Goodpasture Syndrome (Anti-GBM Disease)

What is Goodpasture syndrome?

Goodpasture syndrome is a rare, life-threatening autoimmune disease that affects your kidneys and lungs. It happens when your immune system attacks collagen because it mistakes it as foreign. Collagen is a protein that helps make up your:

• Skin.
• Muscles.
• Connective tissues, like tendons and ligaments.
• Bones.

If you have Goodpasture syndrome, your body produces proteins (antibodies) that attach to collagen in certain parts of your lungs and kidneys. When this happens, it causes inflammation and destroys the tissues. Without treatment, Goodpasture syndrome can cause kidney inflammation (glomerulonephritis) that can lead to kidney failure. It can also cause severe bleeding in your lungs (pulmonary hemorrhage), which is the main cause of death from Goodpasture syndrome.

Other names for Goodpasture syndrome include:

• Anti-glomerular basement membrane (anti-GBM) disease.
• Goodpasture disease.

How common is Goodpasture syndrome?

Goodpasture syndrome is very rare. Healthcare experts report fewer than two new cases per 1 million people yearly.

What are the symptoms of Goodpasture syndrome?

Goodpasture syndrome is a pulmonary-renal condition. That means it affects your lungs (pulmonary) and kidneys (renal). Lung-related symptoms usually appear first. They include:

• Shortness of breath (dyspnea).
• Chest pain.
• Cough.
• Rattling lung sound when you breathe in.
• Feeling very tired (fatigue).
• Nosebleeds (epistaxis).
• Coughing up blood.
• Pale skin (pallor).

Kidney-related symptoms include:

• Low red blood cell counts (anemia).
• Blood in your pee (hematuria).
• Peeing less than usual.
• High blood pressure.
• Nausea and vomiting.

What causes Goodpasture syndrome?

Goodpasture syndrome causes your immune system’s antibodies to attack collagen in the glomerular basement membrane (GBM) of your kidneys. The GBM is part of your glomeruli. Glomeruli are tiny blood vessels in your kidneys that help filter your blood. Anti-GBM antibodies also attack collagen in your lungs’ air sacs. This destroys lung tissue, which leads to bleeding and difficulty breathing.

Healthcare providers and medical researchers aren’t exactly sure what causes your immune system to overreact to collagen. It may result from a combination of environmental factors and the genes you inherit from your biological parents. Studies of people with Goodpasture syndrome have shown a strong association with human leukocyte antigen (HLA) DR15. HLAs are proteins that help your immune system tell the difference between your tissues and invading substances.

Sometimes, Goodpasture syndrome can develop after an infection, like a cold or the flu. You may also be more likely to develop it if you:

• Smoke.
• Inhale (snort) cocaine.
• Have exposure to metal dust and hydrocarbon chemicals, like methane or propane.

Who does Goodpasture syndrome affect?

Goodpasture syndrome affects people of all ages. But it more often affects those early in life (teens to 30s), then again in the 60s and 70s.

How is Goodpasture syndrome diagnosed?

A healthcare provider will ask about your symptoms and perform a physical exam. They’ll also order tests to help diagnose Goodpasture syndrome, including:

• Blood tests. Providers use blood tests to measure your estimated glomerular filtration rate (eGFR). This measures how well your kidneys are working.
• Pee test (urinalysis). This can determine if you have blood or high levels of protein (proteinuria) in your pee.
• Imaging tests. A chest X-ray or CT scan will determine if you have lung damage.
• Bronchoscopy. A provider will use a long, thin tube with a light and camera at the end (bronchoscope) to look for lung damage.
• Kidney biopsy. A provider will remove a small sample of kidney tissue to help diagnose kidney disease.

Is Goodpasture syndrome treatable?

Yes, with proper diagnosis, Goodpasture syndrome is treatable.

How is Goodpasture syndrome treated?

Goodpasture syndrome treatment can vary depending on its severity. Healthcare providers treat mild cases with medications, including:

• Corticosteroids, like prednisone, to stop bleeding in your lungs.
• Immunosuppressant drugs, like cyclophosphamide, to prevent your immune system from attacking your tissues.
• Blood pressure medications (antihypertensives) to help lower your blood pressure and reduce kidney damage.

Providers also use plasmapheresis. During this procedure, they use a needle to remove blood from a vein in your body, usually your arm. They separate the liquid part of your blood (plasma) from the blood cells. The plasma contains the harmful anti-GBM antibodies. They replace your plasma with healthy plasma from blood donors and return it to your body.

How soon after treatment will I feel better?

You may need to take immunosuppressant drugs for six to 12 months. In most cases, you need plasmapheresis every day for several weeks.

Can Goodpasture syndrome be prevented?

You may not be able to prevent Goodpasture syndrome. But you can lower your risk by avoiding:

• Hydrocarbons, including methane, propane, gasoline, kerosene, tar and asphalt.
• Metal dust.
• Cocaine.
• Hair-coloring products.

What can I expect if I have Goodpasture syndrome?

Without treatment, Goodpasture syndrome may cause life-threatening bleeding in your lungs. It can also lead to kidney failure. But with an early diagnosis, treatments are effective. Your immune system will only make the antibodies for a short period — a few weeks up to two years. Relapses or recurrences of the disease are very rare.

The most severe complication of Goodpasture syndrome is kidney failure. Kidney failure treatment requires dialysis or a kidney transplant.

What is my life expectancy if I have Goodpasture syndrome?

The five-year survival rate for people with Goodpasture syndrome is 80%.

How do I take care of myself if I have Goodpasture syndrome?

If you have Goodpasture syndrome, it’s a good idea to:

• Quit smoking and avoid secondary smoke.
• Engage in physical activity for at least 30 minutes each day.
• Reduce stress.

When should I see a healthcare provider?

See a healthcare provider right away if you’re coughing up blood, having trouble breathing, peeing less than usual or having any other symptoms of Goodpasture syndrome. Early diagnosis can significantly improve your outlook. Without treatment, Goodpasture syndrome can cause permanent kidney damage or fatal lung problems.

What questions should I ask a healthcare provider?

• How will you diagnose Goodpasture syndrome?
• How serious is my case?
• What treatment do you recommend?
• How can I help take care of my kidneys?
• Should I see a nephrologist or pulmonologist?
• Can you recommend a Goodpasture syndrome support group?

What is the triad of Goodpasture syndrome?

In medicine, a triad is a group of three symptoms that describe a medical condition. The triad of Goodpasture syndrome is:

• Bleeding in your lungs.
• Inflammation in your kidneys that progresses quickly.
• The presence of anti-GBM antibodies.