Kidney nontumor
Hereditary renal disease
Fibronectin glomerulopathy

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

Revised: 29 November 2017, last major update November 2017

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

PubMed Search: Fibronectin glomerulopathy [title]
Cite this page: Sangle, N. Fibronectin glomerulopathy. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneyfibronectinglomerulopathy.html. Accessed December 12th, 2017.
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
  • 3 year old boy with microhematuria and hypertension (Pediatr Nephrol 2002;17:363)
  • 34 year old man with microhematuria and hypertension (Ultrastruct Pathol 2010;34:240)
  • 41 year old man with nephrotic syndrome (Int J Clin Exp Pathol 2009;3:210)
  • Nephrol Dial Transplant 1998;13:2417
  • 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
    • 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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    CAP Enlargement of glomerular basement membrane and massive mesangial electron-dense depositsTION