Adult survivors of childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome treated with cyclosporine: a long-term single-center experience

Abstract Background Although evidence has confirmed that cyclosporine A (CsA) is efficacious against childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome (SD/SRNS), some patients may continue to relapse during adulthood. However, predictive factors for active disease into adulthood and renal complications in this cohort remain unknown.Methods We conducted a retrospective study on the long-term outcomes of 81 young adults with childhood-onset SD/SRNS treated with CsA. The primary endpoint was the probability of active disease into adulthood, defined as disease relapse or ongoing immunosuppressive treatment throughout the 2 years preceding the last follow-up.Results At last follow-up (median age, 23.2 years; median disease duration, 15.8 years), 44 adult patients (54%) continued to have active disease, whereas 16 (20%) and 16 (20%) patients developed mild chronic kidney disease (CKD) and hypertension, respectively. Young age at NS onset and history of relapse during initial CsA treatment were independent predictive factors for active disease into adulthood. Acute kidney injury at NS onset, focal segmental glomerulosclerosis, and irreversible CsA nephrotoxicity were identified as risk factors for the development of CKD, whereas older age was identified as a risk factor for the development of renal complications. No correlation was observed between active disease into adulthood and the development of renal complications.Conclusions After CsA initiation for SD/SRNS, more than half of adult survivors continued to have active disease. Long-term follow-up is necessary to identify the development of renal complications later in adulthood that can be attributed to prior disease and CsA treatment in childhood, irrespective of disease activity.