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Goodpasture's syndrome with concomitant immune complex mixed membranous and proliferative glomerulonephritis

Jurcic, Vesna; Vizjak, Alenka; Rigler, Andreja Ales; Jeruc, Jera; Wieslander, Jörgen LU and Ferluga, Dusan (2014) In Clinical Nephrology 81(3). p.216-223
Abstract
Classical Goodpasture's (GP) syndrome is a monophasic illness characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis with linear IgG deposition along the glomerular and distal tubular basement membrane and destructive necrotizing diffuse extracapillary crescentic glomerulonephritis. The majority of patients have circulating anti-glomerular basement membrane (GBM) antibodies, detectable with standard anti-GBM ELISA. Concurrence of GP syndrome with proliferative glomerulonephritis has only rarely been described. In this report, for the first time we describe in a 21-year-old woman GP syndrome with 50% crescentic sclerosing glomerulonephritis with linear immunofluorescence characteristic of anti-GBM pathogenesis,... (More)
Classical Goodpasture's (GP) syndrome is a monophasic illness characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis with linear IgG deposition along the glomerular and distal tubular basement membrane and destructive necrotizing diffuse extracapillary crescentic glomerulonephritis. The majority of patients have circulating anti-glomerular basement membrane (GBM) antibodies, detectable with standard anti-GBM ELISA. Concurrence of GP syndrome with proliferative glomerulonephritis has only rarely been described. In this report, for the first time we describe in a 21-year-old woman GP syndrome with 50% crescentic sclerosing glomerulonephritis with linear immunofluorescence characteristic of anti-GBM pathogenesis, combined with mixed membranous and membranoproliferative glomerulonephritis with granular immunofluorescence and subepithelial, mesangial and subendothelial deposits characterizing immune complex pathogenesis. The clinical picture was also unusual for GP syndrome, manifesting a recurrent but non-progressive course, nephrotic syndrome, normal renal function and low values of anti-GBM antibodies, identified only by novel more sensitive techniques. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Goodpasture's syndrome, anti-GBM, membranous glomerulonephritis, membranoproliferative glomerulonephritis
in
Clinical Nephrology
volume
81
issue
3
pages
216 - 223
publisher
Dustri-Verlag
external identifiers
  • wos:000344794600011
  • scopus:84894042225
ISSN
0301-0430
DOI
10.5414/CN107454
language
English
LU publication?
yes
id
dc206ed6-6048-4e4c-985e-b0ccb7090cb7 (old id 4871865)
date added to LUP
2015-01-07 10:46:22
date last changed
2017-01-01 05:51:40
@article{dc206ed6-6048-4e4c-985e-b0ccb7090cb7,
  abstract     = {Classical Goodpasture's (GP) syndrome is a monophasic illness characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis with linear IgG deposition along the glomerular and distal tubular basement membrane and destructive necrotizing diffuse extracapillary crescentic glomerulonephritis. The majority of patients have circulating anti-glomerular basement membrane (GBM) antibodies, detectable with standard anti-GBM ELISA. Concurrence of GP syndrome with proliferative glomerulonephritis has only rarely been described. In this report, for the first time we describe in a 21-year-old woman GP syndrome with 50% crescentic sclerosing glomerulonephritis with linear immunofluorescence characteristic of anti-GBM pathogenesis, combined with mixed membranous and membranoproliferative glomerulonephritis with granular immunofluorescence and subepithelial, mesangial and subendothelial deposits characterizing immune complex pathogenesis. The clinical picture was also unusual for GP syndrome, manifesting a recurrent but non-progressive course, nephrotic syndrome, normal renal function and low values of anti-GBM antibodies, identified only by novel more sensitive techniques.},
  author       = {Jurcic, Vesna and Vizjak, Alenka and Rigler, Andreja Ales and Jeruc, Jera and Wieslander, Jörgen and Ferluga, Dusan},
  issn         = {0301-0430},
  keyword      = {Goodpasture's syndrome,anti-GBM,membranous glomerulonephritis,membranoproliferative glomerulonephritis},
  language     = {eng},
  number       = {3},
  pages        = {216--223},
  publisher    = {Dustri-Verlag},
  series       = {Clinical Nephrology},
  title        = {Goodpasture's syndrome with concomitant immune complex mixed membranous and proliferative glomerulonephritis},
  url          = {http://dx.doi.org/10.5414/CN107454},
  volume       = {81},
  year         = {2014},
}