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:
circulating antiglomerular basement membrane antibodies
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Epidemiology
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.
Clinical presentation
Patients may present with cough, dyspnea, hemoptysis, hypoxemia, anemia, fatigue, chest pain, uremia or hematuria. Hypertension is rare.
Pathology
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.
Radiographic features
Plain radiograph
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
CT
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
Treatment and prognosis
The overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.
Differential diagnosis
Often radiograph and CT findings are indistinguishable from pulmonary edema. Other causes of pulmonary hemorrhage should also be considered.
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