COLLAGENOFIBROTIC GLOMERULOPATHY – A RARE CAUSE OF NEPHROTIC RANGE PROTEINURIA

Introduction:

Collagenofibrotic glomerulopathy (CG) is a rare cause of idiopathic nephrotic syndrome characterized by massive accumulation of atypical Type III collagen fibrils within the mesangial matrix and subendothelial space of the glomeruli.

CG may present as an isolated, sporadic form usually seen in adults, or as a familial form, with autosomal recessive inheritance, seen in children. Patients typically present with proteinuria, most often in the nephrotic range and usually microscopic hematuria.

The disease produces a progressive decline in renal function often leading to end-stage kidney disease.

Methods:

Diagnosis is made on renal biopsy where characteristic electron microscopic findings in conjunction with supporting light microscopic findings and negative immunofluorescence studies are seen. Additionally, immunohistochemical assays specific for type III collagen can be used to support the diagnosis.

Results:

A 60 years old lady who is a known case of Diabetes Mellitus and  Hypertension admitted to a super specialty hospital in New Delhi, India for management of caecal carcinoma, was found to have renal dysfunction with nephrotic range proteinuria. Renal biopsy revealed CG as the cause for the same.

Conclusions:

A high degree of suspicion and histopathological correlation can help clench the diagnosis in patients with nephrotic range proteinuria. Prompt recognition can help us guide development of targeted therapies to prevent collagen deposition in the kidneys in future.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.