Abstract
Cyclosporin (Cs-A) is an effective treatment for difficult cases of nephrotic syndrome (NS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We reviewed the charts of 10 Cs-A-dependent patients and 4 patients with steroid-dependent nephrotic syndrome (SDNS) not previously treated with Cs-A therapy. All patients had persistent NS, even after prior treatment with oral cyclophosphamide. Of 10 patients treated with Cs-A, 4 had surveillance renal biopsies consistent with Cs-A toxicity, and 8 of 10 had interstitial fibrosis prior to mycophenolate mofetil (MMF). Patients were treated with MMF, at 1,200 mg/m2 per day, in an attempt to allow weaning of Cs-A and/or steroid therapy, and reduce the frequency of relapses. Overall, a significant decrease in frequency of relapses was noted after initiation of MMF therapy. In addition, 5 patients were weaned off Cs-A by 1–2 years of follow-up. One patient was weaned off Cs-A and MMF, and remained in complete remission. However, the subgroup of patients with frequently relapsing SDNS not treated with Cs-A appeared to have a reduction in the number of relapses while on MMF that did not reach statistical significance. Two patients with intractable steroid-resistant NS continued to relapse repeatedly on MMF and Cs-A therapy. We conclude that in this small, single-center, uncontrolled experience, MMF therapy in patients with Cs-A-dependent NS appears to be effective in reducing Cs-A exposure. In addition, MMF appears to significantly decrease the frequency of relapses in this patient population. Further controlled studies are warranted to better define the potential efficacy and side effects of long-term MMF therapy in this setting.
References
Mendoza SA, Tune BM (1995) Management of the difficult nephrotic patient. Pediatr Clin North Am 42:1459–1468
Ingulli E, Singh A, Baqi N, Ahmad H, Moazami S, Tejani A (1995) Aggressive, long-term cyclosporine therapy for steroid-resistant focal segmental glomerulosclerosis. J Am Soc Nephrol 5:1820–1825
Gregory MJ, Smoyer WE, Sedman A, Kershaw DB, Valentini RP, Johnson K, Bunchman TE (1996) Long-term cyclosporine therapy for pediatric nephrotic syndrome: a clinical and histologic analysis. J Am Soc Nephrol 7:543–549
Collaborative Study Group of Sandimmune in Nephrotic Syndrome (1991) Safety and tolerability of Cyclosporin A (Sandimmune) in idiopathic nephrotic syndrome. Clin Nephrol 35:S48–S60
Report of the International Study of Kidney Disease in Children (1974) Prospective, controlled trial of cyclophosphamide therapy in children with nephrotic syndrome. Lancet II:423–427
Tricontinental Mycophenolate Mofetil Renal Transplantation Study Group (1996) A blinded, randomized clinical trial of mycophenolate mofetil for the prevention of acute rejection in cadaveric renal transplantation. Transplantation 61:1029–1037
Vanrenterghem Y (1997) The use of mycophenolate mofetil (Cellcept) in renal transplantation. Nephron 76:393–399
Miller G, Zimmerman R, Radhakrishnan J, Appel G (2000) Use of mycophenolate mofetil in resistant membranous nephropathy. Am J Kidney Dis 36:250–257
Briggs WA, Choi MJ, Scheel PJ (1998) Successful treatment of glomerular disease with mycophenolate mofetil. Am J Kidney Dis 31:213–217
Chandra M, Susin M, Abitbol C (2000) Remission of relapsing childhood nephrotic syndrome with mycophenolate mofetil. Pediatr Nephrol 14:224–226
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Barletta, GM., Smoyer, W.E., Bunchman, T.E. et al. Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndrome. Pediatr Nephrol 18, 833–837 (2003). https://doi.org/10.1007/s00467-003-1175-4
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DOI: https://doi.org/10.1007/s00467-003-1175-4