What is Goodpasture syndrome (anti–glomerular basement membrane disease) (anti-GBM)?

Updated: Dec 16, 2020
  • Author: Pranay Kathuria, MD, FACP, FASN, FNKF; Chief Editor: Vecihi Batuman, MD, FASN  more...
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Answer

Goodpasture syndrome is an eponym that has been used to describe the clinical entity of diffuse pulmonary hemorrhage (as seen in the images below) and acute or rapidly progressive glomerulonephritis. Goodpasture disease is a term used to describe glomerulonephritis, with or without pulmonary hemorrhage, and the presence of circulating anti–glomerular basement membrane (anti-GBM) antibodies. The definitions of these terms have not been consistent, however. [1]  Anti-GBM disease, a more precise term, should be used to refer to either of the two distinct clinical manifestations of this disorder.

Goodpasture syndrome. A 45-year-old man was admitt Goodpasture syndrome. A 45-year-old man was admitted to the intensive care unit with respiratory failure secondary to massive hemoptysis and acute renal failure. The antiglomerular basement membrane antibodies were strongly positive. The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.
Goodpasture syndrome. Close-up view of gross patho Goodpasture syndrome. Close-up view of gross pathology in a 45-year-old man admitted to the intensive care unit with respiratory failure secondary to massive hemoptysis and acute renal failure. The antiglomerular basement membrane antibodies were strongly positive. The autopsy showed consolidated lung from extensive bleeding, which led to asphyxiation.

Goodpasture syndrome (ie, anti-GBM disease) is an uncommon disorder of complex pathogeneses. Early recognition and treatment of this syndrome are critical because the prognosis for recovery of renal function depends on the initial extent of injury. 

The three principles of therapy are as follows (see Treatment) [2] :

  1. Rapidly remove circulating antibody, primarily by plasmapheresis

  2. Stop further production of antibodies using immunosuppression with medications

  3. Remove offending agents that may have initiated the antibody production

Go to Pediatric Anti-GBM Disease (Goodpasture Syndrome) for complete information on this topic.

See Autoimmune Disorders: Making Sense of Nonspecific Symptoms, a Critical Images slideshow, to help identify several diseases that can cause a variety of nonspecific symptoms.


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