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

ABSTRACT

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.