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Goodpasture Syndrome

  • Goodpasture Syndrome is an autoimmune disease with a specific etiopathogenesis and is characterized by both pulmonary and renal pathology.
Etiology and Pathogenesis
  • Goodpasture Syndrome is fundamentally initiated by the development of auto-antibodies to pulmonary and renal basement membrane antigens. Consequently, goodpasture syndrome can be considered a Type II Hypersensitivity. Binding of auto-antibodies results in activation of complement, yielding inflammation of the lung and kidneys, specifically of the alveoli and glomeruli. Inflammation ultimately compromises these barriers, leading to a combined pulmonary-renal syndrome described below.
  • Lung
    • Scattered necrosis and hemorrhage into alveoli along with inflammation and thickening of the pulmonary interstitium.
  • Kidney
    • Light Microscopy: Overt disease results in Rapidly Progressive Glomerulonephritis (See Page). Immunofluorescence shows a linear, ribbon-like deposit of IgG along the length of the glomerular basement membrane.
Clinical Consequences
  • Pulmonary Symptomology
    • Mild to potentially major hemorrhage into the lung that frequently manifests as hemoptysis. Hemorrhage combined with the interstitial inflammation (i.e. pneumonitis) and interstitial thickening can lead to a restrictive pattern of pulmonary function.
  • Renal Symptomology
    • Inflammation of the glomerulus initiates the pathogenesis of a nephritic syndrome with symptomology of hematuria, pyuria, secondary hypertension, oliguria, and azotemia (See nephritic syndrome page).
  • The incidence of Goodpasture syndrome shows a bimodal distribution with one peak among young men in their late-twenties and another peak among elderly men and women after their sixtieth year of age. Goodpasture syndrome displays an increased risk in those with possession of HLA-DR2 allele.