Limits...
C3 glomerulopathy: consensus report.

Pickering MC, D'Agati VD, Nester CM, Smith RJ, Haas M, Appel GB, Alpers CE, Bajema IM, Bedrosian C, Braun M, Doyle M, Fakhouri F, Fervenza FC, Fogo AB, Frémeaux-Bacchi V, Gale DP, Goicoechea de Jorge E, Griffin G, Harris CL, Holers VM, Johnson S, Lavin PJ, Medjeral-Thomas N, Paul Morgan B, Nast CC, Noel LH, Peters DK, Rodríguez de Córdoba S, Servais A, Sethi S, Song WC, Tamburini P, Thurman JM, Zavros M, Cook HT - Kidney Int. (2013)

Bottom Line: C3 glomerulopathy is a recently introduced pathological entity whose original definition was glomerular pathology characterized by C3 accumulation with absent or scanty immunoglobulin deposition.In August 2012, an invited group of experts (comprising the authors of this document) in renal pathology, nephrology, complement biology, and complement therapeutics met to discuss C3 glomerulopathy in the first C3 Glomerulopathy Meeting.This meeting report represents the current consensus view of the group.

View Article: PubMed Central - PubMed

Affiliation: Centre for Complement and Inflammation Research, Imperial College London, London, UK.

ABSTRACT
C3 glomerulopathy is a recently introduced pathological entity whose original definition was glomerular pathology characterized by C3 accumulation with absent or scanty immunoglobulin deposition. In August 2012, an invited group of experts (comprising the authors of this document) in renal pathology, nephrology, complement biology, and complement therapeutics met to discuss C3 glomerulopathy in the first C3 Glomerulopathy Meeting. The objectives were to reach a consensus on: the definition of C3 glomerulopathy, appropriate complement investigations that should be performed in these patients, and how complement therapeutics should be explored in the condition. This meeting report represents the current consensus view of the group.

Show MeSH

Related in: MedlinePlus

Electron microscopy in C3 glomerulopathy. (a) Dense deposit disease showing very electron-dense osmiophilic deposit occupying most of the glomerular basement membrane. (b) A case of dense deposit disease in which the highly osmiophilic deposit is only seen in segments of the glomerular basement membrane. (c) A case of C3 glomerulopathy in which there is electron-dense material that is expanding the basement membrane. The material is less electron dense and less well defined than in dense deposit disease. (d) A case of C3 glomerulopathy showing two large subepithelial hump-shaped deposits.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3842953&req=5

fig2: Electron microscopy in C3 glomerulopathy. (a) Dense deposit disease showing very electron-dense osmiophilic deposit occupying most of the glomerular basement membrane. (b) A case of dense deposit disease in which the highly osmiophilic deposit is only seen in segments of the glomerular basement membrane. (c) A case of C3 glomerulopathy in which there is electron-dense material that is expanding the basement membrane. The material is less electron dense and less well defined than in dense deposit disease. (d) A case of C3 glomerulopathy showing two large subepithelial hump-shaped deposits.

Mentions: C3 glomerulopathy may show a range of features on light microscopy and EM. Light microscopic appearances include mesangial proliferative, membranoproliferative, and endocapillary proliferative; in each case, crescents may also be present. In rare cases, glomeruli may be normal by light microscopy. One of the most variable findings between cases is in the quality of the deposits seen in glomeruli on EM. In many cases, these have a distinctive highly electron-dense, osmiophilic appearance and this has been designated as dense deposit disease (Figure 2a). It is not known why the deposits develop this particular morphological appearance. In other cases, the deposits do not have this characteristic density. However, the meeting recognized that, although in typical cases there is generally good agreement among pathologists on a diagnosis of dense deposit disease, there may be cases where the decision to call the deposit ‘dense' is not clear cut. In addition, the typical dense appearance may only be present in some segments of glomeruli (Figure 2b) and therefore diagnosis may be affected by EM sampling. In dense deposit disease, the deposits are typically found within the glomerular basement membrane, as rounded deposits in the mesangium, and, in many cases, in Bowman's capsule and tubular basement membranes. The glomerular deposits often form band-like or sausage-like shapes punctuated by skip areas of more normal-appearing glomerular basement membrane. These deposits tend to thicken and transform the lamina densa, but may also involve the subendothelial region and produce hump-shaped subepithelial deposits that resemble those seen in acute PIGN. Other than the difference in the morphology of the deposits on EM, which when well established may also lead to characteristic appearances on light microscopy, there are no other specific histopathological or clinical features that allow a distinction between cases of C3 glomerulopathy with the appearance of dense deposit disease and those without. By light microscopy, cases of dense deposit disease may show a range of appearances. In some, there is a typical membranoproliferative pattern, whereas others show predominantly mesangial proliferation. There may be prominent endocapillary proliferation, leukocyte infiltration, and/or crescents.5 Glomerular and tubular basement membrane deposits are often visible by light microscopy with the help of trichrome and silver stains.


C3 glomerulopathy: consensus report.

Pickering MC, D'Agati VD, Nester CM, Smith RJ, Haas M, Appel GB, Alpers CE, Bajema IM, Bedrosian C, Braun M, Doyle M, Fakhouri F, Fervenza FC, Fogo AB, Frémeaux-Bacchi V, Gale DP, Goicoechea de Jorge E, Griffin G, Harris CL, Holers VM, Johnson S, Lavin PJ, Medjeral-Thomas N, Paul Morgan B, Nast CC, Noel LH, Peters DK, Rodríguez de Córdoba S, Servais A, Sethi S, Song WC, Tamburini P, Thurman JM, Zavros M, Cook HT - Kidney Int. (2013)

Electron microscopy in C3 glomerulopathy. (a) Dense deposit disease showing very electron-dense osmiophilic deposit occupying most of the glomerular basement membrane. (b) A case of dense deposit disease in which the highly osmiophilic deposit is only seen in segments of the glomerular basement membrane. (c) A case of C3 glomerulopathy in which there is electron-dense material that is expanding the basement membrane. The material is less electron dense and less well defined than in dense deposit disease. (d) A case of C3 glomerulopathy showing two large subepithelial hump-shaped deposits.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3842953&req=5

fig2: Electron microscopy in C3 glomerulopathy. (a) Dense deposit disease showing very electron-dense osmiophilic deposit occupying most of the glomerular basement membrane. (b) A case of dense deposit disease in which the highly osmiophilic deposit is only seen in segments of the glomerular basement membrane. (c) A case of C3 glomerulopathy in which there is electron-dense material that is expanding the basement membrane. The material is less electron dense and less well defined than in dense deposit disease. (d) A case of C3 glomerulopathy showing two large subepithelial hump-shaped deposits.
Mentions: C3 glomerulopathy may show a range of features on light microscopy and EM. Light microscopic appearances include mesangial proliferative, membranoproliferative, and endocapillary proliferative; in each case, crescents may also be present. In rare cases, glomeruli may be normal by light microscopy. One of the most variable findings between cases is in the quality of the deposits seen in glomeruli on EM. In many cases, these have a distinctive highly electron-dense, osmiophilic appearance and this has been designated as dense deposit disease (Figure 2a). It is not known why the deposits develop this particular morphological appearance. In other cases, the deposits do not have this characteristic density. However, the meeting recognized that, although in typical cases there is generally good agreement among pathologists on a diagnosis of dense deposit disease, there may be cases where the decision to call the deposit ‘dense' is not clear cut. In addition, the typical dense appearance may only be present in some segments of glomeruli (Figure 2b) and therefore diagnosis may be affected by EM sampling. In dense deposit disease, the deposits are typically found within the glomerular basement membrane, as rounded deposits in the mesangium, and, in many cases, in Bowman's capsule and tubular basement membranes. The glomerular deposits often form band-like or sausage-like shapes punctuated by skip areas of more normal-appearing glomerular basement membrane. These deposits tend to thicken and transform the lamina densa, but may also involve the subendothelial region and produce hump-shaped subepithelial deposits that resemble those seen in acute PIGN. Other than the difference in the morphology of the deposits on EM, which when well established may also lead to characteristic appearances on light microscopy, there are no other specific histopathological or clinical features that allow a distinction between cases of C3 glomerulopathy with the appearance of dense deposit disease and those without. By light microscopy, cases of dense deposit disease may show a range of appearances. In some, there is a typical membranoproliferative pattern, whereas others show predominantly mesangial proliferation. There may be prominent endocapillary proliferation, leukocyte infiltration, and/or crescents.5 Glomerular and tubular basement membrane deposits are often visible by light microscopy with the help of trichrome and silver stains.

Bottom Line: C3 glomerulopathy is a recently introduced pathological entity whose original definition was glomerular pathology characterized by C3 accumulation with absent or scanty immunoglobulin deposition.In August 2012, an invited group of experts (comprising the authors of this document) in renal pathology, nephrology, complement biology, and complement therapeutics met to discuss C3 glomerulopathy in the first C3 Glomerulopathy Meeting.This meeting report represents the current consensus view of the group.

View Article: PubMed Central - PubMed

Affiliation: Centre for Complement and Inflammation Research, Imperial College London, London, UK.

ABSTRACT
C3 glomerulopathy is a recently introduced pathological entity whose original definition was glomerular pathology characterized by C3 accumulation with absent or scanty immunoglobulin deposition. In August 2012, an invited group of experts (comprising the authors of this document) in renal pathology, nephrology, complement biology, and complement therapeutics met to discuss C3 glomerulopathy in the first C3 Glomerulopathy Meeting. The objectives were to reach a consensus on: the definition of C3 glomerulopathy, appropriate complement investigations that should be performed in these patients, and how complement therapeutics should be explored in the condition. This meeting report represents the current consensus view of the group.

Show MeSH
Related in: MedlinePlus