Goodpasture’s (anti-GBM) disease

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

This is a short account. Click here for a more detailed, illustrated account of Goodpasture’s disease.

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 may cough up blood, but some people may 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 is 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 almost always be controlled over a few days of treatment. Lung disease usually recovers completely. 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.

What can I do to help?
  • do not smoke
  • take medication carefully as prescribed
  • have blood counts and other tests regularly
  • follow fluid and diet recommendations
  • watch out for signs of infection
Further information

More detailed (but quite technical in parts) information is available on this website. We have found few other sources of good information for patients with this disease.

 

Acknowledgements:  The author of this page was Neil Turner. It was first published in January 2000. The date is was last modified is shown in the footer.