Type III collagen disorders: A case report and review of literature

Abstract

Collagen type III is a normal component of interstitium and blood vessels. Collagenofibrotic glomerulopathy (CG) and nail patella syndrome (NPS) are the diseases of abnormal type III collagen deposition. In spite of these curved frayed structures with a periodicity of 45-60 nm are deposited in subendothelium and mesangium in CG, they are found only in the basement membrane in NPS. The clinical features of CG are confined to the kidney, NPS has associated extra-renal manifestations. Electron microscopy is essential to make the renal diagnosis in both these rare diseases. Both the entities considered to be systemic diseases evidence to suggest similar deposition in other organs, understanding etiopathogenesis and disease progression await research.

Telmisartan alleviates collagen type III glomerulopathy: A case report with literature review

“…1B ). This pathology had not been previously encountered at our hospital; literature consultation of the results for light microscopy indicated CG ( 2 ). Subsequent IHC examination suggested deposition of collagen III in subendothelial and mesangial regions ( Fig.…”

“…The dysregulated formation of type III collagen is deposited in the GBM, subendothelium or mesangium, thus propagating fibrosis. According to the anatomic region of deposition, CG is divided into collagenofibrotic glomerulopathy and NPS ( 2 ). Deposition is mostly detected in the subendothelium and mesangium of patients with CG, while deposition is mainly located at the basement membrane with extra-renal manifestations in patients with NPS ( 2 ).…”

“…According to the anatomic region of deposition, CG is divided into collagenofibrotic glomerulopathy and NPS ( 2 ). Deposition is mostly detected in the subendothelium and mesangium of patients with CG, while deposition is mainly located at the basement membrane with extra-renal manifestations in patients with NPS ( 2 ). The incidence of CG is low with only ~100 cases reported worldwide to date ( Table II ) most of them in Asia ( 2 , 13-16 ).…”

“…In CG, deposits containing type III collagen fibrils are mainly located in the subendothelium and mesangium, while those in NPS are located at the basement membrane. Furthermore, the clinical symptoms of CG are confined to the kidney, while patients with NPS usually have a range of extra-renal manifestations (2). CG is a rare renal disease that may occur at any age in humans and animals.…”

“…The major clinical manifestations of CG are proteinuria, hypertension and subsequent slowly progressive chronic kidney disease (1,4,5). Electron microscopic examination of glomerular basement membrane (GBM) biopsy specimens has revealed massive deposition of banded type III collagen fibrils (diameter, 50-60 nM) (2). In settings such as developing countries without ready access to electron microscopy, immunohistochemical (IHC) examination may be useful.…”