Type III Collagen Glomerulopathy

Type III Collagen Glomerulopathy

Helen Liapis, MD

Joseph Gaut, MD, PhD

Type III collagen glomerulopathy has a varied glomerular pathology. In this particular case, the glomeruli are hypercellular with a lobular appearance. (Courtesy G. Herrera, MD.)

Immunohistochemical stain for collagen III shows deposits of collagen III in the GBM and mesangium in a case of type III collagen glomerulopathy. Normal glomeruli have no detectable collagen III.



  • Collagenofibrotic glomerulopathy


  • Idiopathic glomerular disease defined by accumulation of mesangial and subendothelial type III collagen

  • Initially described by Arakawa and colleagues in 1979


Proposed Etiologies

  • Probably systemic disease of collagen metabolism

    • Increased level of N-terminal procollagen type III in blood

      • Indicative of increased synthesis/catabolism

      • Deposit in glomeruli

      • Excreted in urine

  • Occasional cases in siblings suggest a genetic component, as yet unidentified

  • Some believe this is a primary disease of the glomerulus



  • Incidence

    • Rare (˜ 0.1% of biopsies)

  • Age

    • All ages

  • Gender

    • M:F = 1:1

  • Ethnicity

    • Majority of reported cases are Japanese

    • Cases also reported in USA, Europe, India, South America, China


  • Microhematuria

  • Hypertension

  • Edema

  • Proteinuria, nephrotic range in 60%

  • Anemia

    • Adults typically have anemia of chronic disease

    • Children occasionally present with thrombotic microangiopathy

  • Hemolytic uremic syndrome, children

Laboratory Tests

  • 10-100x increase in serum and urine N-terminal procollagen type III peptide (PIIINP)


  • Drugs

    • No specific treatment

    • Steroid therapy is reported to slow disease progression


  • Variable disease course, limited data

    • ˜ 35% of adults and ˜ 90% of children progress to end-stage renal failure


Histologic Features

  • Glomeruli

    • Lobular or mesangial hypercellularity, variable

      • Nodular pattern at times resembles diabetic glomerulopathy

    • Expansion of mesangium with eosinophilic, silver-positive material

    • Thickening of GBM

      • Occasional double contours

    • Closure of capillary loops

    • No inflammation

  • No specific changes in tubules, interstitium, or vessels

Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Type III Collagen Glomerulopathy
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