The performances of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 classification criteria in pediatric systemic lupus erythematosus.

Objective Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The ACR (American College of Rheumatology) 1997, SLICC (Systemic Lupus International Collaborating Clinics) 2012, and EULAR (European League Against Rheumatism)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric SLE patients. Methods Pediatric SLE patients (n=262; 80.9% female) were included from three different centers in Turkey. As controls, 174 children (60.9% female) with other diseases who had ANA (antinuclear antibody) test results were included. The gold standard for SLE diagnosis was expert opinion. Results The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 68.7%, 95.4%, and 91.6%, respectively. The specificities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 94.8%, 89.7%, and 88.5%, respectively. 18 SLE patients met the SLICC 2012 but not the EULAR/ACR 2019 criteria. Among these, hematologic involvement was prominent (13/18; 72.2%). Eight SLE patients fulfilled the EULAR/ACR 2019 but not the SLICC 2012 criteria. Among these, joint involvement was prominent (6/8; 75%). Conclusion This is the largest cohort study of pediatric SLE testing the performances of all three classification criteria. The SLICC 2012 criteria yielded the best sensitivity, while the ACR 1997 criteria had the best specificity. SLICC 2012 criteria performed better than EULAR/ACR 2019 criteria. Separation of different hematological manifestations in the SLICC 2012 criteria might have contributed to the higher performance of this criteria set.