Collagenofibrotic glomerulopathy - Case report with review of literature

Abstract

Collagenofibrotic glomerulopathy is a rare, idiopathic glomerular disease characterized by abnormal accumulation of type III collagen fibrils within the mesangial matrix and subendothelial space and a marked increase in serum type III procollagen peptide levels. Proteinuria (commonest feature), edema, hypertension, and occasional progression to end-stage renal disease are the various features of this disease. The etiology and pathogenesis remain elusive. There have been reports of the disease running in the family, suggesting the possibility of genetic transmission. We report two cases of this rare entity.

Clinical and morphological features of collagen type III glomerulopathy: a report of nine cases from a single institution

“…A summary of the previously published cases was compiled by Alchi et al . Thus far, five and 40 cases have been reported in China and in other countries, respectively . In total, the male:female ratios were 6:8 in China (including the patients presented in this paper) and 19:21 in other countries ( P = 0.764).…”

“…Collagen type III glomerulopathy is a rare, idiopathic glomerular disease characterized by the abnormal accumulation of collagen type III fibrils in the subendothelial and mesangial areas . To date, only approximately 45 cases have been reported in the literature, and the age of disease onset varies. The youngest patient was only 3 months old at the time of onset; the eldest was aged 79 years .…”

Unusual cause of glomerular deposition disease: Collagenofibrotic glomerulopathy

“…[6] Ethnic/genetic factors play an important role in etiopathogenesis. There are two fundamental theories regarding the genesis of this disease.…”

“…Light microscopy[6] shows lobular bland appearing glomeruli due to the global expansion of the mesangium with thickening of the peripheral capillary walls. The mesangial expansion is due to the accumulation of amorphous, weakly PAS- and silver-positive material.…”

“…[45] The patients range in age from 2 to 66 years, with no sex predilection. [6] The most common clinical presentation is proteinuria with or without associated nephrotic syndrome, with minor alterations in renal function. [7] Only 17 cases have been reported from India.…”