What is Goodpasture syndrome?

Goodpasture syndrome is a rare and potentially life-threatening autoimmune disease. It causes buildup of autoimmune proteins in the kidneys and lungs that leads to damage of these organs. The disorder is named after Dr. Ernest Goodpasture, who first identified the syndrome in 1919. It’s estimated to occur in 1 out of 1 million people per year.

Without prompt diagnosis and treatment, the condition can lead to significant bleeding in your lungs, kidney inflammation and failure, and even death.

What are the symptoms?

Symptoms can be nonspecific, as well as related to both your lungs and kidneys. Damage can progress rapidly, becoming severe in a matter of days. Initial symptoms may include:

When the disease affects your lungs, the following symptoms may occur:

Sometimes symptoms affecting your lungs can become life-threatening, causing respiratory failure, particularly if there is a lot of bleeding.

When the disease affects your kidneys, it may cause:

  • burning sensation during urination
  • blood in your urine or foamy urine
  • swelling of your hands and feet
  • elevated blood pressure readings
  • back pain below your ribs

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 immune system. Exposure to hydrocarbon fumes, metallic dust, tobacco smoke, or certain drugs, such as cocaine, may also increase risk.

Scientists believe the immune system attacks lung and kidney tissue because your body’s defenses identify parts of those organs as foreign to the body itself.

Certain individuals appear to be more susceptible to this condition due to genetics. This includes people who have inherited specific proteins from their parents, found as part of the HLA (human leukocyte antigens) system. For example, a specific HLA, known as DR15, is found in 88 percent of people who have Goodpasture syndrome.

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.

How is Goodpasture syndrome diagnosed?

Your doctor may use several tests to diagnose Goodpasture syndrome. They will start with a physical examination, checking for high blood pressure, bleeding, and abnormal heart and lung sounds, including an abdominal exam. Your doctor will also review your family and medical history. It isn’t uncommon to have a heart murmur, abnormal lung sounds, or an enlarged liver due to this condition.

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 abnormal kidney function.

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 or CT scan may show signs that indicate lung injury and bleeding in your lungs.

A kidney biopsy may reveal changes that indicate the presence of Goodpasture syndrome. During this test, a sample of tissue is taken from your kidney using an ultrasound as a guide, 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?

Once diagnosed, you will need treatment as soon as possible to decrease your risk of complications. Goodpasture syndrome is a life-threatening condition. It requires hospitalization, which often includes treatment in an intensive care unit (ICU).

Treatments involve medications that slow down your immune system. These may include one or more of the following:

  • Immunosuppressive or cytoxic drugs keep your immune system from making the antibodies that damage your lungs and kidneys (one example is cyclophosphamide).
  • Corticosteroids, such as prednisone (Rayos), which decrease inflammation as well as suppress your immune system.

A treatment called plasmapheresis may be needed to filter out harmful antibodies in your blood. During this procedure, blood is withdrawn, and the liquid portion (plasma) is removed and replaced. The filtered 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 buildup 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 long-term 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 your kidneys begin to fail.

An early diagnosis and treatment 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 with proper care.

Fewer than 30 percent of people with Goodpasture syndrome will suffer long-term kidney damage that requires dialysis.

Quitting smoking, if you smoke, and avoiding secondhand smoke is another important step to improving your long-term outlook.