Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion

ABSTRACT Collagenofibrotic glomerulopathy, a very rare glomerular disease, characterised by abnormal deposition of Type III collagen in the mesangium and subendothelial space. This disease may have an indolent course clinically but is capable of finally progressing to end stage renal failure. Thus,...

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Main Authors: Anisha Manocha, Pallav Gupta
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2020-06-01
Series:Journal of Clinical and Diagnostic Research
Subjects:
Online Access:https://jcdr.net/articles/PDF/13748/44208_CE[Ra1]_F(SHU)_PF1(AJ_KM)_PN(SL).pdf
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spelling doaj-19ff0927e7744b6298074b623e462de02020-11-25T03:27:45ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2020-06-01146ED01ED0210.7860/JCDR/2020/44208.13748Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial ExpansionAnisha Manocha0Pallav Gupta1Senior Resident, Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India.Consultant, Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India.ABSTRACT Collagenofibrotic glomerulopathy, a very rare glomerular disease, characterised by abnormal deposition of Type III collagen in the mesangium and subendothelial space. This disease may have an indolent course clinically but is capable of finally progressing to end stage renal failure. Thus, it is imperative for clinicians and pathologists to be mindful of this entity due to its rarity, nonspecific presenting signs and symptoms and varied differentials on histopathology which must be ruled out. A definite diagnosis depends on the awareness of this entity, a high index of suspicion and confirmation by immunohistochemistry and electron microscopy. Here, the present authors report a case of a 41-year-old hypertensive female who came with persistent pedal oedema and proteinuria. The biopsy showed nodular expansion of the mesangium by a homogenous, Congo red negative, eosinophilic material. A diagnosis of Collagenofibrotic glomerulopathy was confirmed on immunohistochemistry and electron microscopy.https://jcdr.net/articles/PDF/13748/44208_CE[Ra1]_F(SHU)_PF1(AJ_KM)_PN(SL).pdfcollagen type iiiglomerular mesangiumkidney injurynephrotic syndromeproteinuria
collection DOAJ
language English
format Article
sources DOAJ
author Anisha Manocha
Pallav Gupta
spellingShingle Anisha Manocha
Pallav Gupta
Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion
Journal of Clinical and Diagnostic Research
collagen type iii
glomerular mesangium
kidney injury
nephrotic syndrome
proteinuria
author_facet Anisha Manocha
Pallav Gupta
author_sort Anisha Manocha
title Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion
title_short Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion
title_full Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion
title_fullStr Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion
title_full_unstemmed Collagenofibrotic Glomerulopathy: A Rare Diagnosis and Seldom Thought of Differential for Nodular Glomerular Mesangial Expansion
title_sort collagenofibrotic glomerulopathy: a rare diagnosis and seldom thought of differential for nodular glomerular mesangial expansion
publisher JCDR Research and Publications Private Limited
series Journal of Clinical and Diagnostic Research
issn 2249-782X
0973-709X
publishDate 2020-06-01
description ABSTRACT Collagenofibrotic glomerulopathy, a very rare glomerular disease, characterised by abnormal deposition of Type III collagen in the mesangium and subendothelial space. This disease may have an indolent course clinically but is capable of finally progressing to end stage renal failure. Thus, it is imperative for clinicians and pathologists to be mindful of this entity due to its rarity, nonspecific presenting signs and symptoms and varied differentials on histopathology which must be ruled out. A definite diagnosis depends on the awareness of this entity, a high index of suspicion and confirmation by immunohistochemistry and electron microscopy. Here, the present authors report a case of a 41-year-old hypertensive female who came with persistent pedal oedema and proteinuria. The biopsy showed nodular expansion of the mesangium by a homogenous, Congo red negative, eosinophilic material. A diagnosis of Collagenofibrotic glomerulopathy was confirmed on immunohistochemistry and electron microscopy.
topic collagen type iii
glomerular mesangium
kidney injury
nephrotic syndrome
proteinuria
url https://jcdr.net/articles/PDF/13748/44208_CE[Ra1]_F(SHU)_PF1(AJ_KM)_PN(SL).pdf
work_keys_str_mv AT anishamanocha collagenofibroticglomerulopathyararediagnosisandseldomthoughtofdifferentialfornodularglomerularmesangialexpansion
AT pallavgupta collagenofibroticglomerulopathyararediagnosisandseldomthoughtofdifferentialfornodularglomerularmesangialexpansion
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