High-dose mizoribine therapy for childhood-onset frequently relapsing steroid-dependent nephrotic syndrome with cyclosporin nephrotoxicity

Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We report 9 adolescent patients with childhood-onset FR-SDNS who had been treated...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2005-12, Vol.20 (12), p.1744-1749
Main Authors: Ohtomo, Yoshiyuki, Fujinaga, Shu-ichiro, Takada, Masaru, Murakami, Hitohiko, Akashi, Shunji, Shimizu, Toshiaki, Kaneko, Kazunari, Yamashiro, Yuichiro
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age of Onset
Anti-Inflammatory Agents, Non-Steroidal - administration & dosage
Child
Child, Preschool
Cyclosporine
Cyclosporine - administration & dosage
Cyclosporine - adverse effects
Cyclosporine - therapeutic use
Drug Administration Schedule
Drug therapy
Drug Therapy, Combination
Female
Follow-Up Studies
Health aspects
Humans
Immunosuppressive Agents - administration & dosage
Infant
Japan - epidemiology
Male
Nephrosis, Lipoid - drug therapy
Nephrotic syndrome
Nephrotic Syndrome - diagnosis
Nephrotic Syndrome - drug therapy
Nephrotic Syndrome - epidemiology
Nephrotic Syndrome - pathology
Ribonucleosides - administration & dosage
Secondary Prevention
Time Factors
Treatment Outcome
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Summary:Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We report 9 adolescent patients with childhood-onset FR-SDNS who had been treated with long-term CsA that resulted in moderate-to-severe CsA nephropathy (CsAN). They were treated with high-dose (mean: 10.1 mg/kg per day) mizoribine (MZR) in an attempt to allow weaning of CsA and/or steroid therapy, and reduce the frequency of relapses. Seven out of 9 patients were weaned off CsA by 1-year follow-up, although in the remaining 2 patients, MZR did not show any beneficial effects. Overall, this high-dose MZR therapy results in significant steroid sparing and reduction in relapse rates in our patients. Our experience shows that high-dose MZR therapy in patients with FR-SDNS who are also CsA-dependent appears to be effective in reducing CsA exposure as well as in decreasing the frequency of relapses.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-005-2025-3