Goodpasture syndrome (or antiglomerular basement membrane (anti-GBM) antibody disease) is an autoimmune disease characterised by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. It is type of pulmonary renal syndrome.
Goodpasture syndrome is defined by:
It typically affects young adult men with a M:F of 3:1.
Patients may present with cough, dyspnoea, haemoptysis and hypoxia. Pulmonary manisfestations are usually the cause of presentation although most also have evidence of renal disease.
Type II hypersensitivity reaction with antibodies directed primarily against renal glomerular basement membrane collagen type IV and cross-reacts with alveolar basement membrane to produce the renal injury, usually rapidly progressive glomerulonephritis and necrotising hemorrhagic interstitial pneumonitis characteristic of this disorder.
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 changes on chest radiographs
- bilateral, coalescent airspace opacities, which in several days resolve to give reticular opacities in the same distribution
- complete radiographic resolution 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 acute phase
Treatment and prognosis
The overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.
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