Kidney nontumor
Inherited glomerular diseases
Thin glomerular basement membrane disease / lesion


Topic Completed: 3 March 2014

Revised: 26 February 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Thin glomerular basement membrane disease / lesion

Nikhil Sangle, M.D.
Page views in 2018: 490
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Cite this page: Sangle N. Thin glomerular basement membrane disease / lesion. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneythinmem.html. Accessed October 22nd, 2019.
Definition / general
  • Hereditary, often autosomal dominant disorder of thinning of lamina dense of glomerular basement membrane; normal renal function initially, but possibly late development of renal insufficiency or hypertension
  • Also called benign familiar hematuria
Clinical features
Pathogenesis
  • Associated with abnormalities in alpha 3 and alpha 4 genes for type IV collagen
  • Rarely due to mutations in COL4A5 (Pediatr Nephrol 2010;25:545)
  • Most patients are heterozygous; in homozygotes, resembles Alport disease and progresses to renal failure, even in women (Arch Pathol Lab Med 2009;133:224)
Microscopic (histologic) description
Microscopic (histologic) images

Images hosted on other servers:
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Various images

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Glomerulus shows
no abnormalities
on H&E
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Strong staining
for alpha 3 - 5, in
comparison to
Alport syndrome
Immunofluorescence description
  • Strong linear glomerular basement membrane staining for alpha 3 and 4 protein (similar to normals, Arch Pathol Lab Med 2001;125:631), positive antibody staining to NC1 domain of glomerular basement membrane from patients with thin basement membrane disease
  • Occasional IgM and IgG deposits
Electron microscopy description
  • Uniform thinning of lamina dense of glomerular basement membrane to 200 nm in > 50% of glomerular capillaries
  • Some authors believe segmental thinning is sufficient for diagnosis (Arch Pathol Lab Med 2006;130:1533)
  • Glomerular membrane occasionally ruptures
  • No thickening, lamellation or inclusions of glomerular basement membrane
Electron microscopy images

Images hosted on other servers:
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Diffusely thin glomerular basement membranes

Differential diagnosis
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