Format

Send to

Choose Destination
Indian J Pathol Microbiol. 2020 Feb;63(Supplement):S47-S49. doi: 10.4103/IJPM.IJPM_341_18.

Collagenofibrotic glomerulopathy - A rare disease diagnosed with the aid of transmission electron microscopy.

Author information

1
Department of GI Sciences, Central Electron Microscopy Facility, Wellcome Trust Research Laboratory, Vellore, Tamil Nadu, India.
2
Department of Nephrology, Christian Medical College, Vellore, Tamil Nadu, India.
3
Department of General Pathology, Christian Medical College, Vellore, Tamil Nadu, India.

Abstract

Collagenofibrotic glomerulopathy (CFG) is a rare idiopathic kidney disease characterized by abnormal deposition of atypical Type III collagen fibers in the glomerulus causing subendothelial and mesangial expansion, manifesting as progressive renal dysfunction accompanied by proteinuria. The majority of CFG cases reported in literature are from Japan where this disease entity was initially recognized. There is an increased awareness and diagnosis of this rare renal disease in India with the recent increase in utilization of electron microscopy (EM) in clinical diagnostic settings. We describe a 28-year-old Bangladeshi woman who presented with hypertension and nephrotic range proteinuria not amenable to treatment with steroids and cyclophosphamide, whose renal biopsy demonstrated diagnostic ultrastructural features of CFG. This illustrative case is presented to highlight the role of EM analysis for diagnostic accuracy in renal biopsy evaluation in addition to demonstrating the unusual renal biopsy findings of this rare entity.

KEYWORDS:

Collagenofibrotic glomerulopathy; Type III collagenopathy; nephrotic syndrome; renal ultrastructural pathology

PMID:
32108627
DOI:
10.4103/IJPM.IJPM_341_18
Free full text

Supplemental Content

Full text links

Icon for Medknow Publications and Media Pvt Ltd
Loading ...
Support Center