Kidney non-tumor - Collagen type III glomerulopathy

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Kidney non-tumor

Hereditary renal disease

Collagen type III glomerulopathy

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 28 August 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Hereditary glomerulpathy related to nail-patella syndrome (Pediatr Nephrol 1993;7:354)
● Also called primary glomerular fibrosis, collagenofibrotic glomerulopathy
● Autosomal recessive and sporadic, due to deposition of type III collagen, normally absent in kidneys

Clinical features
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● Either gender, variable age
● Most reported cases are in Japan (Am J Kidney Dis 2007;49:499), but also reported in South America (Diagn Pathol 2009;4:33)
● Children: increasing proteinuria and nephrotic syndrome, hypertension, hematuria, progressive renal failure, possibly hemolytic uremic syndrome
● Adults: indolent course
● Markedly elevated serum precursor collagen type III protein (Adv Chronic Kidney Dis 2012;19:101)

Case reports
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● 26 year old man with simultaneous Hodgkin lymphoma (Saudi J Kidney Dis Transpl 2011;22:126)
● 43 year old woman and 20 year old man (Indian J Nephrol 2011;21:52)

Micro description
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● Diffuse increase in mesangial matrix, generalized widening of glomerular capillary walls

Micro images
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Various images including EM

Left: diffuse thickening of capillary walls and moderate expansion of mesangial matrix; right: thickening associated with podocyte hypertrophy

Left: enlarged glomerulus with hyaline cap deposits; right: lobular appearance

With Hodgkin's lymphoma

Stains: left-silver, right-Congo Red

Negative stains
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● Congo Red; stains poorly with PAS, trichrome and GMS

Immunofluorescence
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● Strong anti-collagen type III staining in capillary loops and mesangium (normally absent in kidneys)
● Negative or focal IgM deposition

Immunofluorescence images
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Deposits were negative for all IgG, IgM and complements

Electron microscopy description
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● Large accumulation of collagen fibrils in subendothelial glomerular basement membrane and mesangial matrix

Electron microscopy images
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Left to right: collagen fibrils; marked thickening of capillary wall; bundles of collagen fibrils in mesangial matrix and capillary wall

Abundant subendothelial deposits of large fibers with long spacing striations

Differential diagnosis
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● Hereditary onycho-osteodysplasia: also called nail-patella syndrome; fibers located in lamina densa of glomerular basement membrane versus subendothelial or mesangial location in collagenofibrotic glomerulopathy
● Membranoproliferative glomerulonephritis: electron microscopy findings are distinctive

End of Kidney non-tumor > Hereditary renal disease > Collagen type III glomerulopathy

Ref Updated: 6/8/12

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