Lung-nontumor - Goodpasture's syndrome

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Lung-nontumor

Other non-neoplastic disease

Goodpasture's syndrome

Reviewers: Dr. Elliot Weisenberg, M.D. (see Reviewers page)
Revised: 9 November 2011, last major update October 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

General
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● 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
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● Plasma exchange (removes antibodies and chemical mediators)
● Immunosuppressive therapy (prevents further antibody production)

Micro description
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● 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
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● 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

End of Lung-nontumor > Other non-neoplastic disease > Goodpasture's syndrome

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