Lung - nontumor
Other nonneoplastic disease
Goodpasture syndrome

Author: Elliot Weisenberg, M.D. (see Authors page)

Revised: 9 March 2017, last major update October 2011

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed search: Goodpasture syndrome [title]

Cite this page: Goodpasture syndrome. PathologyOutlines.com website. http://pathologyoutlines.com/topic/lungnontumorgoodpasture.html. Accessed December 13th, 2017.
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