Résumé
Aims: the clinical presentation, treatment, and short-term outcome of steroid-sensitive nephrotic syndrome (SSNS) in pediatric patients have been extensively studied. By contrast, there are limited data available on the long-term outcome of these patients in adulthood. This retrospective study aimed to assess the adult outcomes of a large cohort of patients with pediatric-onset SSNS in Occitanie region, France.Methods: patients born before 1997 with a pediatric onset SSNS followed up in Occitanie were included. Clinical and biological data were collected for each patient. The patients were considered as cured if there was no relapse or 4 years. Patient outcomes were analysed at transfer time to adulthood and at last followup. Risk factors for active SSNS and chronic kidney disease in adulthood were analysed. Results: 134 patients were included in the study. At the transfer time to the adult nephrology team, 96 patients (71%) had still active SSNS. Risk factors for active disease included younger age at SSNS onset, the need for prednisolone pulses to obtain remission at the first episode, and the use of immunosuppressive therapies. At the last follow-up in adulthood out of the 96 patients with active disease at transfer time, 14 were lost of follow-up, 39 were considered cured, and 43 were not. 11 patients had chronic renal failure and 3 of them reached end-stage-renal disease. No specific risk factors for SSNS persistence in adulthood were identified.The educational level was similar to the general population of Occitanie and the percentage of active workers was higher. The disease strongly influenced the daily life (professional, private, or educational) for 72.1% of patients surveyed. Conclusion: this study highlights a higher rate of active SSNS at the transfer time to adulthood than previously described. However, the long-term prognosis remains favorable with nearly 90% of patients having normal renal function and about two-thirds considered as cured at the last follow-up.