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Genetic and Rare Diseases Information Center (GARD)

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Goodpasture syndrome


Other Names for this Disease

  • Anti-glomerular basement membrane antibody disease
  • Glomerulonephritis - pulmonary hemorrhage
  • Pulmonary renal syndrome
  • Rapidly progressive glomerulonephritis with pulmonary hemorrhage
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My mother-in-law died from Goodpasture syndrome. Is my husband at an increased risk for developing this syndrome in the future?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Goodpasture syndrome?

Goodpasture syndrome is an autoimmune disease of unknown cause that affects the lungs and kidneys. The typical symptoms of Goodpasture syndrome include pulmonary alveolar hemorrhage (bleeding in the lungs), glomerulonephritis, and the presence of anti-glomerular basement membranes (anti-GBM) antibodies in the blood.[1][2]
Last updated: 9/17/2008

What causes Goodpasture syndrome? Is it genetic?

There is still much to learn about the cause of Goodpasture syndrome. It is thought that a combination of genetic and environment factors, such as cigarette smoke, inhaled hydrocarbons, and viruses play a role in the development of this autoimmune condition.[2]

In autoimmune disorders the body makes antibodies that attacks its own tissues. In the case of Goodpasture syndrome, antibodies form against a certain type of protein called collagen. The collagen is present in the alveoli (tiny air sacs in the lungs) and in the glomeruli (the filtering units of the kidney).[3] The antibody attack leads to bleeding in the air sacs and inflammation in the glomeruli of the kidney. Symptoms of the antibody attack may include shortness of breath, cough, and bloody sputum, blood and protein in the urine, and kidney failure.[3]

Genetic predisposition to Goodpasture syndrome involves the human leukocyte antigen (HLA) system.

The HLA system is involved in helping our immune system know the difference between "self" and "non-self." Human leukocyte antigens determine a person's tissue type. Each person has 3 pairs of major HLA antigens known as A, B, and DR. We inherit 1 set of A, B, and DR antigens from each of our parents (and pass 1 of our 2 sets on to each of our children).[4]

Below we have provided some facts regarding HLA antigens and Goodpasture syndrome:

  1. A certain HLA antigen, HLA-DR2, is found in 88% of patients with Goodpasture syndrome, as compared to 25-32% of those without it.[2]
  2. People with Goodpasture syndrome who have both HLA-B8 and HLA-DR2 tend to have a worse prognosis.[2]
  3. HLA-DR7 and HLA-DR1 is thought to confer some protection against developing Goodpasture syndrome.[2]
Last updated: 7/3/2008

How can I find a genetics professional in my area?

Genetics clinics are a source of information for individuals and families regarding genetic conditions, treatment, inheritance, and genetic risks to other family members. More information about genetic consultations is available from Genetics Home Reference. To find a genetics clinic, we recommend that you contact your primary healthcare provider for a referral.

The following online resources can help you find a genetics professional in your community:
Last updated: 6/5/2014

References
Other Names for this Disease
  • Anti-glomerular basement membrane antibody disease
  • Glomerulonephritis - pulmonary hemorrhage
  • Pulmonary renal syndrome
  • Rapidly progressive glomerulonephritis with pulmonary hemorrhage
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.