The Prognosis of Focal Segmental Glomerulosclerosis Patients with Methylprednisolone Pulse Therapy Alone

PURPOSE: Since the first report by Mendoza in 1990, there have been several studies reporting that long-term intravenous methylprednisolone(MP) pulse therapy combined with cyclosporin A(CsA) or cyclophosphamide might be beneficial for the treatment of steroid resistant focal segmental glomerulosclerosis(FSGS). We investigated the therapeutic effect of long-term MP pulse therapy without CsA or cyclophosphamide on steroid resistant FSGS. METHODS: The medical records of the 10 steroid resistant FSGS patients who were treated with MP pulse therapy by the Mendoza protocol without CsA or cyclophosphamide in our hospital were retrospectively reviewed. RESULTS: The median age at onset was 2.6 years(range 1.1-10.6 years) and the median age at the initiation of therapy was 5.7 years(range 1.8-20 years). The median duration of follow-up was 35 months(range 4-132 months). At the end of therapy, 5 patients achieved complete remission(50%) and 2 partial remission(20%), one of whom relapsed after the therapy. Three patients did not respond to the therapy, two of whom progressed to end-stage renal failure during the therapy eventually requiring kidney transplantation. CONCLUSION: Intravenous long-term MP pulse therapy without CsA or cyclophosphamide by the Mendoza protocol may be effective in a subset of patients with steroid-resistant FSGS.

The Effects of Intravenous Methylprednisolone Pulse Therapy by Mendoza Protocol in Primary and Secondary Nephrotic Syndrome

PURPOSE: Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulo -sclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid-dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. METHODS: We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methyl- prednisolone, often in combination with oral cyclophosphamide for 82 weeks. RESULTS: In all the 10 children with SD-MCNS, complete remission was visible on average of 18+/- days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was 2.1+/-.0 relpases/year, which was reduced to 1.4+/-.9 relapses/year during MP therapy(P>0.05) and rose to 2.7+/-.0 relapse/year after MP therapy. Of the 6 children with FSGS, 4 children(67%) showed complete remission, of whom 3 children(50%) remained in the remission status during the follow up period, 1.2+/-.7 years, after the end of MP therapy. 2 children(33%) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, 1.7+/-.6 years. The only side effect of MP therapy was transient hypertension in 10 children of all subjects during the intravenous infusion of methylprednisolone. CONCLUSION: We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome.