Goodpasture Syndrome (Anti-GBM Disease)

Overview

What is Goodpasture syndrome?

Goodpasture syndrome (or anti-GBM disease) is a rare, life-threatening autoimmune disease that affects the lungs and the kidneys. It happens when the immune system mistakenly attacks a protein called collagen because it recognizes it as a foreign substance. In Goodpasture syndrome, the body produces proteins (antibodies) that attach to the collagen in certain parts of the lungs and the kidneys. When they attach to the collagen, these antibodies cause severe inflammation and destruction of those tissues.

Symptoms of the disease include coughing up blood, difficulty breathing, fatigue and anemia. When the disease affects the kidneys, patients may have blood in the urine, swelling of the legs and high blood pressure. If the kidney disease is very severe, patients may notice that they are passing only small amounts of urine and may have nausea and vomiting as well. Treatments include medications and a procedure called plasmapheresis. This procedure removes plasma that contains these harmful antibodies and replaces it with healthy plasma.

Untreated, Goodpasture syndrome can cause inflammation of the kidneys (glomerulonephritis) and can lead to permanent kidney failure. The disorder can cause severe bleeding in the lungs, which is the main cause of death from Goodpasture syndrome.

How common is Goodpasture syndrome?

Goodpasture syndrome was first described in 1919 and is very rare. It is estimated that there are fewer than two cases per one million people. The syndrome affects men more often than women. It usually begins between ages 20-30 or after age 60.

Symptoms and Causes

What are the symptoms of Goodpasture syndrome?

Goodpasture syndrome is a pulmonary-renal syndrome. It affects the lungs (pulmonary) and the kidneys (renal). Lung-related symptoms usually appear first. They include:

Goodpasture syndrome symptoms that result from kidney damage include:

What causes Goodpasture syndrome?

As a type of autoimmune disorder, Goodpasture syndrome causes antibodies produced by the immune system to attack collagen. Collagen is a protein that has many functions. It forms part of the structure of many tissues and helps blood clot, among others. Goodpasture syndrome attacks collagen in the glomerular basement membrane (GBM) of the kidneys. These anti-GBM antibodies also attack collagen in the lung’s air sacs, destroying the tissues and leading to bleeding and difficulty breathing.

We aren’t sure what causes Goodpasture syndrome. It may result from a combination of environmental factors and genetics. In people with Goodpasture syndrome, 8 to 90% have a type of human leukocyte antigen (HLA) called HLA-DR15. HLAs are proteins that help the immune system tell the difference between the body’s own tissues and foreign (or invading) substances. In people with HLA-DR15, the antigen doesn’t work like it should.

Sometimes, Goodpasture can develop after an infection, such as a cold or the flu. People who smoke, use cocaine or are exposed to metal dust and hydrocarbon chemicals (such as methane or propane) are more likely to develop Goodpasture syndrome. Scientists believe that these environmental factors may trigger the disorder in people who have HLA-DR15.

Diagnosis and Tests

How do healthcare providers diagnose Goodpasture syndrome?

Your provider will examine you and ask about your symptoms. To diagnose Goodpasture syndrome, your provider will order:

  • Blood tests to check how your kidneys are working and look for antibodies in your blood.
  • Urine test to check for blood or protein.
  • CT scan or chest X-ray to look for lung damage.
  • Bronchoscopy to examine your lungs.
  • Kidney biopsy, to look for glomerulonephritis (inflammation of the kidneys) and to check for anti-GBM antibodies in the kidney tissue.

Management and Treatment

How do providers treat Goodpasture syndrome?

Treatments vary depending on the severity of the condition. Providers treat mild Goodpasture syndrome with medications, including:

  • Corticosteroids: Such as prednisone, to stop bleeding in the lungs.
  • Immunosuppressant drugs: Such as cyclophosphamide, to prevent the immune system from attacking the body’s own tissues.

Providers treat severe Goodpasture syndrome with these medications as well as a procedure called plasmapheresis. In this procedure, blood is removed through an IV (a vein in your arm) and the liquid part of the blood (plasma) is separated from the blood cells. This plasma, which contains the harmful anti-GBM antibodies, is replaced with healthy plasma from blood donors and returned to your body.

Prevention

Can I prevent Goodpasture syndrome?

You may not be able to prevent Goodpasture syndrome. But you can lower your risk by avoiding tobacco and chemicals that may trigger the disorder.

Healthcare providers can do a blood test to check for the HLA-DR15 antigen. If you know you have the HLA-DR15 antigen, ask your provider about regular checkups to monitor your health. If you smoke, talk to your provider about how to quit smoking.

If you’re frequently exposed to gasoline, kerosene, tar or asphalt, you have a higher risk of developing the condition. You should avoid these hydrocarbons if possible.

Outlook / Prognosis

What is the outlook for people with Goodpasture syndrome?

Goodpasture syndrome causes life-threatening bleeding in the lungs. Without treatment, this bleeding is fatal. It can also lead to kidney failure. With an early diagnosis, treatments are effective. After receiving treatment, see your provider regularly so they can monitor your kidney function.

Some people with Goodpasture syndrome have long-term kidney problems. They may need dialysis and eventually, a kidney transplant.

Living With

When should I see my healthcare provider about Goodpasture syndrome?

See your provider right away if you are coughing up blood, have trouble breathing or if you have any other symptoms of Goodpasture syndrome. Early diagnosis can significantly improve the outlook for people with this disease. Without treatment, Goodpasture syndrome can cause fatal lung problems.

A note from Cleveland Clinic

See your provider right away if you have signs of Goodpasture syndrome. Without treatment, the disorder can cause long-term damage to the kidneys and life-threatening bleeding in the lungs. If you smoke, talk to your provider about a plan to help you quit. If you’ve received treatment for Goodpasture syndrome and your job requires you to be around gasoline, tar or asphalt, consider changing jobs. Exposure to chemicals like these increases your chance of the syndrome returning.

Last reviewed by a Cleveland Clinic medical professional on 08/30/2021.

References

  • Genetic and Rare Diseases Information Center. . Accessed 9/21/2021.Goodpasture syndrome (https://rarediseases.info.nih.gov/diseases/2551/goodpasture-syndrome)
  • Merck Manual (Consumer Version). . Accessed 9/21/2021.Goodpasture Syndrome (https://www.merckmanuals.com/home/lung-and-airway-disorders/autoimmune-disorders-of-the-lungs/goodpasture-syndrome)
  • National Organization for Rare Disorders. . Accessed 9/21/2021.Goodpasture Syndrome (https://rarediseases.org/rare-diseases/goodpasture-syndrome/)

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