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一例以肾病综合征为表现且合并肺隐球菌病的坏死性肾小球肾炎病例。

A case of necrotizing glomerulonephritis presenting with nephrotic syndrome associated with pulmonary cryptococcosis.

作者信息

Nakayama Masaru, Hori Kei, Ishida Itsuko, Masutani Kohsuke, Katafuchi Ritsuko

机构信息

Division of Nephrology and Clinical Research Institute, Department of Internal Medicine, National Kyushu Medical Center Hospital, 1-8-1 Jigyohama, Fukuoka 810-8563, Japan.

出版信息

Clin Exp Nephrol. 2005 Mar;9(1):74-8. doi: 10.1007/s10157-004-0337-4.

Abstract

We describe a 68-year-old man with necrotizing glomerulonephritis who presented with nephrotic syndrome accompanied by pulmonary cryptococcosis. He developed rheumatoid arthritis in July 1999 and was treated with low-dose prednisolone. He was admitted to our hospital on November 22 following the appearance of bilateral leg edema in October 2000. Laboratory tests at presentation revealed nephrotic syndrome with renal impairment. Renal biopsy specimens revealed necrotizing glomerulonephritis with crescent, but immunofluorescence study showed lack of staining for immunoglobulins or complement components. Chest X-ray and CT showed abnormal shadows in the right upper lung field, and Cryptococcus neoformans was isolated in a transbronchial lung biopsy. After the diagnosis of pulmonary cryptococcosis was made, the patient was treated with 200 mg/day fluconazole. The pulmonary abnormal shadows immediately improved and urinary protein excretion dramatically decreased. A second renal biopsy, performed about 2 months after the first biopsy, showed disappearance of crescent. Electron microscopic examination of the second renal biopsy showed partial effacement of foot processes without electron-dense deposits. Our findings suggest that necrotizing glomerulonephritis with nephrotic syndrome in this patient represented pauci-immune T-cell-mediated injury related to pulmonary cryptococcosis.

摘要

我们描述了一名68岁患有坏死性肾小球肾炎的男性,他表现为肾病综合征并伴有肺隐球菌病。他于1999年7月患上类风湿性关节炎,并接受了小剂量泼尼松龙治疗。2000年10月出现双侧腿部水肿后,于11月22日入住我院。入院时的实验室检查显示为伴有肾功能损害的肾病综合征。肾活检标本显示为伴有新月体的坏死性肾小球肾炎,但免疫荧光研究显示免疫球蛋白或补体成分染色阴性。胸部X线和CT显示右上肺野有异常阴影,经支气管肺活检分离出新型隐球菌。在诊断为肺隐球菌病后,患者接受了每日200毫克氟康唑的治疗。肺部异常阴影立即改善,尿蛋白排泄显著减少。在首次活检约2个月后进行的第二次肾活检显示新月体消失。第二次肾活检的电子显微镜检查显示足突部分消失,无电子致密沉积物。我们的研究结果表明,该患者的伴有肾病综合征的坏死性肾小球肾炎代表了与肺隐球菌病相关的少免疫T细胞介导的损伤。

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