Kidney nontumor
Hereditary renal disease
Collagen type III glomerulopathy

Author: Kriselle Maris N. Lao, M.D. (see Authors page)
Editor: Joseph Grande, M.D., Ph.D.

Revised: 10 July 2018, last major update May 2018

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Collagen type III glomerulopathy
Cite this page: Lao, K.M.N. Collagen type III glomerulopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneycollagentypeIIIglomerulopathy.html. Accessed August 15th, 2018.
Definition / general
  • Also called collagenofibrotic glomerulopathy
  • Idiopathic deposition of type III collagen in glomeruli, normally absent in kidneys
Essential features
  • Deposition of abnormally curved and serpentine type III collagen fibers in mesangial and subendothelial regions
Epidemiology
Sites
Etiology
Clinical features
Laboratory
  • 10 - 100x increase in levels of procollagen type III peptide (PIIINP), not specific
  • Serum hyaluronan may be increased to > 1000x, more specific (Intern Med 2014;53:1801)
Case reports
Treatment
Microscopic (histologic) description
  • Diffuse increase in mesangial matrix and generalized widening of glomerular capillary walls with pale eosinophilic material; may not be obvious in pediatric cases
  • Negative or weakly positive with periodic acid Schiff, negative with methenamine silver, blue with Masson trichrome
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Joseph Grande, M.D., Ph.D.

Lobular mesangial expansion

Silver negative material deposits

PAS weak / negative deposits

Trichrome stains deposits blue



Images hosted on other servers:
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Left: enlarged glomerulus with hyaline cap deposits; right: lobular appearance

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With Hodgkin's lymphoma

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Stains: left-silver, right-Congo red

Positive stains
  • Strong collagen type III staining in capillary loops and mesangium (normally absent in kidneys)
Negative stains
Immunofluorescence
  • Negative; nonspecific IgM and C3 entrapment
Immunofluorescence images

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Deposits were negative for all IgG, IgM and complements

Electron microscopy description
  • Large accumulation of collagen fibrils in subendothelial glomerular basement membrane and mesangial matrix (Ultrastruct Pathol 2010;34:68)
    • Banded with 60 nm periodicity
    • Serpentine, curved, frayed
    • More prominent curvilinear structure and better detail on phosphotungstic acid or tannic acid lead staining
Electron microscopy images

Images hosted on PathOut server:

Extensive mesangial deposits

Higher power of subendothelial deposit



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Abundant subendothelial deposits of large fibers

Differential diagnosis
  • Nail-patella syndrome: has other clinical features (e.g. bone / nail abnormalities), sparser fibers, typically located in lamina densa of glomerular basement membrane versus more subendothelial or mesangial location in collagenofibrotic glomerulopathy
Board review question #1
    Onset of collagen type III glomerulopathy in childhood is associated with what manifestation?

  1. Aplastic anemia
  2. Hemolytic uremic syndrome
  3. Warm autoimmune hemolytic anemia
  4. Idiopathic thrombocytopenic purpura
Board review answer #1
B. Hemolytic uremic syndrome

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