Goodpasture Syndrome
Epidemiology:
- Teens, twenties
mostly
- M >F
Common sites:
Gross features:
- heavy
- areas of red-brown
consolidation
Histologic features:
- focal necrosis of
alveolar walls
- alveolar
hemorrhages (may be seen in biopsy-related bleeding)
- hemosiderin-laden macrophages (not seen in
biopsy-related bleeding)
- mild hyperplasia
of alveolar lining cells of alveolar septa
- focal acute capillaritis
- sparse lymphocytic infiltrate sometimes
- organization of
alveolar blood
- precipitation of
iron in the vessles with giant cell reaction
may be seen
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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- Linear
pattern of IgG and C3 staining in basement
membrane of alveolus and glomerulus
Molecular features:
Other features:
- autoantibodies against the noncollagenous
domain of the alpha-3 chain of collagen IV (in basement membrane)