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Collagenofibrotic Glomerulopathy: Report a Rare Case.

Rakesh Agarwal1, Rashmi Baid2, Jotideb Mukhopadhyay1

  • 1Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, India.

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|June 16, 2020
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Summary

Collagenofibrotic glomerulopathy, a rare kidney disease, was diagnosed in an adult with nephrotic syndrome and intestinal villous atrophy. This case highlights a potential new extra-renal manifestation of this condition.

Keywords:
Collaagenofibrotic glomerulopathyNephrotic syndrome

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Area of Science:

  • Nephrology
  • Pathology
  • Gastroenterology

Background:

  • Collagenofibrotic glomerulopathy (CFG) is a rare kidney disease characterized by collagen type III deposition.
  • Fewer than 40 cases of CFG have been reported globally, often presenting with nephrotic syndrome.
  • Extra-renal manifestations like hypertension and anemia are documented, but gastrointestinal involvement is not.

Observation:

  • This report details a unique case of adult-onset nephrotic syndrome.
  • The patient also presented with partial villous atrophy of the intestine.
  • Diagnosis confirmed CFG with subendothelial and mesangial collagen type III deposition.

Findings:

  • The case presents collagenofibrotic glomerulopathy (CFG) with associated partial villous atrophy.
  • This represents a previously unreported extra-renal manifestation of CFG.
  • Elevated procollagen III peptide levels were noted, consistent with CFG.

Implications:

  • This case expands the known clinical spectrum of collagenofibrotic glomerulopathy.
  • It suggests a potential link between CFG and gastrointestinal pathology, specifically villous atrophy.
  • Further research is warranted to investigate this association and its underlying mechanisms.