Kidney nontumor / medical renal

Glomerular disease

Other hereditary renal disease

Fibronectin glomerulopathy

Last author update: 6 February 2017
Last staff update: 15 September 2023

Copyright: 2002-2024,, Inc.

PubMed Search: Fibronectin glomerulopathy

Nikhil Sangle, M.D.
Page views in 2024 to date: 199
Cite this page: Sangle N. Fibronectin glomerulopathy. website. Accessed May 19th, 2024.
Definition / general
  • Massive fibronectin deposition in glomeruli due to autosomal dominant, non sex-linked disorder with 1q32 abnormality (Am J Hum Genet 1998;63:1724, OMIM #601894)
  • Proteinuria, often nephrotic syndrome, microhematuria, hypertension and progressive loss of renal function
  • May recur after renal transplant
Case reports
Microscopic (histologic) description
  • Lobular accentuation of glomeruli with minimal hypercellularity (Nihon Jinzo Gakkai Shi 1999;41:49)
  • Marked enlargement of mesangium and subendothelial space due to massive deposition of fibronectin and fibulin (PAS+, Congo red negative homogenous substance, Mod Pathol 2012;25:709), causing obliteration of capillary lumens
Microscopic (histologic) images

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Eosinophilic material in mesangial regions

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Immunoelectron microscopy with anti-fibronectin

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Glomeruli show massive deposits of a homogeneous structureless

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Massive flooding of the glomerulus with fibronectin

Immunofluorescence description
  • Strongly positive for plasma fibronectin (not cell-derived fibronectin), scanty immunoglobulin or complement deposition
Electron microscopy description
  • Fibronectin has dense granular appearance with 12 - 16 nm fibrils
Electron microscopy images

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Enlargement of glomerular basement membrane and massive mesangial electron-dense deposits

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