Mar
Goodpasture's disease
from EdREN, the website of the Renal Unit of the Royal Infirmary of Edinburgh
Goodpasture's disease is a rare but serious condition that can cause severe kidney and lung damage. The disease can be controlled by treatment, but often leaves patients with severe kidney damage.
Other names for Goodpasture's disease
Anti-glomerular basement membrane disease
Anti-GBM disease
Goodpasture's syndrome
What are the signs of Goodpasture's disease?
Some people have only lung disease, others have only kidney disease, while some have both.
Lung disease: you might cough up blood, but some people might have just a dry cough or breathlessness. It can be mild or very severe.
Kidney disease: ranges from just a hint of blood on urine testing, to very severe kidney damage which happens very rapidly.
How is it diagnosed?
A blood test to look for anti-GBM antibodies is one way. However most patients who are thought to have Goodpasture's disease, or related conditions, will need to have a kidney biopsy (a sample of kidney removed through a needle).
What’s the treatment?
Kidney failure may need dialysis treatment. Severe lung disease needs oxygen, and occasionally artificial ventilation in an intensive care unit.
Treatment to control the disease usually has three parts;
Steroid treatment (prednisolone, prednisone) reduces inflammation.
Cyclophosphamide (Cytoxan, other trade names) suppresses the immune system.
Plasma exchange is a treatment with a machine that removes plasma from the patient and replaces it with donor plasma.
These are powerful treatments, and their most important side effect is a serious risk of infection. Regular blood tests help to minimise this. The good news is that these treatments are usually only required for a few months, not life-long. For some patients who do not have lung disease and whose kidneys are very severely damaged, it may be safest to have no treatment at all.
Will I recover?
The disease can nearly always be controlled over a few days of treatment. Lung disease usually recovers totally. Kidney damage can sometimes be arrested or reversed, but unfortunately is often very severe, and many patients are left with permanent kidney failure requiring dialysis and transplantation. Transplantation is only possible when all signs of the disease have disappeared, otherwise the transplanted kidney will also be affected.
What causes Goodpasture's disease?
Goodpasture's disease is an autoimmune condition, in which the body's immune system mistakenly attacks a target within the body. In Goodpasture's disease the target is found in the filtering units of the kidney (the glomeruli) and the oxygen-exchanging units of the lungs (alveoli). As for all other autoimmune disorders, it is usually not known what triggers the disease. It does not run in families.