Journal Article

Treatment of idiopathic nephrosis by immunophillin modulation

Alain Meyrier

in Nephrology Dialysis Transplantation

Published on behalf of European Renal Association - European Dialysis and Transplant Assoc

Volume 18, issue suppl_6, pages vi79-vi86
Published in print August 2003 | ISSN: 0931-0509
Published online August 2003 | e-ISSN: 1460-2385 | DOI:
Treatment of idiopathic nephrosis by immunophillin modulation

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Until 1985, glucocorticoids and cytotoxic drugs were the only treatments available for idiopathic nephrotic syndrome (nephrosis), that is, minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). Trials of cyclosporine (CsA) treatment of nephrosis, the rationale of which was based on pathophysiologic considerations, have shown that this immunophillin modulator is effective in inducing and maintaining remission in patients suffering from idiopathic nephrotic syndrome. It appears that the best results, in the order of 80% remission rate, are obtained in steroid-sensitive cases, essentially MCD, and that in steroid-resistant FSGS the drug obtains remission in no more than 20% of the cases. Addition of glucocorticoids increases the success rate to ∼30% of cases. Renal toxicity is proportional to previous impairment of renal function, primary renal disease (FSGS vs MCD) dosage >5.5 mg/kg/day and duration of treatment. The better bioavailability of the new formulation of CsA (Neoral), implies that the former dosage recommendations be reconsidered for distinctly lower figures. Repeat renal biopsy after 1 year of continuous CsA treatment is advisable, as stable serum creatinine levels may be falsely reassuring. CsA dependency is the rule during the first year of treatment. However, in some 25% of cases stable remission may be maintained after slow tapering off following 3–4 years of treatment. Other immunophillin modulators have been tried in the treatment of idiopathic nephrotic syndrome. Despite few preliminary reports indicating some success of tacrolimus the effects of this drug do not seem convincingly superior to CsA in terms of remission rate, toxicity and dependency. Rapamycin has not been tried in the treatment of nephrosis. Anecdotal cases of de novo FSGS induced by rapamycin in transplanted patients might indicate that this drug is in fact contraindicated in the treatment of nephrosis.

Keywords: adults; children; cyclosporine; focal segmental glomerulosclerosis; immunophillin modulation; idiopathic; nephrosis; nephrotic syndrome; minimal change disease; rapamycin; sirolimus; tacrolimus; treatment

Journal Article.  0 words. 

Subjects: Nephrology

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