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Collagenofibrotic glomerulopathy.

Kurien AA, Larsen CP, Cossey LN - Clin Kidney J (2015)

Bottom Line: All immunofluorescence studies were negative.All patients appear to have sporadic disease occurrence with no family history of renal disease.No hemolytic uremic syndrome, liver fibrosis, lymphoma or co-occurrence of other renal disease were seen.

View Article: PubMed Central - PubMed

Affiliation: Center for Renal and Urological Pathology , Chennai, Tamil Nadu , India.

ABSTRACT

Background: Collagenofibrotic glomerulopathy is a rare renal disease of unknown etiology that is secondary to deposition of type III collagen within the glomerulus. Only rare case series exist in the literature.

Methods: Renal biopsies diagnosed with collagenofibrotic glomerulopathy were prospectively collected at the Center for Renal and Urological Pathology (AAK) (Chennai, Tamil Nadu, India) from 2012 to 2015. Eight patients were entered into the study. The average age was 38 years with five males and three females.

Results: All patients presented with nephrotic syndrome, and five displayed hypertension. The average serum creatinine was 146.5 µmol/L (88.4-282.9 µmol/L range). All serologic testing was negative, and complement levels were normal. No clinical evidence of nail-patella syndrome was seen. All cases showed diffuse mesangial expansion and double contour formation by peroidic acid-Schiff (PAS)-negative material. All immunofluorescence studies were negative. By electron microscopy all cases showed electron dense, banded to curvilinear collagen bundles within the mesangium and subendothelial aspect of the peripheral capillary walls. All patients appear to have sporadic disease occurrence with no family history of renal disease. No hemolytic uremic syndrome, liver fibrosis, lymphoma or co-occurrence of other renal disease were seen.

Conclusion: Collagenofibrotic glomerulopathy is a rare disease that appears to occur more frequently in adult Indian populations in a sporadic, non-familial manner. To our knowledge, this is the largest cases series of collagenofibrotic glomerulopathy in an adult population.

No MeSH data available.


Related in: MedlinePlus

Morphology of collagenofibrotic glomerulopathy. (A) Global mesangial expansion and focal double contour formation (H&E stain, ×400 original magnification). (B) Mesangial expansion and peripheral capillary loop double contour formation staining a variegated blue on Masson trichrome stain (×200 original magnification). (C) Jones silver stain showing loss of silver staining within the expanded mesangium and peripheral capillary loop double contours (×400 original magnification). (D) Type III collagen immunohistochemical stain showing staining of the peripheral capillary loops and mesangium (×600 original magnification).
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SFV061F1: Morphology of collagenofibrotic glomerulopathy. (A) Global mesangial expansion and focal double contour formation (H&E stain, ×400 original magnification). (B) Mesangial expansion and peripheral capillary loop double contour formation staining a variegated blue on Masson trichrome stain (×200 original magnification). (C) Jones silver stain showing loss of silver staining within the expanded mesangium and peripheral capillary loop double contours (×400 original magnification). (D) Type III collagen immunohistochemical stain showing staining of the peripheral capillary loops and mesangium (×600 original magnification).

Mentions: All renal biopsies showed adequate tissue by light microscopy, immunofluorescence and electron microscopy. An average of 24 (14–32 range) glomeruli were present for evaluation by light microscopy with an average of 30% global glomerulosclerosis (13–73% range) (see Table 2). Double contour formation was seen in all biopsies. Diffuse mesangial expansion by PAS-negative, flocculent material was seen in all biopsies (Figure 1A–C). This material was also frequently seen on the luminal side of the peripheral capillary loops and between double contours. No extension into the extraglomerular arterioles/arteries, Bowman space, podocytes or the tubulointerstitium was present. Staining for Congo red was negative in all biopsies. Staining for collagen type III was performed on three biopsies where tissue was available and showed positive staining within the mesangium and capillary walls in all (Figure 1D). By immunofluorescence, staining for IgA, IgG, IgM, C3, C1q, kappa and lambda light chains was negative in intact glomeruli in all biopsies. By electron microscopy all cases showed flocculent expansion of the mesangium and subendothelial aspect of the peripheral capillary wall with deposition of electron-dense, frayed, banded to curvilinear fibrillar material consistent with collagen bundles (Figure 2A–D). No collagen deposition was present within the intramembranous or subepithelial compartments of the peripheral capillary walls.Table 2.


Collagenofibrotic glomerulopathy.

Kurien AA, Larsen CP, Cossey LN - Clin Kidney J (2015)

Morphology of collagenofibrotic glomerulopathy. (A) Global mesangial expansion and focal double contour formation (H&E stain, ×400 original magnification). (B) Mesangial expansion and peripheral capillary loop double contour formation staining a variegated blue on Masson trichrome stain (×200 original magnification). (C) Jones silver stain showing loss of silver staining within the expanded mesangium and peripheral capillary loop double contours (×400 original magnification). (D) Type III collagen immunohistochemical stain showing staining of the peripheral capillary loops and mesangium (×600 original magnification).
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4581380&req=5

SFV061F1: Morphology of collagenofibrotic glomerulopathy. (A) Global mesangial expansion and focal double contour formation (H&E stain, ×400 original magnification). (B) Mesangial expansion and peripheral capillary loop double contour formation staining a variegated blue on Masson trichrome stain (×200 original magnification). (C) Jones silver stain showing loss of silver staining within the expanded mesangium and peripheral capillary loop double contours (×400 original magnification). (D) Type III collagen immunohistochemical stain showing staining of the peripheral capillary loops and mesangium (×600 original magnification).
Mentions: All renal biopsies showed adequate tissue by light microscopy, immunofluorescence and electron microscopy. An average of 24 (14–32 range) glomeruli were present for evaluation by light microscopy with an average of 30% global glomerulosclerosis (13–73% range) (see Table 2). Double contour formation was seen in all biopsies. Diffuse mesangial expansion by PAS-negative, flocculent material was seen in all biopsies (Figure 1A–C). This material was also frequently seen on the luminal side of the peripheral capillary loops and between double contours. No extension into the extraglomerular arterioles/arteries, Bowman space, podocytes or the tubulointerstitium was present. Staining for Congo red was negative in all biopsies. Staining for collagen type III was performed on three biopsies where tissue was available and showed positive staining within the mesangium and capillary walls in all (Figure 1D). By immunofluorescence, staining for IgA, IgG, IgM, C3, C1q, kappa and lambda light chains was negative in intact glomeruli in all biopsies. By electron microscopy all cases showed flocculent expansion of the mesangium and subendothelial aspect of the peripheral capillary wall with deposition of electron-dense, frayed, banded to curvilinear fibrillar material consistent with collagen bundles (Figure 2A–D). No collagen deposition was present within the intramembranous or subepithelial compartments of the peripheral capillary walls.Table 2.

Bottom Line: All immunofluorescence studies were negative.All patients appear to have sporadic disease occurrence with no family history of renal disease.No hemolytic uremic syndrome, liver fibrosis, lymphoma or co-occurrence of other renal disease were seen.

View Article: PubMed Central - PubMed

Affiliation: Center for Renal and Urological Pathology , Chennai, Tamil Nadu , India.

ABSTRACT

Background: Collagenofibrotic glomerulopathy is a rare renal disease of unknown etiology that is secondary to deposition of type III collagen within the glomerulus. Only rare case series exist in the literature.

Methods: Renal biopsies diagnosed with collagenofibrotic glomerulopathy were prospectively collected at the Center for Renal and Urological Pathology (AAK) (Chennai, Tamil Nadu, India) from 2012 to 2015. Eight patients were entered into the study. The average age was 38 years with five males and three females.

Results: All patients presented with nephrotic syndrome, and five displayed hypertension. The average serum creatinine was 146.5 µmol/L (88.4-282.9 µmol/L range). All serologic testing was negative, and complement levels were normal. No clinical evidence of nail-patella syndrome was seen. All cases showed diffuse mesangial expansion and double contour formation by peroidic acid-Schiff (PAS)-negative material. All immunofluorescence studies were negative. By electron microscopy all cases showed electron dense, banded to curvilinear collagen bundles within the mesangium and subendothelial aspect of the peripheral capillary walls. All patients appear to have sporadic disease occurrence with no family history of renal disease. No hemolytic uremic syndrome, liver fibrosis, lymphoma or co-occurrence of other renal disease were seen.

Conclusion: Collagenofibrotic glomerulopathy is a rare disease that appears to occur more frequently in adult Indian populations in a sporadic, non-familial manner. To our knowledge, this is the largest cases series of collagenofibrotic glomerulopathy in an adult population.

No MeSH data available.


Related in: MedlinePlus