Lung
Other nonneoplastic disease
Goodpasture syndrome
Author: Elliot Weisenberg, M.D.
Last author update: 1 October 2011
Last staff update: 24 August 2020
Copyright: 2003-2024, PathologyOutlines.com, Inc.
PubMed search: Goodpasture syndrome [title]
Table of Contents
Definition / general | Treatment | Microscopic (histologic) description | Differential diagnosisCite this page: Weisenberg E. Goodpasture syndrome. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungnontumorgoodpasture.html. Accessed December 25th, 2024.
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
