Change search
ReferencesLink to record
Permanent link

Direct link
Diagnosis and classification of Goodpastures disease (anti-GBM)
Lund University, Sweden .
Linköping University, Department of Medical and Health Sciences, Division of Drug Research. Linköping University, Faculty of Health Sciences. Östergötlands Läns Landsting, Heart and Medicine Center, Department of Nephrology.
2014 (English)In: Journal of Autoimmunity, ISSN 0896-8411, E-ISSN 1095-9157, Vol. 48-49, 108-112 p.Article in journal (Refereed) Published
Abstract [en]

Goodpastures 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 auto-antibodies. The disease is a prototype of autoimmune disease, where the patients develop auto-antibodies 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 Goodpastures syndrome or Goodpastures 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.

Place, publisher, year, edition, pages
Elsevier , 2014. Vol. 48-49, 108-112 p.
Keyword [en]
Anti-GBM disease; Goodpastures disease; Autoantibodies; Glomerulonephritis; Vasculitis
National Category
Medical and Health Sciences
URN: urn:nbn:se:liu:diva-106524DOI: 10.1016/j.jaut.2014.01.024ISI: 000334009300023OAI: diva2:716616
Available from: 2014-05-12 Created: 2014-05-09 Last updated: 2015-06-29

Open Access in DiVA

fulltext(925 kB)827 downloads
File information
File name FULLTEXT01.pdfFile size 925 kBChecksum SHA-512
Type fulltextMimetype application/pdf

Other links

Publisher's full text

Search in DiVA

By author/editor
Segelmark, Mårten
By organisation
Division of Drug ResearchFaculty of Health SciencesDepartment of Nephrology
In the same journal
Journal of Autoimmunity
Medical and Health Sciences

Search outside of DiVA

GoogleGoogle Scholar
Total: 827 downloads
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

Altmetric score

Total: 145 hits
ReferencesLink to record
Permanent link

Direct link