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Collagenofibrotic glomerulopathy-a review.

Duggal R, Nada R, Rayat CS, Rane SU, Sakhuja V, Joshi K - Clin Kidney J (2012)

Bottom Line: A search of the English language literature identified 38 cases of CG, of which 22 are reported from Asian countries.An additional three cases are being reported from this Institute in India and are illustrated herein.These reports contribute to a better understanding of this disease, which although not as prevalent, should be considered as a differential diagnosis in cases of mesangiocapillary form of glomerular injury.

View Article: PubMed Central - PubMed

Affiliation: Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

ABSTRACT
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. A definite diagnosis can be established when typical histological findings are supported by electron microscopy. This disease exhibits indolent progression and as yet has no specific treatment. The present article reviews the clinicopathological features, epidemiology and proposed mechanisms of pathogenesis of CG. A search of the English language literature identified 38 cases of CG, of which 22 are reported from Asian countries. An additional three cases are being reported from this Institute in India and are illustrated herein. These reports contribute to a better understanding of this disease, which although not as prevalent, should be considered as a differential diagnosis in cases of mesangiocapillary form of glomerular injury.

No MeSH data available.


Related in: MedlinePlus

Electron microscopic features of collagenofibrotic glomerulopathy. (a) Expanded subendothelial and mesangial space by deposition of fibrillary material. The overlying podocytes show foot process effacement (uranyl acetate and lead citrate; original magnification ×10 000); (b) the collagen deposits are noted in the sub-endothelial location and have a disorganized appearance. The glomerular basement membrane (indicated by arrow) itself shows no collagen fibrils (uranyl acetate and lead citrate; original magnification ×17 000); (c) collagen fibrils have typical curvilinear and disorganized morphology when transversely cut indicative of atypical Type III collagen (uranyl acetate and lead citrate; original magnification ×21 500); (d) On high magnification, the organized deposits have banded appearance (indicated by arrow) with typical periodicity of 60 nm, indicative of fibrillary Type III collagen (uranyl acetate and lead citrate; original magnification ×28 000).
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fig2: Electron microscopic features of collagenofibrotic glomerulopathy. (a) Expanded subendothelial and mesangial space by deposition of fibrillary material. The overlying podocytes show foot process effacement (uranyl acetate and lead citrate; original magnification ×10 000); (b) the collagen deposits are noted in the sub-endothelial location and have a disorganized appearance. The glomerular basement membrane (indicated by arrow) itself shows no collagen fibrils (uranyl acetate and lead citrate; original magnification ×17 000); (c) collagen fibrils have typical curvilinear and disorganized morphology when transversely cut indicative of atypical Type III collagen (uranyl acetate and lead citrate; original magnification ×21 500); (d) On high magnification, the organized deposits have banded appearance (indicated by arrow) with typical periodicity of 60 nm, indicative of fibrillary Type III collagen (uranyl acetate and lead citrate; original magnification ×28 000).

Mentions: Histopathologically, light microscopy shows lobular bland-appearing glomeruli due to global expansion of the mesangium with thickening of the peripheral capillary walls but no substantial mesangial hypercellularity (Figure 1a and b). The mesangial expansion is due to the accumulation of amorphous, weakly periodic acid Schiff (PAS)-positive material mimicking amyloid deposits. However, Congo red and thioflavine stains are completely negative. On Masson’s trichrome stain, the deposited material reveals blue staining (Figure 2d). The capillary lumina are narrowed but not occluded. The thickened capillary walls show focal reduplication; however, PAS and methenamine silver stains clearly highlight that the capillary basement membranes are normal and thickening of the wall is due to sub-endothelial deposition of pale amorphous material (Figure 1c). Usually, no endocapillary or extracapillary proliferation is seen in CG. In the advanced stage, capillary lumens are narrowed by the expanded mesangium and thickened capillary walls and glomeruli show a nodular appearance suggestive of diabetic nephropathy or monoclonal immunoglobulin deposition disease. However, unlike these two entities, the nodular lesions are weakly PAS positive or PAS negative in CG. Patchy tubular atrophy and interstitial fibrosis may be present, and these changes are proportional to the degree of global glomerulosclerosis. Arteriolar hyalinosis and thickening of the walls of arteries can be seen, probably secondary to hypertension.


Collagenofibrotic glomerulopathy-a review.

Duggal R, Nada R, Rayat CS, Rane SU, Sakhuja V, Joshi K - Clin Kidney J (2012)

Electron microscopic features of collagenofibrotic glomerulopathy. (a) Expanded subendothelial and mesangial space by deposition of fibrillary material. The overlying podocytes show foot process effacement (uranyl acetate and lead citrate; original magnification ×10 000); (b) the collagen deposits are noted in the sub-endothelial location and have a disorganized appearance. The glomerular basement membrane (indicated by arrow) itself shows no collagen fibrils (uranyl acetate and lead citrate; original magnification ×17 000); (c) collagen fibrils have typical curvilinear and disorganized morphology when transversely cut indicative of atypical Type III collagen (uranyl acetate and lead citrate; original magnification ×21 500); (d) On high magnification, the organized deposits have banded appearance (indicated by arrow) with typical periodicity of 60 nm, indicative of fibrillary Type III collagen (uranyl acetate and lead citrate; original magnification ×28 000).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4400455&req=5

fig2: Electron microscopic features of collagenofibrotic glomerulopathy. (a) Expanded subendothelial and mesangial space by deposition of fibrillary material. The overlying podocytes show foot process effacement (uranyl acetate and lead citrate; original magnification ×10 000); (b) the collagen deposits are noted in the sub-endothelial location and have a disorganized appearance. The glomerular basement membrane (indicated by arrow) itself shows no collagen fibrils (uranyl acetate and lead citrate; original magnification ×17 000); (c) collagen fibrils have typical curvilinear and disorganized morphology when transversely cut indicative of atypical Type III collagen (uranyl acetate and lead citrate; original magnification ×21 500); (d) On high magnification, the organized deposits have banded appearance (indicated by arrow) with typical periodicity of 60 nm, indicative of fibrillary Type III collagen (uranyl acetate and lead citrate; original magnification ×28 000).
Mentions: Histopathologically, light microscopy shows lobular bland-appearing glomeruli due to global expansion of the mesangium with thickening of the peripheral capillary walls but no substantial mesangial hypercellularity (Figure 1a and b). The mesangial expansion is due to the accumulation of amorphous, weakly periodic acid Schiff (PAS)-positive material mimicking amyloid deposits. However, Congo red and thioflavine stains are completely negative. On Masson’s trichrome stain, the deposited material reveals blue staining (Figure 2d). The capillary lumina are narrowed but not occluded. The thickened capillary walls show focal reduplication; however, PAS and methenamine silver stains clearly highlight that the capillary basement membranes are normal and thickening of the wall is due to sub-endothelial deposition of pale amorphous material (Figure 1c). Usually, no endocapillary or extracapillary proliferation is seen in CG. In the advanced stage, capillary lumens are narrowed by the expanded mesangium and thickened capillary walls and glomeruli show a nodular appearance suggestive of diabetic nephropathy or monoclonal immunoglobulin deposition disease. However, unlike these two entities, the nodular lesions are weakly PAS positive or PAS negative in CG. Patchy tubular atrophy and interstitial fibrosis may be present, and these changes are proportional to the degree of global glomerulosclerosis. Arteriolar hyalinosis and thickening of the walls of arteries can be seen, probably secondary to hypertension.

Bottom Line: A search of the English language literature identified 38 cases of CG, of which 22 are reported from Asian countries.An additional three cases are being reported from this Institute in India and are illustrated herein.These reports contribute to a better understanding of this disease, which although not as prevalent, should be considered as a differential diagnosis in cases of mesangiocapillary form of glomerular injury.

View Article: PubMed Central - PubMed

Affiliation: Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

ABSTRACT
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. A definite diagnosis can be established when typical histological findings are supported by electron microscopy. This disease exhibits indolent progression and as yet has no specific treatment. The present article reviews the clinicopathological features, epidemiology and proposed mechanisms of pathogenesis of CG. A search of the English language literature identified 38 cases of CG, of which 22 are reported from Asian countries. An additional three cases are being reported from this Institute in India and are illustrated herein. These reports contribute to a better understanding of this disease, which although not as prevalent, should be considered as a differential diagnosis in cases of mesangiocapillary form of glomerular injury.

No MeSH data available.


Related in: MedlinePlus