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Collagenofibrotic glomerulopathy: three case reports in Brazil

Renata D R Ferreira1, Fabiano B Custódio, Camila S O Guimarães

  • 1Discipline of General Pathology, Biological Sciences Department, Triângulo Mineiro Federal University, Uberaba, Minas Gerais State, Brazil. rosanaasor@hotmail.com

Diagnostic Pathology
|September 29, 2009
PubMed

Insights

Collagenofibrotic glomerulopathy (CG), a kidney disease, is now reported in South America, indicating it affects diverse populations globally. This finding expands the known geographical distribution of CG beyond Japan.

Area of Science:

  • Nephrology
  • Pathology
  • Genetics

Background:

  • Collagenofibrotic glomerulopathy (CG) is a rare kidney disease.
  • Previously, CG was primarily reported in Japan, suggesting a potential geographical limitation.

Purpose of the Study:

  • To report the first cases of collagenofibrotic glomerulopathy in South America.
  • To highlight that CG is not limited to specific races or locations.

Main Methods:

  • Case series describing three patients with common clinical factors: sex, age, non-nephrotic proteinuria, and microscopic hematuria.
  • Immunofluorescence microscopy showed typically negative results for immunoglobulins and complement components.
  • Picrosyrius staining revealed reddish material in the mesangium, which appeared greenish under polarized light, indicating collagen.
  • Electron microscopy confirmed characteristic curved and disorganized collagen fibers.

Main Results:

  • Identification of collagenofibrotic glomerulopathy in three patients from South America.
  • Clinical presentation included proteinuria and hematuria with negative immunofluorescence findings.
  • Distinctive collagen fiber morphology confirmed via electron microscopy.

Conclusions:

  • These cases represent the first reported instances of CG in South America.
  • Collagenofibrotic glomerulopathy is an idiopathic renal disease with a global distribution.
  • CG should be considered in the differential diagnosis of proteinuria, particularly in unexplained cases.
Abstract

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