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
  • Smoking
  • 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.

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