Goodpasture’s syndrome (anti-GBM antibody disease)
This is a rare, genetic, autoimmune condition characterised by a combination of glomerulonephritis with alveolar hemorrhage in the presence of circulating anti-glomerular basement membrane (GBM) antibodies. Circulating anti-GBM antibodies bind to basement membranes in the kidneys and lungs, fix complement and trigger a cell-mediated inflammatory response, causing glomerulonephritis and pulmonary capillaritis.
Specific predisposing factors include the following:
- Association with HLA-DR2
- Exposure to organic solvents or hydrocarbons
- Infection, e.g. influenza A2
- Cocaine inhalation
- Exposure to metal dusts
Diagnosis of Goodpasture’s requires presence of anti-GBM antibodies in serum, but confirming a diagnosis lies with performing a renal biopsy when glomerulonephritis is present.