Systemic lupus of pediatric onset: the experience of the French overseas departments of America

Introduction: Systemic diseases of pediatric onset are more frequent in the Afro-Caribbean population. We performed a study of patients followed in French overseas departments of America (FOAD) for pediatric systemic lupus (pSLE). The aims were to describe their clinical and biological specificities during childhood. Methods: A retrospective study was conducted between January 2000 and September 2021. Listings of patients with pediatric onset were obtained through multiple sources: computerized hospital archives, registry of referent pediatricians and adult specialists in internal medicine and French National Registry for rare diseases. The spectrum of diseases studied was systemic lupus according to international criteria with pediatric onset. Results: Overall, 2148 patients were identified, and 54 patients were included. The average follow-up was 8.3 years (range: 0.3 - 25 years). We found an increase in new diagnoses throughout the years. At onset, pSLE patients had a median of 10 SLICC criteria (range: 4-12), and the median EULAR/ACR 2019 score was 38 (12 - 54). At onset, one-third of patients had renal involvement, 15% neurolupus and 41% cardiac involvement. During childhood, 54% had renal involvement, and 26% suffered from neurolupus. Patients suffered a median of 3 flares during childhood and 26% from more than 5 flares. Younger patients at onset had worst outcomes; they had more flares (median at 5 p = 0,02) and needed an average of 4 background therapies (p = 0,04). Conclusion: The outcomes of Afro-Caribbean patients, where pSLE is more frequent, were similar to those in Western countries with worse disease activity at onset. Further studies should be performed to identify the genetic and environmental factors in this population.