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

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...

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Main Authors: Smita Mary Matthai, Anjali Mohapatra, Neelaveni Duhli, Vinoi G David, Santosh Varughese
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2020-01-01
Series:Indian Journal of Pathology and Microbiology
Subjects:
Online Access:http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2020;volume=63;issue=5;spage=47;epage=49;aulast=Matthai
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spelling doaj-bf469164684f4099acf802e17d80e2be2020-11-25T03:03:37ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292020-01-01635474910.4103/IJPM.IJPM_341_18Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopySmita Mary MatthaiAnjali MohapatraNeelaveni DuhliVinoi G DavidSantosh VarugheseCollagenofibrotic 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.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2020;volume=63;issue=5;spage=47;epage=49;aulast=Matthaicollagenofibrotic glomerulopathynephrotic syndromerenal ultrastructural pathologytype iii collagenopathy
collection DOAJ
language English
format Article
sources DOAJ
author Smita Mary Matthai
Anjali Mohapatra
Neelaveni Duhli
Vinoi G David
Santosh Varughese
spellingShingle Smita Mary Matthai
Anjali Mohapatra
Neelaveni Duhli
Vinoi G David
Santosh Varughese
Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy
Indian Journal of Pathology and Microbiology
collagenofibrotic glomerulopathy
nephrotic syndrome
renal ultrastructural pathology
type iii collagenopathy
author_facet Smita Mary Matthai
Anjali Mohapatra
Neelaveni Duhli
Vinoi G David
Santosh Varughese
author_sort Smita Mary Matthai
title Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy
title_short Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy
title_full Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy
title_fullStr Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy
title_full_unstemmed Collagenofibrotic glomerulopathy – A rare disease diagnosed with the aid of transmission electron microscopy
title_sort collagenofibrotic glomerulopathy – a rare disease diagnosed with the aid of transmission electron microscopy
publisher Wolters Kluwer Medknow Publications
series Indian Journal of Pathology and Microbiology
issn 0377-4929
publishDate 2020-01-01
description 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.
topic collagenofibrotic glomerulopathy
nephrotic syndrome
renal ultrastructural pathology
type iii collagenopathy
url http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2020;volume=63;issue=5;spage=47;epage=49;aulast=Matthai
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AT anjalimohapatra collagenofibroticglomerulopathyararediseasediagnosedwiththeaidoftransmissionelectronmicroscopy
AT neelaveniduhli collagenofibroticglomerulopathyararediseasediagnosedwiththeaidoftransmissionelectronmicroscopy
AT vinoigdavid collagenofibroticglomerulopathyararediseasediagnosedwiththeaidoftransmissionelectronmicroscopy
AT santoshvarughese collagenofibroticglomerulopathyararediseasediagnosedwiththeaidoftransmissionelectronmicroscopy
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