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新月体性肾小球肾炎的静脉“冲击”环磷酰胺治疗

Intravenous "pulse" cyclophosphamide therapy of crescentic glomerulonephritis.

作者信息

Kunis C L, Kiss B, Williams G, D'Agati V, Appel G B

机构信息

Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York.

出版信息

Clin Nephrol. 1992 Jan;37(1):1-7.

PMID:1541058
Abstract

Pulse steroids and monthly intravenous cyclophosphamide with a rapid steroid taper were used to treat seven patients with crescentic glomerulonephritis. Despite major impairment of renal function, all patients had a marked improvement in GFR by six months and improvement in proteinuria occurred in six of the seven patients. Two patients were able to discontinue dialysis. The doses of steroids and cyclophosphamide used were less than in comparable studies and side-effects of therapy were minimal. Repeat renal biopsies at over one year demonstrated marked glomerulosclerosis despite relatively stable renal function. Histologic analysis of total glomerular involvement indices on serial biopsies suggested no recruitment of new glomeruli into the crescenteric process once treatment had been initiated. Thus, intravenous pulse cyclophosphamide with pulse steroid therapy appears to be a safe and effective initial therapy for some patients with RPGN. Significant glomerulosclerosis on repeat biopsies suggests that glomerulosclerosis may be predetermined by the initial degree of irreversible glomerular damage and that the long-term course of the disease remains guarded.

摘要

采用脉冲式类固醇激素及每月静脉注射环磷酰胺并快速减量的方法治疗了7例新月体性肾小球肾炎患者。尽管肾功能严重受损,但所有患者在6个月时肾小球滤过率(GFR)均有显著改善,7例患者中有6例蛋白尿情况有所改善。2例患者能够停止透析。所使用的类固醇激素和环磷酰胺剂量低于同类研究,且治疗的副作用极小。一年多后重复肾活检显示,尽管肾功能相对稳定,但仍有明显的肾小球硬化。对系列活检中总肾小球受累指数的组织学分析表明,一旦开始治疗,新的肾小球不会再参与新月体形成过程。因此,静脉注射脉冲式环磷酰胺联合脉冲式类固醇激素治疗似乎是一些急进性肾小球肾炎(RPGN)患者安全有效的初始治疗方法。重复活检时出现的显著肾小球硬化表明,肾小球硬化可能由最初不可逆的肾小球损伤程度预先决定,且该疾病的长期病程仍不容乐观。

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Nephrol Dial Transplant. 2017 Nov 1;32(11):1818-1830. doi: 10.1093/ndt/gfx047.
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Long-term clinical course in acute crescentic glomerulonephritis.急性新月体性肾小球肾炎的长期临床病程
Int Urol Nephrol. 1996;28(3):439-53. doi: 10.1007/BF02550508.