Anti-glomerular basement membrane disease
ORPHA:375· ICD-10 M31.0+
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.
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly