Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Clin Kidney J. 2012 Feb;5(1):7-12. doi: 10.1093/ndtplus/sfr144. Epub 2012 Jan 30.
Collagenofibrotic glomerulopathy (CG) is a rare cause of idiopathic nephrotic syndrome characterized by massive accumulation of atypical Type III collagen fibrils within the mesangial matrix and subendothelial space of the glomeruli. A definite diagnosis can be established when typical histological findings are supported by electron microscopy. This disease exhibits indolent progression and as yet has no specific treatment. The present article reviews the clinicopathological features, epidemiology and proposed mechanisms of pathogenesis of CG. A search of the English language literature identified 38 cases of CG, of which 22 are reported from Asian countries. An additional three cases are being reported from this Institute in India and are illustrated herein. These reports contribute to a better understanding of this disease, which although not as prevalent, should be considered as a differential diagnosis in cases of mesangiocapillary form of glomerular injury.
胶原纤维性肾小球病 (CG) 是一种罕见的特发性肾病综合征病因，其特征是在肾小球系膜基质和内皮下空间内大量堆积非典型 III 型胶原纤维。当典型的组织学发现得到电子显微镜的支持时，可以明确诊断。该疾病呈惰性进展，目前尚无特异性治疗方法。本文综述了 CG 的临床病理特征、流行病学和发病机制的研究进展。通过检索英文文献，共发现 38 例 CG 病例，其中 22 例来自亚洲国家。本文还报道了来自印度该研究所的另外 3 例病例，并进行了图示说明。这些报告有助于更好地了解这种疾病，尽管它并不常见，但在肾小球系膜毛细血管损伤的情况下，应将其作为鉴别诊断。
