- anti-GBM antibody disease
Goodpasture syndrome is a condition in which a person's own immune system attacks the lungs and kidneys. This may result in coughing up blood and rapid kidney failure.
What is going on in the body?
Goodpasture syndrome is a type of autoimmune disease, in which the person's body attacks its own tissue for unknown reasons. It occurs when a person's antibodies mistakenly attack the air-filled sacs of the lungs and the tiny filters in the kidneys. This can lead to rapid kidney failure and lung problems.
What are the causes and risks of the disease?
The cause of Goodpasture syndrome is unknown. It is more commonly seen in people who smoke, or have been exposed to hydrocarbons, such as gasoline and paint thinners. A tendency to the condition may be inherited.
What can be done to prevent the disease?
There is no known way to prevent Goodpasture syndrome.
How is the disease diagnosed?
A person's symptoms may make a healthcare professional suspect Goodpasture syndrome.
To confirm the diagnosis, he or she may then lab tests such as:
urinalysis and other urine tests, which can detect kidney damage
a chest x-ray, which may show signs of swelling in the lungs
a complete blood count, or CBC, to detect anemia, or a low red blood cell count
kidney function tests, to see how badly the kidney has been damaged
an antibody titer blood test, to see if abnormal antibodies are present
- a biopsy of the kidney. In this test, a tiny sliver of kidney tissue is obtained with a long needle inserted through the skin of the back, and examined for signs of damage to the small filters of the kidney
Long Term Effects
What are the long-term effects of the disease?
If Goodpasture syndrome is not diagnosed and treated right away, the damage from the disease can lead to end-stage renal failure, in which the kidney ceases to function. Being exposed to hydrocarbons, smoking, and having a lung infection may increase a person's risk of bleeding from lungs with Goodpasture syndrome.
What are the risks to others?
Goodpasture syndrome is not contagious, and poses no risk to others.
What are the treatments for the disease?
Some of the treatments for Goodpasture syndrome include the following:
- plasma exchange, or plasmapheresis. This is a procedure in which the abnormal antibodies, along with other blood proteins, are filtered out of the blood. There are then fewer of these antibodies to cause damage to the kidneys and the lungs.
- steroids to treat inflammation in the lungs and kidneys
- immunosuppressive medications, such as cyclophosphamide (i.e., Cytoxa), to stop the white blood cells from making abnormal antibodies
If there is complete kidney failure, dialysis may be necessary. This is a procedure in which a machine filters the blood when the kidneys are no longer able to do so.
What are the side effects of the treatments?
The side effects depend on the treatment used.
- Plasmapheresis may remove proteins that help the blood clot. This can lead to bleeding.
- Steroids and cyclophosphamide suppress the body's immune system. This means that the person is at higher risk for infections.
- Cyclophosphamide can sometimes cause bleeding from the bladder. Drinking large amounts of fluids helps to reduce this risk.
What happens after treatment for the disease?
Even after it has been treated, Goodpasture syndrome can sometimes occur again. If a person needs dialysis when he or she is first treated, it is unlikely that the kidney damage can be reversed.
How is the disease monitored?
The healthcare professional should be contacted right away if there is blood in the urine or if the person coughs up blood. Regular urinalyses will be done to check for protein and blood in the urine. Kidney function and antibody formation will be monitored periodically with laboratory tests.
Isselbacher KJ, Braunwald E, Wilson, JD, et al: Harrison's principles of internal medicine. ed 13; p1309. New York, 1994, McGraw-Hill