摘要目的：分析Ⅲ型胶原肾小球病的临床病理特点并探讨其相关病因、发病机制及预后。方法收集北京大学医学部病理学系从2005年至2014年收集的5例Ⅲ型胶原肾小球病的肾活检病例，进行常规光学显微镜（ HE、PASM、PAS、Masson染色）、免疫荧光（免疫球蛋白、补体的多种标记）、Ⅲ型胶原免疫组织化学染色及透射电镜检查，分析其病变及临床特点。结果Ⅲ型胶原肾小球病患者多表现为大量蛋白尿或肾病综合征；病理特点为肾小球系膜基质增生，基底膜增厚，并可出现系膜结节状硬化，各种免疫球蛋白和补体阴性，仅免疫组织化学出现Ⅲ型胶原阳性，透射电镜检查可见肾小球系膜区和基底膜大量Ⅲ型胶原沉积。其病因、发病机制不明，可能与Ⅲ型胶原代谢的基因异常有关。结论Ⅲ型胶原肾小球病较罕见，其病因和发病机制可能为Ⅲ型胶原代谢的基因异常，确诊的依据是在肾小球内发现大量Ⅲ型胶原沉积，目前缺乏有效的治疗方法，预后较差。

abstractsObjective To study the clinicopathologic features of collagen Ⅲglomerulopathy and its cause, pathogenesis and prognosis.Methods Five cases of collagen Ⅲglomerulopathy that collected from 2005 to 2014 were observed by renal biopsy.The morphologic characteristics were studied by light microscopy , immunofluorescence , immunohistochemical and electron microscopy.Results The glomerular mesangium became expansion but no hypercellularity , basement membrane appeared thickened.The glomeruli showed collagen type Ⅲdeposit by immunohistochemistry method , and collagen fibers increased by electron microscopy.The patients often show serious proteinuria , nephrotic syndrome and renal function damage.Conclusions Collagen Ⅲ glomerulopathy is an idiopathic glomerular disease , characterized by massive accumulation of collagen type Ⅲ within the glomerular mesangial areas and basement membrane.Collagen Ⅲglomerulopathy is extremely rare.The etiology and pathogenesis may relate to the abnormality of collagen Ⅲgene.There is no specific treatment for it and its prognosis is poor.