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环磷酰胺和泼尼松龙联合治疗可使一名患有与类风湿性关节炎相关的肾淀粉样变性的肾病综合征患者病情缓解。

Combined treatment with cyclophosphamide and prednisolone can induce remission of nephrotic syndrome in a patient with renal amyloidosis, associated with rheumatoid arthritis.

作者信息

Maezawa A, Hiromura K, Mitsuhashi H, Tsukada Y, Kanai H, Yano S, Naruse T

机构信息

Third Department of Internal Medicine, Gunma University School of Medicine, Japan.

出版信息

Clin Nephrol. 1994 Jul;42(1):30-2.

PMID:7923963
Abstract

A 67-year-old woman, who had been diagnosed with classical rheumatoid arthritis (RA), was admitted to our hospital because of massive proteinuria. Biopsy of the kidney revealed deposition of amyloid fibrils in the subepithelial and subendothelial spaces of the glomerular capillary walls. Though the treatment with prednisolone and dipyridamole against nephrotic syndrome and amyloidosis due to RA was not effective, cyclophosphamide, which was added after tapering of prednisolone, was able to induce remission of nephrotic syndrome after two years. The levels of CRP and serum amyloid A protein (SAA) returned to within the normal limits. As the impairment of renal function is thought to be due to deposition of amyloid supplied from the precursors of amyloid fibrils filtered from the general circulation in RA patients, remission of nephrotic syndrome might result from the suppression of production of SAA or removal of amyloid fibrils. Cyclophosphamide, which has the potential both to suppress disease activity in RA and to produce degradation of amyloid fibrils in glomeruli, may be useful against renal or systemic amyloidosis complicated by RA.

摘要

一名67岁女性,被诊断为典型类风湿关节炎(RA),因大量蛋白尿入住我院。肾脏活检显示肾小球毛细血管壁上皮下和内皮下空间有淀粉样纤维沉积。尽管使用泼尼松龙和双嘧达莫治疗RA所致肾病综合征和淀粉样变性无效,但在泼尼松龙逐渐减量后加用的环磷酰胺,两年后能够诱导肾病综合征缓解。CRP和血清淀粉样蛋白A(SAA)水平恢复至正常范围。由于肾功能损害被认为是由于从RA患者体循环滤过的淀粉样纤维前体供应的淀粉样物质沉积所致,肾病综合征的缓解可能是由于SAA产生的抑制或淀粉样纤维的清除。环磷酰胺既有抑制RA疾病活动的潜力,又能使肾小球中的淀粉样纤维降解,可能对并发RA的肾或系统性淀粉样变性有用。

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