Lung

Other nonneoplastic disease

Goodpasture syndrome



Topic Completed: 1 October 2011

Minor changes: 24 August 2020

Copyright: 2003-2021, PathologyOutlines.com, Inc.

PubMed search: Goodpasture syndrome [title]

Elliot Weisenberg, M.D.
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Cite this page: Weisenberg E. Goodpasture syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumorgoodpasture.html. Accessed October 16th, 2021.
Definition / general
  • Autoimmune disease affecting men > women, usually ages 15 - 29 years, with simultaneous sometimes massive hemorrhagic interstitial pneumonitis and rapidly progressive (crescentic) glomerulonephritis
  • Sometimes preceded by chemical or drug exposure, viral infection or malignancy
  • Genetic predisposition, higher incidence with some HLA subtypes
  • Death common due to uremia
  • Due to IgG antibodies to basement membrane of alveoli and glomeruli
Treatment
  • Plasma exchange (removes antibodies and chemical mediators)
  • Immunosuppressive therapy (prevents further antibody production)
Microscopic (histologic) description
  • Lungs: heavy, focal necrosis of alveolar wall, fibrous thickening of septa with mild hyperplasia of alveolar lining cells, organization of blood in alveolar space and hemosiderin laden macrophages; linear deposits of immunoglobulin along basement membrane

  • Kidney: focal proliferative to crescentic glomerulonephritis with linear deposits of immunoglobulin and complement along basement membrane, similar to lung
Differential diagnosis
  • Mitral stenosis, periarteritis nodosa, SLE and systemic vasculitis all cause secondary pulmonary alveolar bleeding and hemosiderosis
  • Immune complex glomerulonephritis: granular pattern of immunofluorescence
  • Idiopathic pulmonary hemosiderosis: no antibodies
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