Sometimes, even when your body tries to protect you, it can end up causing harm instead. When you have any infection, your immune system is activated to attack the germs. However, when the immune system mistakenly attacks your own tissues, it can damage your organs. This happens in autoimmune disorders like Goodpasture syndrome, a rare kidney disease that also affects the lungs. While Goodpasture syndrome can be life-threatening, doctors have ways to treat it. Finding it early gives you a better chance of recovery.

In this article, we’ll walk you through what Goodpasture syndrome is, how to spot the symptoms, and how doctors diagnose and treat it.

What Is Goodpasture Syndrome?

Goodpasture syndrome is a rare autoimmune condition that affects the air sacs in your lungs (where oxygen enters your blood) and the filtering units in your kidneys. It gets its name from Dr. Ernest Goodpasture, the doctor who first identified the disease in 1919. In autoimmune disorders, your immune system — which normally protects you — starts making antibodies that attack your own body by mistake. These harmful antibodies damage the basement membrane (lining) around the tiny blood vessels in the lungs and kidneys. This can lead to bleeding in the air sacs of the lungs and in the glomeruli (filtering units) of the kidneys, causing serious kidney problems.

How Common Is Goodpasture Syndrome?

Goodpasture syndrome is a rare disease. In the United States, fewer than two in every 1 million people are diagnosed each year. According to Johns Hopkins Medicine, it’s most common in young men in their 20s and older men in their 60s. Doctors may also refer to the disease as anti-glomerular basement membrane disease or anti-GBM disease. Goodpasture syndrome is more likely to affect people of white or European ancestry, as well as certain ethnicities, including the Maori people of New Zealand.

Symptoms of Goodpasture Syndrome

If you notice any of the lung and kidney symptoms discussed below, it’s important to let your healthcare team know right away. They can help figure out if it’s related to Goodpasture syndrome or something else.

Goodpasture Syndrome Triad

Doctors describe the three major symptoms of Goodpasture disease as a triad. These three symptoms are:

• Bleeding in the lungs
• Inflammation in the kidneys that develops rapidly
• Anti-GBM antibodies in the blood

Lung Symptoms

Other symptoms of Goodpasture disease depend on whether the lungs or kidneys are affected. Lung symptoms are more likely to appear first and may include:

• Shortness of breath or trouble breathing
• Coughing up blood
• Chest pain
• Fatigue (feeling very tired)
• Nosebleeds
• Paleness

Kidney Symptoms

Signs and symptoms of Goodpasture syndrome that affect the kidneys include:

• Hematuria (blood in the urine, which may look pink, red, or brown)
• Foamy urine (a sign of protein in the urine or proteinuria)
• Burning sensation when urinating
• Edema (swelling in the legs or feet)
• Hypertension (high blood pressure)

Diagnosis of Goodpasture Syndrome

Diagnosing Goodpasture syndrome can be challenging because its symptoms often look like other lung and kidney problems. To make a diagnosis, your doctor will start by reviewing your medical history and symptoms. They’ll also do a physical exam and, if needed, order specific tests to confirm the proper diagnosis.

Lab Tests

For example, blood tests can help check for special proteins called anti-GBM antibodies. These are harmful antibodies that attack the kidneys and lungs. If these are found, it usually confirms the disease. Blood tests are also useful in checking how well your kidneys are working by measuring things like creatinine, which is a waste product produced by the kidneys. They can also reveal signs of anemia (low red blood cells), which can happen if you’ve lost blood from the lungs or in the urine if your kidneys are damaged.

Urine tests (also called a urinalysis) check for signs of kidney damage. If there’s blood in your urine or too much protein, it can mean your kidneys are inflamed.

Imaging

A chest X-ray might also be used to give your doctor pictures of your lungs. If there’s bleeding, it may show up as cloudy white areas on the scan. This bleeding, called pulmonary hemorrhage, is a common sign of Goodpasture syndrome and can be very serious.

Kidney Biopsy

A kidney biopsy is considered the gold standard for diagnosing Goodpasture syndrome. To do this, your nephrologist or radiologist will remove a small piece of kidney tissue and send it to a lab to be evaluated under a microscope. This can help determine how much and where the kidneys have been affected by the disease. If a kidney biopsy isn’t possible, your doctor may choose to perform a lung biopsy instead. However, doctors usually prefer to do a kidney biopsy, as they tend to be more useful for understanding the extent of the disease.

What Causes Goodpasture Syndrome?

Goodpasture syndrome — or anti-GBM disease — happens when the body produces anti-GBM antibodies, which attack a part of the body called the basement membrane. This is found in both the kidneys and lungs. Doctors don’t know exactly why this happens in some people and not others. However, they suspect that it might result from a mix of environmental and genetic factors.

Genetic Factors

Some people have genes that make them more likely to get this rare disease. One of these genes is called HLA-DR15. This gene helps the immune system tell the difference between healthy cells and foreign invaders. In people with this gene, the immune system may get confused more easily. Since there is a genetic link in Goodpasture syndrome, if you have a family member with the disease, you may be more likely to get it, too.

Environmental Factors

Environmental triggers may also play a role. These are things in your surroundings that can set off the disease, especially if you already have genetic risk factors. Known environmental triggers for Goodpasture syndrome include:

• Cigarette smoking
• Exposure to certain chemicals (like those in paint thinners or gasoline)
• Exposure to metal dust (such as from jobs like welding)
• Certain viral infections
• Cocaine use, especially when smoked
• Use of hair-coloring products

These things may damage the lung tissue, which then makes it easier for the immune system to see and attack the basement membrane. Goodpasture syndrome isn’t contagious, so you can’t catch it from someone else.

Treatments for Goodpasture Syndrome

While Goodpasture can be life-threatening, there are effective treatments available. Getting it diagnosed and treated early is crucial. The goal is to stop the immune system from making anti-GBM antibodies, remove the ones already in the blood, and protect the lungs and kidneys from further damage. If not treated early or if the disease is already advanced, it can lead to kidney failure where the kidneys lose most of their ability to clear the waste products from the blood. Doctors may use a combination of treatments.

Medicines To Calm the Immune System

Doctors may use strong medications to slow down the immune system and stop it from attacking the lungs and kidneys. For example, corticosteroids, such as prednisone, may be given to help prevent bleeding in the lungs. Immunosuppressants, like cyclophosphamide, can also be prescribed to help prevent the body from making harmful antibodies.

Plasma Exchange

Plasmapheresis — or plasma exchange — is a special procedure that removes the bad antibodies from the blood. First, a machine takes out your blood and separates the plasma (the part of the blood that contains the antibodies). Then it replaces your plasma with healthy plasma from a donor and returns your blood to your body.

Dialysis

If your kidneys are failing, you may need dialysis. This is an ongoing treatment with the help of a machine that filters your blood when your kidneys no longer can. Sometimes it’s temporary, but if the damage is permanent, dialysis may be needed long term. Dialysis can’t replace all of your kidney function, but it can support some basic functions like removing waste products and excess fluid from the blood and maintaining electrolyte balance.

Kidney Transplant

If the kidneys are damaged and don’t recover after dialysis or other treatments, you may be at risk for kidney failure. If this happens, a kidney transplant may be an option. Your healthcare team will walk you through all of your options and discuss what to expect before, during, and after the procedure.

Life Expectancy and Outlook for Goodpasture Syndrome

With effective treatments now available, doctors estimate that the five-year survival rate for Goodpasture syndrome is about 80 percent. This means that 80 out of 100 people with the disease are still alive five years after their diagnosis.

With early diagnosis and proper treatment, many people with Goodpasture syndrome can recover and go on to live full and healthy lives. The sooner a diagnosis is made and treatment begins, the better the chances of protecting the lungs and kidneys.

Some people may have a full recovery, especially if the disease is caught before major damage happens. Others may need long-term dialysis or even a kidney transplant if the kidneys were badly injured. Most people don’t have lasting lung problems after the bleeding stops.

After treatment, your doctor will continue to monitor for signs that the disease is coming back. This means regular blood tests, urine tests, and visits with a nephrologist (kidney specialist). However, the good news is that relapses (periods when the disease returns) are very rare.

Talk With Others Who Understand

On MyKidneyDiseaseTeam, the social network for people with kidney disease and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with kidney disease.

Have you been diagnosed with Goodpasture disease? What symptoms did you have, and what has your journey been like? Share your experience in the comments below, or start a conversation by posting on your Activities page.