Orphanet: Anti glomerular basement membrane disease

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Anti-glomerular basement membrane disease

Disease definition

A rare, fulminant small vessel vasculitis that affects the capillary beds of the kidneys and lungs and characterized by the presence of anti-glomerular basement membrane (GBM) and, in its full-blown form, anti-alveolar basement membrane (ABM) antibodies. Consequently, it may manifest as a rapidly progressive, isolated glomerulonephritis (anti-GBM nephritis) or as a pulmonary-renal syndrome with severe lung hemorrhage.


Classification level: Disorder
  • Synonym(s):
    • Anti-GBM syndrome
    • Goodpasture syndrome
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Elderly, Adolescent, Adult
  • ICD-10: M31.0+  N08.5*
  • ICD-11: MF85
  • OMIM: 233450
  • UMLS: C0403529
  • MeSH: -
  • GARD: 2551
  • MedDRA: 10018620

Detailed information

General public


ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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