Fibronectin glomerulopathy

Kidney nontumor
Hereditary renal disease
Fibronectin glomerulopathy

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

Revised: 10 July 2018, last major update November 2017

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

PubMed Search: Fibronectin glomerulopathy [title]

Table of Contents

Cite this page: Sangle, N. Fibronectin glomerulopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyfibronectinglomerulopathy.html. Accessed September 22nd, 2018.

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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