Diagnosis and classification of Goodpasture's disease (anti-GBM).
Hellmark, Thomas LU
and Segelmark, Mårten
LU
(2014)
In Journal of Autoimmunity
48-49(Jan 20).
p.108-112
- Abstract
- Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies that bind to the basement membranes and activate the classical pathway of the complement system, which start a neutrophil dependent inflammation. The diagnosis of anti-GBM disease relies on the detection of anti-GBM antibodies in conjunction with glomerulonephritis and/or alveolitis. Overt clinical symptoms are most prominent in the glomeruli... (More)
- Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies that bind to the basement membranes and activate the classical pathway of the complement system, which start a neutrophil dependent inflammation. The diagnosis of anti-GBM disease relies on the detection of anti-GBM antibodies in conjunction with glomerulonephritis and/or alveolitis. Overt clinical symptoms are most prominent in the glomeruli where the inflammation usually results in a severe rapidly progressive glomerulonephritis. Despite modern treatment less than one third of the patients survive with a preserved kidney function after 6 months follow-up. Frequencies vary from 0.5 to 1 cases per million inhabitants per year and there is a strong genetic linkage to HLA-DRB1(∗)1501 and DRB1(∗)1502. Essentially, anti-GBM disease is now a preferred term for what was earlier called Goodpasture's syndrome or Goodpasture's disease; anti-GBM disease is now classified as small vessel vasculitis caused by in situ immune complex formation; the diagnosis relies on the detection of anti-GBM in tissues or circulation in conjunction with alveolar or glomerular disease; therapy is effective only when detected at an early stage, making a high degree of awareness necessary to find these rare cases; 20-35% have anti-GBM and MPO-ANCA simultaneously, which necessitates testing for anti-GBM whenever acute test for ANCA is ordered in patients with renal disease. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/4290772
- author
- Hellmark, Thomas
LU
and Segelmark, Mårten
LU
- organization
- publishing date
- 2014
- type
- Contribution to journal
- publication status
- published
- subject
- in
- Journal of Autoimmunity
- volume
- 48-49
- issue
- Jan 20
- pages
- 108 - 112
- publisher
- Elsevier
- external identifiers
-
- pmid:24456936
- wos:000334009300023
- scopus:84895875154
- ISSN
- 0896-8411
- DOI
- 10.1016/j.jaut.2014.01.024
- language
- English
- LU publication?
- yes
- id
- 3b20c270-152a-4aaf-8212-b670f3b8c22f (old id 4290772)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/24456936?dopt=Abstract
- date added to LUP
- 2016-04-01 10:13:52
- date last changed
- 2022-04-27 19:54:47
@article{3b20c270-152a-4aaf-8212-b670f3b8c22f,
abstract = {{Goodpasture's disease or anti-glomerular basement membrane disease (anti-GBM-disease) is included among immune complex small vessel vasculitides. The definition of anti-GBM disease is a vasculitis affecting glomerular capillaries, pulmonary capillaries, or both, with GBM deposition of anti-GBM autoantibodies. The disease is a prototype of autoimmune disease, where the patients develop autoantibodies that bind to the basement membranes and activate the classical pathway of the complement system, which start a neutrophil dependent inflammation. The diagnosis of anti-GBM disease relies on the detection of anti-GBM antibodies in conjunction with glomerulonephritis and/or alveolitis. Overt clinical symptoms are most prominent in the glomeruli where the inflammation usually results in a severe rapidly progressive glomerulonephritis. Despite modern treatment less than one third of the patients survive with a preserved kidney function after 6 months follow-up. Frequencies vary from 0.5 to 1 cases per million inhabitants per year and there is a strong genetic linkage to HLA-DRB1(∗)1501 and DRB1(∗)1502. Essentially, anti-GBM disease is now a preferred term for what was earlier called Goodpasture's syndrome or Goodpasture's disease; anti-GBM disease is now classified as small vessel vasculitis caused by in situ immune complex formation; the diagnosis relies on the detection of anti-GBM in tissues or circulation in conjunction with alveolar or glomerular disease; therapy is effective only when detected at an early stage, making a high degree of awareness necessary to find these rare cases; 20-35% have anti-GBM and MPO-ANCA simultaneously, which necessitates testing for anti-GBM whenever acute test for ANCA is ordered in patients with renal disease.}},
author = {{Hellmark, Thomas and Segelmark, Mårten}},
issn = {{0896-8411}},
language = {{eng}},
number = {{Jan 20}},
pages = {{108--112}},
publisher = {{Elsevier}},
series = {{Journal of Autoimmunity}},
title = {{Diagnosis and classification of Goodpasture's disease (anti-GBM).}},
url = {{http://dx.doi.org/10.1016/j.jaut.2014.01.024}},
doi = {{10.1016/j.jaut.2014.01.024}},
volume = {{48-49}},
year = {{2014}},
}