Kidney nontumor
Glomerular disease
Other hereditary renal disease
Fibronectin glomerulopathy


Topic Completed: 6 February 2017

Minor changes: 11 February 2020

Copyright: 2002-2020, PathologyOutlines.com, Inc.

PubMed Search: Fibronectin glomerulopathy [title]

Nikhil Sangle, M.D.
Page views in 2019: 200
Page views in 2020 to date: 168
Cite this page: Sangle N. Fibronectin glomerulopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyfibronectinglomerulopathy.html. Accessed September 29th, 2020.
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

Images hosted on other servers:
Missing Image

Eosinophilic material in mesangial regions

Missing Image

Immunoelectron microscopy with anti-fibronectin

Missing Image

Glomeruli show massive deposits of a homogeneous structureless

Missing Image

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

Images hosted on other servers:
Missing Image

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

Back to top
Image 01 Image 02