Descriptive epidemiology of Pediatric-onset auto-immune and auto-inflammatory diseases in the French West Indies and French Guiana ; Épidémiologie descriptive des maladies auto-immunes et auto-inflammatoires à début pédiatrique aux Antilles et en Guyane française

Introduction: The epidemiology of pediatric-onset autoimmune and autoinflammatory diseases is poorly described in the Afro-Caribbean populations and, more generally, in Afro-descendant populations. The population of the French Departments of America (TFA) is predominantly Afro-descendant, with a universal and free health care system. The aim of this study was to describe the epidemiology, clinical and biological characteristics and prognosis of children treated for these diseases in the TFA.Methods: This is a retrospective study carried out between 01.01.2000 and 01.01.2024. Listings of patients were obtained from multiple sources: computerized hospital archives and national hospital-based surveillance system, registry of pediatricians and adult specialists in internal medicine and the national registry for rare diseases. Data were then extracted by consulting paper or computerized medical records. The diseases were defined according to international criteria: connective tissue diseases, Still's disease in children and adults, pediatric inflammatory multisystem syndrome (PIMS) and Kawasaki disease.Results: We identified 2.148 patients over a 24-year period, and 156 patients were included. The most common autoimmune disease diagnosed (102) was systemic lupus (53%), followed by dermatomyositis (21%). The mean follow-up of the cohort was 8.3 years (0.3 - 25 years) and the mean age at last news was 21.2 years (14 - 36.7). The incidence of pediatric lupus in the TFAs was 3 times higher than in mainland France, in line with adult data. We observed an increase in the number of new cases over the years. Patients with pediatric lupus had a more florid initial symptomatology (activity scores) and a richer immunological profile than the Caucasian series. Patients under 12 years of age at diagnosis (26%) had the most severe disease. The percentage of renal and neurological involvement and the overall prognosis were similar to the European and North American series. Twenty-one patients had juvenile dermatomyositis, with 33% ...