician diagnosis. Autoimmune controls were defined as patients who were referred for serologies positive for ANA only but who did not fulfill criteria for diagnosis of SLE at the initial visit, or were diagnosed with another autoimmune disease.

Results: There were 156 patients (82.7% female) who were diagnosed with SLE over the past 15 years. The mean age at diagnosis was 13.1±2.8 years, with 21.8% Asian, 27.6% Black, 26.9% Caucasian, 22.4% Other, and 1.3% Unknown; 23.1% also identified as Hispanic. pSLE patients had a median of 6 of 11 (range 2-9) positive domains and a median of 21 (range 6-41) points by 2017 weighted criteria. The most commonly met domains were antibodies and complement, and the most frequently met clinical criteria were hematologic and synovitis. The sensitivity for the 2017 weighted criteria was 97.4 (CI: 0.94-0.99) and specificity was 98.4 (CI: 0.97-0.99). The median score was 21. The sensitivity for the 2012 SLICC criteria was 97.4 (CI: 0.94-0.99) and specificity was 99.7 (CI: 0.99-1.00). The sensitivity for the 1997 ACR criteria was 87.2 (CI: 0.81-0.92) and specificity was 100 (CI: 0.99-1.00).

Conclusion: The 2017 weighted criteria and the 2012 SLICC were more sensitive, and similarly specific to the 1997 ACR criteria. There were no significant difference in sensitivity and specificity between the 2017 weighted and 2012 SLICC criteria. The sensitivity findings are comparable with the observed sensitivities in the adult population. Analyses are underway to further evaluate the performances of these criteria in a larger cohort of patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/validation-of-the-2017-weighted-criteria-in-pediatric-systemic-lupus-erythematosus-sle/