Two-Year Follow-Up of a Prospective Clinical Trial of Cyclosporine for Frequently Relapsing Nephrotic Syndrome in Children

Abstract

SummaryBackground and objectives Although the safety and efficacy of cyclosporine in children with frequently relapsing nephrotic syndrome (FRNS) have been confirmed, no prospective follow-up data on relapse after cyclosporine have appeared. This study is a prospective follow-up trial after 2-year treatment with cyclosporine to investigate cyclosporine dependency after its discontinuation.Design, setting, participants, & measurements Participants who had undergone 2-year protocol treatment with microemulsified cyclosporine for FRNS between January 2000 and December 2005 were followed for an additional 2 years. The primary end point was relapse-free survival after the complete discontinuation of cyclosporine, and the secondary end point was regression-free survival (time to regression to FRNS).Results After exclusion of 7 patients who showed regression to FRNS during the 2-year treatment period, 49 children (median age, 6.5 years) were followed, and classified as children without (n=32; group A) and with (n=17; group B) relapse during the initial cyclosporine treatment. Overall, relapse-free survival probability at 24 months after cyclosporine discontinuation was 15.3% and regression to FRNS-free survival probability was 40.8%. By group, the probability of relapse-free survival was significantly higher in group A (17.9%) than in group B (8.3%) (P,0.001).Conclusions Children with FRNS who receive cyclosporine are at high risk of relapse after discontinuation, particularly those who experience relapse during cyclosporine treatment.

Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children

“…The rate of children who showed hypertension at last examination was relatively high, which might be attributed to the use of prednisolone or cyclosporine. However, the frequency of hypertension in our children with SRNS and in children with SRNS in previously reported studies was higher than that of children with FRNS [24,32], which suggests that SRNS might itself be a significant risk factor for developing hypertension.…”

Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy

“…The problem of cyclosporine therapy is that many patients suffer relapse after termination of cyclosporine therapy (cyclosporine dependence) [35][36][37][38]40]. Some reports also describe that patients who respond initially to cyclosporine may lose the therapeutic responsiveness during the course of treatment and experience repeated relapse [41] or may not respond to resumption of the therapy [35].…”

Nephrotic Syndrome: Updates and Approaches to Treatment

“…Of children with frequently relapsing or steroid dependent NS, 60-85 % will attain prolonged remission with this regimen [46••]. These children are also at high risk of relapse after discontinuing the CNI with up to 85-100 % of children experiencing a relapse after discontinuing CNI in some series [47,48]. Two randomized controlled trials of cyclosporine vs. alkylating agents in children with frequently relapsing or steroiddependent NS showed no difference in risk of relapse as long as the child was taking cyclosporine [49,50].…”