Collagen Type III Glomerulopathy

Collagen type III glomerulopathy (CG), also known as collagenofibrotic glomerulopathy, is caused by accumulation of collagen type III in glomeruli. Collagen type III is not a normal constituent of the extracellular matrix of glomeruli, but it is found in the interstitium and vessel walls. Clinical features of CG include proteinuria (often in the nephrotic range), edema, and hypertension.1,2 The condition can progress to end-stage kidney disease, requiring dialysis or renal transplantation. While initial reports of CG were largely in patients of Asian descent, subsequent cases in patients of European descent were published.