목 적 :신증후군을 동반한 Henoch-$Sch{ddot{o}}nlein$ purpura nephritis는 예후가 좋지 않은 것으로 알려져 있으며 현재 스테로이드와 여러 면역억제제로 치료하고 있으나 정립된 고식적인 치료방법은 없다. Cyclosporine A는 잘 알려진 면역억제제로서 현재 신장 이식환자나 스테로이드에 반응하지 않는 사구체 질환에 많이 쓰이고 있다. 본 연구의 목적은 신증후군을 동반한 소아 Henoch-$Sch{ddot{o}}nlein$ purpura nephritis환자에서 cyclosporine A(CsA)의 치료효과를 알아보고, 이미 보고된바 있는 rifampin(RFP)과 azathioprine(AZA)과의 치료효과를 비교하고자 함이다. 방 법 : 신장조직검사를 통해 확인된 총 37명의 신증후군을 동반한 Henoch-$Sch{ddot{o}}nlein$ purpura nephritis환자가 이 연구에 포함되었으며, 이중 17명은 평균 6-8개월간 CsA(5 mg/kg/day)로, 7명은 9-12개월간 RFP(10-20 mg/kg/day)으로, 13명은 8개월간 AZA(2 mg/kg/day)으로 치료하였다. 모든 환자에서 저용량 경구 스테로이드(0.5-1 mg/kg, qod)를 함께 사용하였으며 치료 종결 후 모든 환자에서 1개월 후에 추적 신장조직검사를 시행하였다. 결 과 : 신증후군의 완전관해율은 평균 추적 관찰 기간 16, 21, 11개월 후 CsA group $58.8\%$, RFP group $57.1\%$, AZA group $38.4\%$이었고, 부분관해를 포함한 관해율은 CsA group $88.2\%$, RFP group $85.7\%$, AZA group $84.6\%$ 이었다. 혈뇨의 소실율은 CsA group $58.8\%$, RFP group $57.1\%$, AZA group $41.2\%$ 이었다. 임상상태의 호전을 보인 경우는 CsA group은 17명 중 17명, RFP group은 7명 중 7명, AZA group은 13명 중 10명이었다. 두 번의 신장조직검사를 통해 병리등급이 상향조정된 경우는 CsA group 5명($29.4\%$), RFP group 0명($0\%$), AZA group 2명($12.4\%$) 이었고, 면역형광검사 소견상 호전을 보인 경우는 CsA group 15명($88.2\%$), RFP group 6명($85.9\%$), AZA group 4명($30.8\%$) 이었다. 결 론 : 본 연구 결과, CsA는 RFP처럼 우수한 신증후군의 완전관해율을 보였으며, 면역형광 검사상에서도 의미있게 호전된 소견을 보이는 바(P=0.004) 아직 치료방법이 확립되지 않은 상황에서 CsA는 RFP과 더불어 신증후군이 동반된 심한 HSP 신염에서 추천할만한 약제로 사료된다.
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.
