Goodpasture syndrome

Last revised by Liz Silverstone on 4 Nov 2023

Goodpasture syndrome, also referred as antiglomerular basement membrane (anti-GBM) antibody disease, is an autoimmune disease characterized by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. It is a type of pulmonary-renal syndrome.

Goodpasture syndrome is defined by:

Goodpasture’s syndrome accounts for 1-2% of cases of rapidly progressive glomerulonephritis. It can occur at any age but most commonly affects men aged 20 - 30 with an M:F of 3:1. Smoking commonly predates pulmonary hemorrhage. Other risk factors may include hydrocarbon exposure, infections such as influenza and HLA type.

Patients may present with cough, dyspnea, hemoptysis, hypoxemia, anemia, fatigue, chest pain, uremia or hematuria. Hypertension is rare.

Goodpasture syndrome is a type II hypersensitivity reaction with antibodies primarily directed against type IV collagen of the renal glomerular basement membrane. There is further cross-reactivity with the alveolar basement membrane resulting in pulmonary injury. This is typically characterized by rapidly progressive glomerulonephritis (RPGN) and necrotizing hemorrhagic interstitial pneumonitis respectively.

The diagnosis is made by immunofluorescent studies of renal or lung tissue, which show a smooth wavy line of fluorescent staining along the basement membrane. 

  • non-specific, bilateral, coalescent airspace opacities, which resolve in several days to give reticular opacities in the same distribution

  • complete radiographic resolution is usually seen within 2-3 weeks

  • ground glass and airspace opacities that progress to reticular "crazy paving" pattern over a few weeks

  • hilar lymphadenopathy may be seen

  • no interlobular septal thickening in the acute phase

The overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.

Often radiograph and CT findings are indistinguishable from pulmonary edema. Other causes of pulmonary hemorrhage should also be considered. 

ADVERTISEMENT: Supporters see fewer/no ads

Cases and figures

  • Case 1
    Drag here to reorder.
  • Case 2
    Drag here to reorder.
  • Case 4
    Drag here to reorder.
  • Case 3: isolated pulmonary involvement
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.