Kidney nontumor
Hereditary renal disease
Collagen type III glomerulopathy

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 31 October 2017, last major update August 2012

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: Collagen type III glomerulopathy
Cite this page: Sangle, N. Collagen type III glomerulopathy. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneycollagentypeIIIglomerulopathy.html. Accessed December 13th, 2017.
Definition / general
  • Hereditary glomerulpathy related to nail-patella syndrome (Pediatr Nephrol 1993;7:354)
  • Also called primary glomerular fibrosis, collagenofibrotic glomerulopathy
  • Autosomal recessive and sporadic, due to deposition of type III collagen, normally absent in kidneys
Clinical features
  • Either gender, variable age
  • Most reported cases are in Japan (Am J Kidney Dis 2007;49:499), but also reported in South America (Diagn Pathol 2009;4:33)
  • Children: increasing proteinuria and nephrotic syndrome, hypertension, hematuria, progressive renal failure, possibly hemolytic uremic syndrome
  • Adults: indolent course
  • Markedly elevated serum precursor collagen type III protein (Adv Chronic Kidney Dis 2012;19:101)
Case reports
Microscopic (histologic) description
  • Diffuse increase in mesangial matrix, generalized widening of glomerular capillary walls
Microscopic (histologic) images

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Left: enlarged glomerulus with hyaline cap deposits; right: lobular appearance

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With Hodgkin's lymphoma

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Stains: left-silver, right-Congo Red

Negative stains
  • Congo Red; stains poorly with PAS, trichrome and GMS
Immunofluorescence
  • Strong anti collagen type III staining in capillary loops and mesangium (normally absent in kidneys)
  • Negative or focal IgM deposition
Immunofluorescence images

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Deposits were negative for all IgG, IgM and complements

Electron microscopy description
  • Large accumulation of collagen fibrils in subendothelial glomerular basement membrane and mesangial matrix
Electron microscopy images

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Abundant subendothelial deposits of large fibers

Differential diagnosis
  • Hereditary onycho-osteodysplasia: also called nail-patella syndrome; fibers located in lamina densa of glomerular basement membrane versus subendothelial or mesangial location in collagenofibrotic glomerulopathy
  • Membranoproliferative glomerulonephritis: electron microscopy findings are distinctive