Abstract
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.