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 new candidate weighted criteria were proposed at the ACR meeting that included entry criteria. Our aim was to validate the 2012 SLICC criteria compared to 1997 ACR and 2017 weighted criteria for SLE in 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 2002 and 2017. The ACR and SLICC classification criteria were applied to these patients. The 2017 weighted criteria were also applied for patients diagnosed after October 2014 as this was the date our laboratory began using the ANA Hep2 assay, which is an entry criterion for the new weighted criteria. We excluded patients diagnosed at another center and those for whom no records were available for review. All criteria sets were compared against a gold standard of physician diagnosis. Autoimmune controls were defined as patients who were referred for serologies positive for ANA but who did not fulfill criteria for diagnosis of SLE at the initial visit, or were diagnosed with another autoimmune disease.

Results: There were 150 patients (82% female) who were diagnosed with SLE over the past 15 years. The mean age at diagnosis was 13±2.7 years, with 23% Asian, 28% Black, 26% White, and 26% Caucasian, and 23% Other; 24% also identified as Hispanic. The sensitivity for the 1997 ACR criteria was 90.3 (CI: 0.84-0.95) and specificity was 95.9 (CI: 0.90-0.99). The sensitivity for the 2012 SLICC criteria was 99.3 (CI: 0.96-0.99) and specificity was 95.7 (CI: 0.90-0.99). The 2017 weighted criteria did not produce significant results as the patient sample size compared was too small.

Conclusion: The 2012 SLICC criteria were more sensitive than, but with similar specificity as, the 1997 ACR criteria. These findings are comparable with the observed statistics seen in the adult population. Further patient samples are needed to determine the test characteristics for the new weighted candidate criteria.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/validation-of-the-2012-systemic-lupus-international-collaborating-clinics-slicc-classification-criteria-compared-to-the-1997-acr-criteria-and-2017-candidate-weighted-criteria-for-lupus-in-pedia/