pulmonary disorders
Goodpasture Syndrome
Pathophysiology of Goodpasture Syndrome
Goodpasture syndrome is the combination of glomerulonephritis with alveolar hemorrhage and anti-GBM antibodies. Goodpasture syndrome most often manifests as Diffuse Alveolar Hemorrhage and Anti-GBM antibodies are directed against the noncollagenous (NC-1) domain of the alpha3 chain of type IV collagen, which occurs in highest concentration in the basement membranes of renal and pulmonary capillaries. Environmental exposures—cigarette smoking most commonly, and viral respiratory infection and hydrocarbon solvent inhalation less commonly—expose alveolar capillary antigens to circulating antibody in genetically susceptible people, most notably those with HLA-DRw15, -DR4, and -DRB1 alleles. Circulating anti-GBM antibodies bind to basement membranes, fix complement, and trigger a cell-mediated inflammatory response, causing glomerulonephritis, pulmonary capillaritis, or both. 