Dong Junwu, Wei Honglan, Han Min, Guan Yang, Wu Yang, Li Hua
Department of Nephrology and Rheumatology, Puai Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430000, P.R. China.
Department of Nephrology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei 430000, P.R. China.
Exp Ther Med. 2015 Oct;10(4):1445-1449. doi: 10.3892/etm.2015.2695. Epub 2015 Aug 20.
Collagen type III glomerulopathy is a non-immune-mediated glomerular disease, characterized by abnormal accumulation of type III collagen fibrils within the mesangial matrix and subendothelial space. The clinical manifestations of this disease are proteinuria, peripheral edema, hypertension and occasional progression to end-stage renal disease. Collagen type III glomerulopathy is extremely rare, and its etiology and pathogenesis remain elusive. To date, only case reports are available and the majority of these are from Japan. To investigate the idiographic features of collagen type III glomerulopathy in China, we report a case of collagen type III glomerulopathy with two differing renal biopsies and review 20 cases in China. The majority of the Chinese patients were adults. Thirty percent of the patients had nephrotic syndrome, and hypertension was observed in 75% of cases. Elevated creatinine was present in 45% of patients. The pathology of collagen type III glomerulopathy in the Chinese cases was similar to that observed in other ethnicities, although certain cases were IgA-positive by immunofluorescence microscopy, and electron-dense material could be observed in the mesangial area. The onset age, clinical manifestations and pathological features of the disease are not exactly the same in China as worldwide.
III型胶原肾小球病是一种非免疫介导的肾小球疾病，其特征是III型胶原纤维在系膜基质和内皮下间隙异常蓄积。该疾病的临床表现为蛋白尿、外周水肿、高血压，偶尔会进展为终末期肾病。III型胶原肾小球病极为罕见，其病因和发病机制仍不清楚。迄今为止，仅有病例报告，其中大多数来自日本。为了研究中国III型胶原肾小球病的独特特征，我们报告了一例经两次不同肾活检确诊的III型胶原肾小球病病例，并回顾了中国的20例病例。大多数中国患者为成年人。30%的患者患有肾病综合征，75%的病例观察到高血压。45%的患者肌酐升高。中国病例中III型胶原肾小球病的病理与其他种族观察到的相似，尽管某些病例免疫荧光显微镜检查显示IgA阳性，且在系膜区可观察到电子致密物质。该疾病在中国的发病年龄、临床表现和病理特征与全球并不完全相同。
