Background: Cyclosporine has improved remission rates in children with steroid-resistant nephrotic syndrome (SRNS). However, little prospective long-term follow-up data is available.
Methods: We prospectively followed and analyzed 5-year outcomes of all 35 patients enrolled in our previous prospective multicenter trial with cyclosporine and steroids in children with SRNS. At enrollment, 23 cases were classified as minimal change (MC), five as diffuse mesangial proliferation (DMP), and seven as focal segmental glomerulosclerosis (FSGS).
Results: Renal survival at 5 years (median 7.7 years) was 94.3 %. Patient status was complete remission (CR) in 31 (88.6 %) (MC/DMP, 25; FSGS, 6); partial remission in one (FSGS); and non-remission in three (MC/DMP), including chronic kidney disease and end-stage kidney disease in one each. Among 31 patients with CR, 22 (71.0 %) were receiving treatment with immunosuppressants at 5 years, including cyclosporine in 19, and seven of these 22 continued to show frequent relapse. Response to cyclosporine at 4 months predicted 5-year outcome in 31 of 35 patients.
Conclusions: Although SRNS treatment with cyclosporine provides high renal survival and remission rates, many children require ongoing immunosuppression. Management has advanced from the prevention of end-stage kidney disease to the long-term maintenance of remission and management of relapse after induction therapy.