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Journal of the Korean Society of Pediatric Nephrology 1998;2(2): 110-117.
중증 Henoch-$Sch{ddot{o}}nlein$ Purpura Nephritis 환아에서 Cyclosporine A의 치료효과
진현종, 김지홍, 김병길, 정현주
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 소아과학교실
3연세대학교 의과대학 소아과학교실
4연세대학교 의과대학 병리학교실
Therapeutic Effect of Cyclosporine A on Severe Henoch-$Sch{ddot{o}}nlein$ Purpura Nephritis
Hyun Jong Chin, Ji Hong Kim, Pyung Kil Kim, Hyeon Joo Jeong
1Departments of Pediatrics, Yonsei University, College of Medicine
2Departments of Pediatrics, Yonsei University, College of Medicine
3Departments of Pediatrics, Yonsei University, College of Medicine
4Departments of Pathology, Yonsei University, College of Medicine
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ABSTRACT
Purpose : Henoch-$Sch{ddot{o}}nlein$ 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) fur 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) fur 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 $58.8%$ 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 improvement of histologic immune-deposition compared with AZA treated group(p=0.004). So, we recommend CsA and REP rather than AZA for immunosuppresant treatment in HSPN with nephrotic syndrome.
Key words: Nephrotic syndrome | Cyclosporine A | Rifampin | Azathioprine
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