Kidney non-tumor - Fibronectin glomerulopathy

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Kidney non-tumor

Hereditary renal disease

Fibronectin glomerulopathy

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 29 August 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● 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
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● 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

Micro description
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● 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

Micro images
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Various images

Recurrence in renal allograft

Massive mesangial expansion by intensely PAS+ deposits that were negative for silver and Congo red stains

Lobular accentuation of glomeruli

Trichrome stain

Fibronectin immunostain

Immunofluorescence
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● Strongly positive for plasma fibronectin (not cell-derived fibronectin), scanty immunoglobulin or complement deposition

Electron microscopy description
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● 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

End of Kidney non-tumor > Hereditary renal disease > Fibronectin glomerulopathy

Ref Updated: 6/8/12

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