Abstract

PURPOSE: Henoch-Schonlein purpura nephritis(HSPN) accompanied by nephrotic syndrome(NS) is known to have a poor prognosis and effective treatment is still controversial, even though both corticosteroids and immunosuppresant have been used for therapy. Cyclosporine A(CsA) is a well known immunosuppresant and widely used in renal transplantation and glomerular diseases especially steroid resistant. The aims of this study was to evaluate the therapeutic effect of CsA and to compare CsA with previously reported our data of rifampin(RFP) and azathioprine(AZA) in children with HSPN accompanied by NS.
METHODS
37 HSPN patients with NS confirmed by renal biopsy were selected. Of these, 17 patients were treated with CsA(5 mg/kg/day) for 6-8 months, 7 children were treated with RFP(10-20 mg/kg/day) for 9-12 months and 13 patients were treated with AZA(2 mg/kg/day) for 8 months. Along with these regimens, low dose oral prednisolone(0.5-1 mg/kg, qod) was also used. Sequential renal biopsy was done in all patients 1 month after termination of treatment. RESULTS: Complete remission rate of nephrotic syndrome was 5S.8% in CsA, 57.1% in RFP and 38.4% in AZA group after 17, 22, 11 months of mean follow-up period. Overall remission rate including partial remission was 88.2% in CsA, 85.7% in RFP and 84.6% in AZA group. Disappearance rate of hematuria was 58.8% in CsA, 57.1% in RFP and 46.2% in AZA group. Improvement of grade of clinical status was observed in 17 out of 17 CsA, 7 out of 7 RFP and 10 out of 13 AZA group. Improvement of pathologic class on sequencial renal biopsy was shown in 5 CsA(29.4%), none RFP(0%) and 2 AZA group(12.4%). Improvement on histologic immune-deposition was seen in 15 CsA(88.2%), 6 RFP(85.9%) and 4 AZA group(30.8%). CONCLUSION: In conclusion, Both CsA and RFP treated groups showed better result in complete remission rate of nephrotic syndrome and significant inprovement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and RFP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.

• Keywords: Laurence Moon-Bardet Biedl Syndrome;Chronic renal failure