Kidney nontumor / medical renal
Thin membrane disease
Author: Nikhil Sangle, M.D.
Last author update: 3 March 2014
Last staff update: 13 September 2021
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Thin glomerular basement membrane disease / lesion
Table of Contents
Definition / general | Clinical features | Pathogenesis | Microscopic (histologic) description | Microscopic (histologic) images | Immunofluorescence description | Electron microscopy description | Differential diagnosisCite this page: Sangle N. Thin membrane disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneythinmem.html. Accessed April 18th, 2024.
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
- 1 - 2% of general population (Arch Pathol Lab Med 2006;130:699), rare after age 50; 20 - 25% have isolated hematuria
- Asymptomatic, hematuria discovered on routine urinalysis; rarely gross hematuria; mild proteinuria in 60% (Pol J Pathol 2009;60:35)
- Normal renal function, excellent prognosis, but up to 30% develop late onset renal insufficiency or hypertension
- Investigation of families and long term follow up recommended (Kidney Int 2010;78:1041, Nephrol Dial Transplant 2005;20:545)
- A diagnosis of exclusion
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
- Red blood cells in Bowman space and renal tubules, but otherwise normal (Arch Pathol Lab Med 1988;112:794)
Microscopic (histologic) images
Images hosted on other servers:
Various images
no abnormalities
on H&E
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
Differential diagnosis
- Alport syndrome in women without typical clinical findings: thick glomerular basement membrane, lamellation, granular inclusions and loss of staining for alpha 3 and alpha 4 protein (Hum Pathol 2002;33:836)
- IgA nephropathy
- Nonspecific GBM thinning is also seen in lupus nephritis and postinfectious glomerulonephritis
