What Is Goodpasture Syndrome?
Goodpasture syndrome is a rare and potentially life-threatening autoimmune disease. It occurs when the immune system attacks the walls of the lungs and the tiny filtering units in the kidneys. The disorder is named after Dr. Ernest Goodpasture, who identified the syndrome in 1919.
Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death.
What Causes Goodpasture Syndrome?
While the exact cause of Goodpasture syndrome is unknown, certain behaviors and environmental factors are believed to put people at higher risk. Certain respiratory infections may trigger the disease. Exposure to hydrocarbon fumes, metallic dust, tobacco smoke, or certain illegal substances such as cocaine may also increase risk.
Scientists believe the immune system attacks lung and kidney tissue because the condition fools body defenses into thinking the parts are foreign to the body itself.
According to the National Institutes of Health (NIH), Goodpasture syndrome affects men eight times more often than women and occurs most commonly in early adulthood. (NIH, 2011) The National Kidney Foundation (NKF) reports that the disease is more common in Caucasians than in other races. (NKF, 2009)
What Are the Symptoms?
Symptoms may start out slowly, gradually affecting the lungs and then the kidneys. Other times they may progress rapidly, becoming severe in a matter of days. Initial symptoms may include:
- fatigue, weakness, or lethargy
- nausea and/or vomiting
- loss of appetite
- unhealthy, pale appearance
If the disease moves to affect the lungs, the following symptoms may occur:
- dry cough or coughing up blood
- shortness of breath or difficulty breathing
Sometimes symptoms affecting the lungs can become life-threatening, particularly if there is a lot of bleeding.
If the disease affects the kidneys, it may cause:
- burning sensation during urination
- blood in the urine or foamy urine
- swelling of the hands and feet
- high blood pressure
- back pain below the ribs
How Is Goodpasture Syndrome Diagnosed?
Doctors use several tests to diagnose Goodpasture syndrome. Your doctor will start with a regular physical examination, checking for high blood pressure, bleeding, and abnormal heart and lung sounds. Your doctor will also review your medical history.
Other tests can help determine whether or not you have the disease. A blood test may show the presence of antibodies that indicate the presence of the disease. It can also show a high level of waste products, which may indicate kidney problems.
The presence of blood and protein in the urine can be detected through urine testing. These symptoms can also indicate kidney problems.
A chest X-ray may show the presence of abnormal white patches that indicate bleeding in the lungs.
A kidney biopsy may reveal changes that indicate the presence of Goodpasture syndrome. During this test, a sample of tissue from the kidney is removed and sent to a lab for testing. Lab technicians will look for the presence of antibodies or other abnormal cells to help your doctor make a diagnosis.
How Is Goodpasture Syndrome Treated?
Treatments involve medications that slow down your immune system. These may include one or more of the following:
- immunosuppressive drugs to keep your immune system from making the antibodies that damage your lungs and kidneys. Cyclophosphamide is one example.
- corticosteroids like prednisone to help control bleeding in your lungs. These medications also suppress your immune system.
A treatment called plasmapheresis may be used to help filter out harmful antibodies in your blood. During this procedure, blood is withdrawn and the liquid portion (plasma) is removed and replaced. The "cleaned" blood is transferred back into your body.
Other treatments depend on the individual’s age, overall health, and the severity of the disease. Doctors may prescribe additional medications to control fluid build-up and high blood pressure. In addition to medication, dietary changes such as cutting down on salt intake can help control swelling and blood pressure.
What Is the Expected Outlook?
The more lung and kidney function can be preserved, the better. The outlook seems particularly dependent on the condition of your kidneys. Damage to the kidneys is often permanent, and a kidney transplant or dialysis (a process that uses specialized machinery to help filter waste and toxins out of the blood) may be necessary if the kidneys begin to fail.
Individuals who obtain an early diagnosis stand the best chance of surviving the disease. According to the National Kidney Foundation, the syndrome can last anywhere from a few weeks to two years. The five-year survival rate is 80 percent.
Fewer than 30 percent of people with Goodpasture syndrome will suffer long-term kidney damage that requires dialysis. (NKF, 2009)