Immunopathological studies of an autopsy case with Goodpasture's syndrome and systemic necrotizing angiitis

A rare autopsy case of Goodpasture's syndrome with systemic necrotizing angiitis is reported. The patient, a 56-year-old male, died of respiratory failure with massive pulmonary hemorrhage and renal failure. The autopsy showed widespread hemorrhage in both lungs, diffuse crescentic glomerulonephritis, and systemic necrotizing angiitis in the small arteries. Immunofluorescence studies demonstrated a linear deposition of IgG along the glomerular basement membrane (GBM) as well as the alveolar basement membrane. A granular deposition of C3 was also found along the GBM. Electron microscopy showed that the GBM was irregularly thickened and wrinkled, but electron-dense deposits were indistinct. Anti-GBM antibody activity was detected in the patient's serum and had cross reactivity with normal alveolar basement membrane. The renal eluates contained IgG antibody activity for normal human GBM. These results suggest that glomerulonephritis and pulmonary hemorrhage in the present case were mediated by anti-basement membrane antibodies. We also discussed whether anti-basement membrane antibody is involved in the pathogenesis of systemic necrotizing angiitis.