Collagen type III glomerulopathy: A case report and review of 20 cases

Abstract

Abstract. Collagen type III glomer ulopathy is a non-immune-mediated glomerular disease, characterized by abnormal accumulation of type III collagen fibrils within the mesangial matrix and subendothelial space. The clinical manifestations of this disease are proteinuria, peripheral edema, hypertension and occasional progression to end-stage renal disease. Collagen type III glomerulopathy is extremely rare, and its etiology and pathogenesis remain elusive. To date, only case reports are available and the majority of these are from Japan. To investigate the idiographic features of collagen type III glomerulopathy in China, we report a case of collagen type III glomerulopathy with two differing renal biopsies and review 20 cases in China. The majority of the Chinese patients were adults. Thirty percent of the patients had nephrotic syndrome, and hypertension was observed in 75% of cases. Elevated creatinine was present in 45% of patients. The pathology of collagen type III glomerulopathy in the Chinese cases was similar to that observed in other ethnicities, although certain cases were IgA-positive by immunofluorescence microscopy, and electron-dense material could be observed in the mesangial area. The onset age, clinical manifestations and pathological features of the disease are not exactly the same in China as worldwide.

The Influence of a Nanopatterned Scaffold that Mimics Abnormal Renal Mesangial Matrix on Mesangial Cell Behavior

“…In addition, one network-forming nanopattern and an unpatterned flat control were also used. Our nanopatterning was inspired by the fact that the diameters of collagen fibrils in natural MM are 40 to 100 nm [23]. However, due to the limitations of the ALD of samples on the patterns fabricated by EBL, approximately 80 nm was the minimum dimension.…”

“…Moreover, TGF-β1 has been demonstrated to induce α-SMA expression by upregulating the fibronectin receptor, integrin α5β1, in human MCs during renal fibrosis in vivo and in vitro [39,55,56]. Increased expression of TGF-β1 by MCs has also been demonstrated when MCs are cultured on COL1 gels [23]. In this study, the F80/200 nanopattern induced higher α-SMA, TGF-β1, and integrin α5β1 expressions in MES13 cells, which is similar to MCs under disease status, revealing that increased COL1 and fibronectin might be modulated by TGF-β1 produced by activated MCs.…”

“…Native collagen fibers are arranged into a 3D structure and are around 300 nm to 1 μm in diameter [21,22]. They are hierarchically structured from collagen fibrils in 40 to 100 nm diameters that are identifiable in the MM [23]. In addition, the renal basement membrane consists of a meshwork-forming structure with pores ranging from 4 to 50 nm [24].…”

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

“…Collagen type III glomerulopathy (CG) is an idiopathic glomerular disease, characterized by massive accumulation of collagen type III within the glomerular mesangial regions and basement membrane. This rare renal disease has been previously reported in Japan and occasionally in China (1). CG may be classified into two subtypes, designated as collagenofibrotic glomerulopathy and nail patella syndrome (NPS), according to the location of collagen deposition.…”

“…The clinical manifestations of CG are not sufficiently specific for diagnosis uninformed by pathological findings. 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).…”