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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.
Archives of Iranian Medicine
|June 16, 2020
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.
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.
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