The Korean Journal of Medicine

The Korean Association of Internal Medicine (대한내과학회)
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Clinical courses of the adult patients with idiopathic membranous nephropathy

특발성 막성신병증의 임상경과

Yoon, Seol-Yong;Lee, Eun-Ju;Huh, Jung-Hoon;Son, Young-Ki;Han, Kyung-Kun;An, Won-Suk;Kim, Seong-Eun;Kim, Ki-Hyun
윤설영;이은주;허정훈;손영기;한경근;안원석;김성은;김기현

  • Published : 20040000
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Abstract

Background : Idiopathic membranous nephropathy (IMN) causes variable clinical courses, such as from asymptomatic urinary abnormalities, nephrotic syndrome to end-stage renal failure. We evaluated clinical findings and effects of steroid and steroid with chlorambucil in patients with IMN. Methods : We reviewed 37 cases of biopsy-proven patents of IMN whose follow-up duration was at least 2 years, retrospectively. The mean follow-up duration of the cases was 74$\pm$49 months. In the cases of steroid therpy, prednisolone 40, 50 or 60 mg/day was given for maximal 16 weeks. Steroid-chlorambucil treatment was done for the cases of no response or relapse after steroid therapy, severe nephrotic syndrome or elevated serum creatinine more than 1.2 mg/dL. We did 3 cycles of treatment. Every cycle was consisted of intravenous 1 gram of methylprednisolone for 3 days followed by prednisolone 0.5 mg/kg/day orally for 27 days then chlorambucil 0.2 mg/kg/day for 28 days. Therapeutic results were evaluated. Results : The mean age was 41$\pm$15 years and 5-6th decade was 48.6%. Male to female ratio was 1.3 : 1. Nephrotic syndrome was in 86.5% in the cases. The results of prednisolone therapy was done in 25 cases were 20% of complete remission (CR), 28% of patial remission (PR) and 52% of no respone (NR). There was no difference between the response rate and dosage of prednisolone. Steroid-chlorambucil therapy was done in 18 cases totally, 5 cases in the first treatment and 13 cases of no response or relapsed cases after prednisolone treatment. The results were 22.2% of CR, 50% of PR and 27.8% of NR. This results were no difference between steroid and chlorambucil combined therapy. Spontaneous remission was observed 35.1% of the total cases, 21.6% of spontaneous CR and 13.5% of spontaneous PR after the final observations. The final results were 16.2% of CR and 10.8% of PR after prednisolone or chlorambucil combined therapy. Progressive renal disease were developed in 6 cases (16.2%) and the mean renal surval time measured by projected reciprocal creatinine from diagnosis to the point of 0.1 was 129$\pm$79 months. Conclusion : Nephrotic syndrome was presented in 86.5% of cases and with high remission rates such as 37.8% of complete and 24.3% of partial remission in IMN. Progressive renal failure was occurred in 16.2% of cases and most of the cases progressed very slowly. There was no different results between steroid alone and chlorambucil combined therapy.

목적 : 특발성 막성신병증은 신증후군을 주 증상으로 하는 질환으로 자연관해에서부터 만성신부전증으로 진행 하는 다양한 임상상을 나타낸다고 알려져 있다. 본 질환의 다양한 임상경과에 관하여 분석함과 아울러 신증후군의 치료법으로 사용하고 있는 steroid 단독요법과 chlorambucil 병합요법의 효과를 평가하고자 하였다. 방법 : 1990년 3월부터 2003년 8월까지 동아대학교병원 내과에서 진료 받고 신생검으로 진단된 특발성 막성신병증 환자 중 2년 이상 경과관찰이 가능하였던 환자 총 37명을 대상으로 각종 임상 소견을 병력지 검토에 의한 후향적 분석을 실시하였으며, 관찰기간은 74$\pm$49개월이었다. Steroid 단독요법은 prednisolone을 1일 40 mg, 50mg 또는 60 mg을 최장 16주 동안 경구투여하였으며 치료반응에 따라 감량하였다. Steroid-chlorambucil 병합요법은 steroid 단독요법에 무반응이거나 관해 후 재발한 증례와 심한 신증후군, 혈청 creatinine이 상승한 증례에서 시행하였다. Methylprednisolone 1g을 3일간 정맥 투여하고 27일간 1일 prednisolone 0.5 mg/kg 경구투여하며, 그 후 1일 0.2 mg/kg의 chlorambucil을 28일간 경구 투여하는 것을 한 주기로 하여 총 3주기를 실시하였다. 치료 결과는 완전관해(CR), 부분관해(PR), 무반응(NR), 자연완전관해(SCR), 자연부분관해(SPR)로 평가하였다. 결과 : 총 37예의 연령은 41$\pm$15세로 40-50대가 48.6%를 차지하였고, 남녀 비는 1.3:1로 남자에 많았다. 신증후군은 86.5%의 환자에서 동반하였다. Prednisolone 단독요법을 시행한 25예의 치료 성적은 CR 20%, PR 28%, NR 52%였으며 prednisolone의 용량에 따른 차이는 없었다. Steroid-chlorambucil 병합요법은 1차 치료 5예와 prednisolone 단독요법 후 무반응 또는 재발하였던 환자 13예의 총 18예에서 실시하여 CR 22.2%, PR 50%, NR 27.8%였다. 치료반응과 재발 모두 steroid 단독요법과 차이가 없었다. 자연 관해는 13예(35.1%)에서 발생하였는데 SCR 8예(21.6%)와 SPR 5예(13.5%)였다. 최종 관찰 결과 prednisolone 요법 또는 chlorambucil 병합요법 후 CR은 6예(16.2%)와 PR 4예(10.8%)에서 유지되었다. 진행성 신부전은 6예(16.2%)에서 관찰되었고, 1/혈청 creatinine이 0.1인 직선 추세선에 의해 산출된 투석예상 시까지 신생존기간은 평균 129$\pm$79개월이었다. 결론 : 특발성 막성신병증은 약 90%에서 신증후군이 발생하고, CR 37.8%와 PR 24.3%의 높은 관해율을 보였다. 진행성 신부전은 16.2%에서 발생하였고, 대부분이 매우 느리게 진행하였다. Steroid 단독 또는 chlorambucil 병합요법의 치료 성적은 유사하였다.

Keywords

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