Response predictors and long-term outcomes of preprandial single-daily cyclosporine in children with steroid-dependent nephrotic syndrome caused by minimal change disease: a single-center experience

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Abstract

Background Clinical practice guidelines for idiopathic nephrotic syndrome (NS) in children recommend twice-daily cyclosporine as a preferred steroid-sparing agent for steroid-dependent nephrotic syndrome (SDNS). Although single-daily cyclosporine (S-CS) may offer an effective therapeutic option with increased compliance and reduced nephrotoxicity, response predictors and long-term outcomes following this regimen remain unclear in this cohort. Methods A retrospective study was conducted on children with SDNS caused by minimal change disease (MCD) who were treated with preprandial S-CS to maintain 2-hour post-dose levels of 500–700 ng/mL between October 2005 and December 2021. The primary endpoint was the probability of SDNS-free survival during S-CS therapy. The secondary endpoint was the long-term outcome at the last visit. Results After initiating S-CS therapy in 48 children, 31 patients, including 18 who did not experience relapse during treatment, were able to discontinue steroid dependency (response group), while 17 patients experienced treatment failure. The median time to the first relapse after NS diagnosis was significantly longer in the response group than in the treatment failure group (4.3 vs . 2.1 months, p < 0.05). Multivariate logistic regression analysis identified a longer time until the first relapse (≥ 2.2 months) after NS diagnosis as an independent predictive factor for a favorable response to S-CS therapy (odds ratio: 21.0, p < 0.005). None of the patients progressed to chronic kidney disease Stage 3 or higher. Conclusions S-CS may offer a promising treatment option for children with SDNS caused by MCD who do not experience early relapse following NS diagnosis.

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