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

Abstract

Collagen type III deposits in the subendothelial and mesangial areas. Some patients show global nodular lesions, while others show subtle changes only via PAS/silver staining. Proteinuria and hypertension are the most common symptoms, and the serum creatinine level at biopsy is an independent predictor of the doubling of serum creatinine during follow-up.

Increased miR‐374b promotes cell proliferation and the production of aberrant glycosylated IgA1 in B cells of IgA nephropathy

“…Formalin‐fixed renal tissue was embedded in paraffin using routine procedures. Thin 3 μm sections were stained with hematoxylin and eosin (HE), periodic acid‐Schiff, silver methenamine and Masson's trichrome [15]. Immunofluorescence staining was performed on 3 μm cryostat sections using FITC‐labeled rabbit anti‐human IgG, IgA, IgM, Complement (C) C3, C4 antibodies (Dako Corporation, Carpentaria, CA, USA).…”

Collagenofibrotic glomerulopathy in a kidney transplant recipient: A first report

“…Collagenofibrotic glomerulopathy, also known as primary glomerular fibrosis and collagen type III glomerulopathy, is a rare form of glomerular disease with less than 50 reported cases in the literature. 3,5 CG belongs to the category of non-amyloid non-immunoglobulinderived, organized deposits. 2 Other diseases in this category are fibronectin glomerulopathy and diabetic sclerosis.…”

“…10 It has been reported in all age groups and in different ethnicities. 3,5 CG is considered a sporadic disease. 9 However, an autosomal recessive type of inheritance affecting some families has been reported predominantly in the pediatric population.…”

“…3,4 The detection of COL3 fibers in the glomeruli can be seen in the context of glomerular lesions related to sclerosis such as diabetic glomerulosclerosis and other nephropathies. 5 In the rare entity of collagenofibrotic glomerulopathy (CG) excess atypical curled COL3 are seen on ultrastructural examination, whereas these fibers are straight in the aforementioned entities. 6,7 Herein, we are describing a first case of CG in a transplant recipient.…”

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

“…Previous light microscopy studies indicated mesangial expansion with weakly PAS-positive material and negative PAM staining, as well as negative IF results (3,20). Electron microscopy indicates typical curved and scattered collagen fibrils in the subendothelial and mesangial regions with a periodicity of 50-60 nm (21). IHC testing indicates strong expression of type III collagen within the mesangium and along GBMs (13).…”