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Goodpasture syndrome is a rare and potentially life-threatening autoimmune disease. It occurs when your immune system mistakenly attacks the walls of your lungs and the tiny filtering units in your 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 your lungs, kidney failure, and even death.
Symptoms may start out slowly, gradually affecting your lungs and then your kidneys. In other cases they may progress rapidly, becoming severe in a matter of days. Initial symptoms may include:
If the disease moves to affect your lungs, the following symptoms may occur:
Sometimes symptoms affecting your lungs can become life threatening, particularly if there is a lot of bleeding.
If the disease affects your kidneys, it may cause:
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 your body’s defenses into thinking those parts are foreign to the body itself.
According to the National Kidney Foundation (NKF), Goodpasture syndrome affects men more often than women and occurs most commonly in early adulthood or after the age of 60. The NFK also reports that the disease is more common in Caucasians than in other races.
Your doctor may 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 (proteins produced by your immune system to fight whatever it is that has been identified as a threat) 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 your 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 your lungs.
A kidney biopsy may reveal changes that indicate the presence of Goodpasture syndrome. During this test, a sample of tissue from your 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.
Treatments involve medications that slow down your immune system. These may include one or more of the following:
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 your age, overall health, and the severity of the disease. Your doctor 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.
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 your kidneys begin to fail.
An early diagnosis is very important to survive the disease and for your long-term outlook. According to the NKF, 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.
Written by: Colleen Story
Medically reviewed on: Jan 11, 2016: Steve Kim, MD
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