Goodpasture's Syndrome

Goodpasture's Syndrome is an uncommon autoimmune disease that affects both the kidneys and the lungs. An autoimmune disease means that the immune system, which usually protects the body from infection, attacks healthy parts of the body by mistake.

The body's immune system produces antibodies, which are proteins that help fight infections. However, in Goodpasture's Syndrome the body makes antibodies that attack and damage the lining of your lungs and kidney. As a result, if you have this disease, you may start to experience fatigue, weakness and loss of appetite. However, the disease may quickly progress and you may bleed from the lungs and cough up blood. It may also lead to inflamed kidneys (glomerulonephritis). It is not exactly known why your antibodies begin to attack your own lungs and kidneys. Factors that may trigger the disease include exposure to viral lung infections, smoking and breathing in vapors from organic solvents.

This problem is most common in people between the ages of 20 and 30 or after age 60. It is not contagious and it is more common in men and Caucasians. Sometimes one will suffer from these symptoms as a result of other diseases, such as lupus erythematosus or Wegener's granulomatosis.

Goodpasture's Syndrome may cause life-threatening bleeding in the lungs, but does not usually cause long-term damage in that area. The harm done to your kidneys, however, can result in kidney failure. You may need either dialysis or a kidney transplant. If your kidney function is affected, you may:

• appear pale and feel tired (anemia)
• have blood or protein in your urine
• suffer from back pain below your ribs
• go to the bathroom more often, especially at night
• have burning or difficulty when urinating
• have swelling of the hands and feet
• experience high blood pressure.
• have nausea and vomiting

There is a specific blood test that can show if you have harmful antibodies attacking your lungs and kidneys.

A doctor can also test your blood and urine to find out if you have kidney disease. A urine test can check for the amount of protein, blood and other things to indicate kidney damage. A blood test for serum creatinine can be used to calculate glomerular filtration rate (GFR), which tells how well your kidneys are filtering wastes from the blood.

Your doctor may also perform a kidney biopsy to see if the kidney tissue is damaged by the antibody.

Your lungs may also be checked for damage with a chest X-ray and lung biopsy.

Early diagnosis and treatment are the best ways to prevent kidney damage. Your doctor may give you medications to help slow the immune system and try to keep it from making harmful antibodies. You may also be given medication to control fluid buildup or high blood pressure. The doctor may suggest that you undergo a special blood filtering process (plasmapheresis) to remove harmful antibodies.

Usually, your body will make the antibodies for a short time, anywhere from a few weeks to two years. Once this stops, you should not have any more problems with your lungs. However, your kidneys may have been slightly or heavily damaged. The five year survival rate is 80%. Fewer than 30% of people require long term dialysis.

Sometimes you will be asked to make changes to your diet due to high blood pressure or lower kidney function. For example, you may need to control you intake of salt, fluids and protein. Your physician, dietitian or another member of your health care team can help you understand these changes.

Not smoking and avoidance of secondary smoke is very important. Other measures that may help to keep you healthy are exercise and stress reduction.

Unfortunately, by the time you see your doctor, your kidneys may already be damaged. If the damage is severe, you may require dialysis or a kidney transplant. Before you can get a transplant, your doctor will make sure the harmful antibodies are out of your system. Medications taken when you get a transplant also help to prevent your body's immune system from making the harmful antibodies.

Yes. Currently, there is much interest in learning what causes the harmful antibodies. This work may lead to new drugs that are more helpful.

Research is being conducted to discover genetic factors that are important in the disease. Also, researchers are searching for better ways to treat the bleeding that happens in the diseased lungs.

Date Reviewed: July 2009

See also in this A-Z guide:

• How Your Kidneys Work
• Tests to Measure Kidney Function, Damage and Detect Abnormalities
• Kidney Biopsy

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© 2015 National Kidney Foundation. All rights reserved. This material does not constitute medical advice. It is intended for informational purposes only. Please consult a physician for specific treatment recommendations.