Background: Focal segmental glomerulosclerosis (FSGS) is a primary glomerular disease that usually progresses to renal failure. Although high-dose pulse methylprednisolone therapy (PMT) has been shown to be effective in the treatment of steroid-resistant FSGS, adverse effects have caused parents to hesitate in approving the treatment. The aim of this study is to investigate whether low-dose PMT based protocol for treatment of young children with steroid resistant FSGS would effectively induce remission of proteinuria and prevent the progression of renal insufficiency. Methods: This is a retrospective study. The authors treated eight children with steroid-resistant FSGS with intravenous methylprednisolone pulse 10 mg/kg per day for three consecutive days weekly for 8 weeks. Partial responders were treated with the addition of chlorambucil or cyclosporine (CsA) and four fortnightly and eight monthly pulses of high-dose PMT (30 mg/kg per day). Results: Of the eight patients, six attained complete remission initially. The median urinary protein excretion in 24 h decreased from 4.25 to 0.39 g following 8 weeks of low dose (P = 0.012). Marked decrease in urinary protein-creatinine ratio was noted soon after treatment (P = 0.012). There was a significant increase in serum albumin level after treatment compared to the pretreatment condition (median, 3.35 vs 4.1 mg/dL, P = 0.018). Five of the eight patients remained in complete remission, and one of the eight patients relapsed during follow up. Relapse responded to repeated treatments of PMT and cyclosporine. The two patients with partial remission initially progressed to renal insufficiency in one patient and end-stage renal disease in the other patient. Conclusions: Low-dose PMT caused a significant decrease in the proteinuria of Chinese children with steroid-resistant FSGS with a low frequency of intolerance.
- Focal segmental glomerulonephritis
- Steroid resistant