Comparative diagnostic performance of ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria in childhood-onset systemic lupus erythematosus: A retrospective study from a tertiary care centre in south India

Abstract Background and Objective Childhood-onset systemic lupus erythematosus (cSLE) accounts for 10%–20% of overall lupus cases. Data on the epidemiology of cSLE in our population are limited, and there are no exclusive classification criteria tailored for childhood lupus. The primary objective was to describe the epidemiological and clinical profile of cSLE in our centre. The secondary objective was to evaluate the performance of the 1997 American College of Rheumatology (ACR), the 2012 Systemic Lupus International Collaborating Clinics (SLICC), and the European Alliance of Associations for Rheumatology/ American College of Rheumatology (EULAR/ACR) 2019 classification criteria in cSLE. Methods We conducted a retrospective medical record review of children with a clinical diagnosis of systemic lupus erythematosus (SLE) at our tertiary care centre between January 2024 and July 2024. Children with overlap connective tissue diseases were excluded. All three classification criteria were applied and compared against the gold standard of physician diagnosis. Clinical and laboratory features were analysed, and the sensitivity of each set of criteria was calculated. Results Sixty-six children with clinically confirmed cSLE were included. The female-to-male ratio was 5.6: 1, with a median age at symptom onset of 15.5 years and a median diagnostic delay of 6 months. The most frequent manifestations were fever (83.0%), alopecia (70.0%), arthritis (64.0%), oral ulcers (39.0%), acute cutaneous lupus (67.0%), leukopenia (24.0%), and biopsy-proven lupus nephritis (31.0%). The SLICC 2012 criteria demonstrated the highest sensitivity (95.5%), followed by EULAR/ACR 2019 (93.9%) and ACR 1997 (84.8%). Notably, 4.5% of clinically diagnosed cases did not fulfil any existing criteria. Alopecia, hypocomplementemia, and biopsy-proven nephritis were frequently missed by ACR 1997 but captured by SLICC 2012. Conclusion The SLICC 2012 criteria showed superior sensitivity compared to ACR 1997, while EULAR/ACR 2019 also performed well. However, a small subset of clinically diagnosed cSLE cases remained unclassified by all criteria, highlighting the need for pediatric-specific classification frameworks.