Anti-GBM (Goodpasture’s) Disease | NIDDK


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What is anti-glomerular basement membrane (anti-GBM) disease?

Anti-GBM disease is a disorder in which your body’s immune system creates antibodies that attack your kidneys and lungs. As a result, you may develop one or both of these conditions

  • glomerulonephritis—inflammation of the glomeruli, tiny units in your kidney that filter wastes and extra fluid from your blood
  • bleeding in your lungs

If left untreated, anti-GBM disease can quickly get worse and may lead to kidney failure and death.

Does anti-GBM disease have another name?

Anti-GBM disease is sometimes also called Goodpasture’s disease.1 Another related term is Goodpasture syndrome, a condition that also affects the kidneys and lungs but is not caused by anti-GBM antibodies.

How common is anti-GBM disease?

Anti-GBM disease is rare, with only about 1 in 1 million new cases being reported per year.1

Who is more likely to develop anti-GBM disease?

Anti-GBM disease most often affects men in their 20s and women in their 60s, but it can occur at any age.1 The condition can occur in children, but this is extremely rare.

What are the complications of having anti-GBM disease?

If not treated promptly, anti-GBM disease can cause serious complications, such as

  • severe kidney inflammation, which can quickly lead to kidney failure
  • severe bleeding in the lungs, which can cause respiratory failure

What are the symptoms of anti-GBM disease?

Anti-GBM disease most often starts with symptoms such as

These symptoms may appear before or along with symptoms of lung and kidney problems.1

Symptoms of lung problems may include

  • coughing up blood
  • dry cough
  • shortness of breath
  • chest pain

Symptoms of kidney problems may include

Seek a health care professional’s help right away if you have these symptoms.

What causes anti-GBM disease?

Researchers don’t fully understand the cause of anti-GBM disease. A combination of your genes and factors in the environment may put you at risk.

Genes

Some genetic traits that parents pass on to their children, while uncommon, may affect the risk for developing anti-GBM disease. For example, scientists have found a link between anti-GBM disease and human leukocyte antigen (HLA), a protein on the surface of cells that plays an important role in immune response.2 Some types of HLA may increase your risk of developing the disorder, while others may decrease this risk.

Environment

Factors in your environment may also increase your risk of developing anti-GBM disease. They include 1

  • inhaled hydrocarbons—chemicals derived from petroleum, found in gasoline, kerosene, paint thinner, furniture polish, and other household products
  • smoking
  • infections
  • inhaled cocaine
  • contact with metal dusts
  • use of the medicine alemtuzumab

How do health care professionals diagnose anti-GBM disease?

Health care professionals diagnose anti-GBM disease by reviewing your symptoms and medical history and ordering certain lab tests. These tests often include

  • urinalysis, or a urine test, which checks a sample of your urine for blood and protein that can pass into the urine when your kidneys are damaged.
  • blood tests, which can detect anti-GBM antibodies in your blood and signs of kidney damage. Testing for anti-GBM antibodies is extremely important because it gives the diagnosis and guides treatment.
  • chest x-ray, which can also help detect any damage to your lungs.
  • kidney biopsy, in which a health care professional examines a small piece of tissue from your kidney under a microscope to look for anti-GBM antibodies and signs of kidney damage. Your health care professional may refer you to a nephrologist to perform this test.
To diagnose anti-GBM disease, health care professionals may use your medical history and a physical exam.

How do health care professionals treat anti-GBM disease?

Health care professionals most often treat anti-GBM disease with1

  • Cyclophosphamide. This medicine suppresses your immune system, stopping your body from making anti-GBM antibodies. Your health care professional may also give you an antibiotic to take while you are on this medicine.
  • Corticosteroids. These medicines curb your body’s immune response and lower inflammation in your body.
  • Plasmapheresis. Also called therapeutic plasma exchange, this therapy uses a machine to filter your blood and remove anti-GBM antibodies.

Plasmapheresis is usually done for 2 to 3 weeks.1 Depending on how your body responds, you may need to continue taking medicines that suppress your immune system for up to 9 months.

Once treated, anti-GBM disease rarely comes back. When it does, it is often due to ongoing exposure to chemicals that irritate the lungs.4 People who have had anti-GBM disease should avoid these chemicals.

How do health care professionals treat the complications of anti-GBM disease?

Patients who are very ill by the time they seek care may need additional treatments, such as3

  • urgent dialysis
  • intubation—a medical procedure in which a tube is placed into the windpipe through the mouth or nose—for respiratory failure

In most cases, bleeding in the lungs stops and anti-GBM disease doesn’t cause lasting damage to your lungs.4 However, the disorder can cause serious damage to your kidneys.

The extent of damage to the kidneys often depends on how well your kidneys were working before you started treatment. If you already need dialysis by the time you start treatment, you are likely to stay on dialysis after treatment. Some people may need a kidney transplant.

Clinical Trials for Anti-GBM Disease

The NIDDK conducts and supports clinical trials in many diseases and conditions, including kidney diseases. The trials look to find new ways to prevent, detect, or treat disease and improve quality of life.

What are clinical trials for anti-GBM disease?

Clinical trials—and other types of clinical studies—are part of medical research and involve people like you. When you volunteer to take part in a clinical study, you help doctors and researchers learn more about disease and improve health care for people in the future.

Find out if clinical trials are right for you.

What clinical studies for anti-GBM disease are looking for participants?

You can view a filtered list of clinical studies on [condition] that are federally funded, open, and recruiting at www.ClinicalTrials.gov. You can expand or narrow the list to include clinical studies from industry, universities, and individuals; however, the National Institutes of Health does not review these studies and cannot ensure they are safe. Always talk with your health care provider before you participate in a clinical study.

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