Goodpasture Syndrome

Goodpasture Syndrome

OVERVIEW

What is Goodpasture syndrome?

Goodpasture syndrome (or anti-GBM disease) is a rare, life-threatening autoimmune disease that affects the lungs and the kidneys. It happens when the immune system mistakenly attacks a protein called collagen because it recognizes it as a foreign substance. In Goodpasture syndrome, the body produces proteins (antibodies) that attach to the collagen in certain parts of the lungs and the kidneys. When they attach to the collagen, these antibodies cause severe inflammation and destruction of those tissues.

Symptoms of the disease include coughing up blood, difficulty breathing, fatigue and anemia. When the disease affects the kidneys, patients may have blood in the urine, swelling of the legs and high blood pressure. If the kidney disease is very severe, patients may notice that they are passing only small amounts of urine and may have nausea and vomiting as well. Treatments include medications and a procedure called plasmapheresis. This procedure removes plasma that contains these harmful antibodies and replaces it with healthy plasma.

Untreated, Goodpasture syndrome can cause inflammation of the kidneys (glomerulonephritis) and can lead to permanent kidney failure. The disorder can cause severe bleeding in the lungs, which is the main cause of death from Goodpasture syndrome.

How common is Goodpasture syndrome?

Goodpasture syndrome was first described in 1919 and is very rare. It is estimated that there are fewer than two cases per one million people. The syndrome affects men more often than women. It usually begins between ages 20-30 or after age 60.

Signs and symptoms

Diagram of a monomeric (one-unit) antibody

The anti–glomerular basement membrane (GBM) antibodies primarily attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains. 60 to 80% of those with the condition experience both lung and kidney involvement; 20-40% have kidney involvement alone, and less than 10% have lung involvement alone. Kidney symptoms usually include blood in the urine, protein in the urine, unexplained swelling of limbs or face, high amounts of urea in the blood, and high blood pressure. Lung symptoms usually antedate kidney symptoms and usually include: coughing up blood, chest pain (in less than 50% of cases overall), cough, and shortness of breath. Some other signs and symptoms that could be used to identify Goodpasture syndrome during a physical exam include an increased respiratory rate, cyanosis, crackles, hepatosplenomegaly, and hypertension.

What causes Goodpasture syndrome?

As a type of autoimmune disorder, Goodpasture syndrome causes antibodies produced by the immune system to attack collagen. Collagen is a protein that has many functions. It forms part of the structure of many tissues and helps blood clot, among others. Goodpasture syndrome attacks collagen in the glomerular basement membrane (GBM) of the kidneys. These anti-GBM antibodies also attack collagen in the lung’s air sacs, destroying the tissues and leading to bleeding and difficulty breathing.

We aren’t sure what causes Goodpasture syndrome. It may result from a combination of environmental factors and genetics. In people with Goodpasture syndrome, 8 to 90% have a type of human leukocyte antigen (HLA) called HLA-DR15. HLAs are proteins that help the immune system tell the difference between the body’s own tissues and foreign (or invading) substances. In people with HLA-DR15, the antigen doesn’t work like it should.

Sometimes, Goodpasture can develop after an infection, such as a cold or the flu. People who smoke, use cocaine or are exposed to metal dust and hydrocarbon chemicals (such as methane or propane) are more likely to develop Goodpasture syndrome. Scientists believe that these environmental factors may trigger the disorder in people who have HLA-DR15

How do healthcare providers diagnose Goodpasture syndrome?

Your provider will examine you and ask about your symptoms. To diagnose Goodpasture syndrome, your provider will order:

Blood tests to check how your kidneys are working and look for antibodies in your blood.

Urine test to check for blood or protein.

CT scan or chest X-ray to look for lung damage.

Bronchoscopy to examine your lungs.

Kidney biopsy, to look for glomerulonephritis (inflammation of the kidneys) and to check for anti-GBM antibodies in the kidney tissue.

MANAGEMENT AND TREATMENT

How do providers treat Goodpasture syndrome?

Treatments vary depending on the severity of the condition. Providers treat mild Goodpasture syndrome with medications, including:

Corticosteroids: Such as prednisone, to stop bleeding in the lungs.

Immunosuppressant drugs: Such as cyclophosphamide, to prevent the immune system from attacking the body’s own tissues.

Providers treat severe Goodpasture syndrome with these medications as well as a procedure called plasmapheresis. In this procedure, blood is removed through an IV (a vein in your arm) and the liquid part of the blood (plasma) is separated from the blood cells. This plasma, which contains the harmful anti-GBM antibodies, is replaced with healthy plasma from blood donors and returned to your body.

PREVENTION

Can I prevent Goodpasture syndrome?

You may not be able to prevent Goodpasture syndrome. But you can lower your risk by avoiding tobacco and chemicals that may trigger the disorder.

Healthcare providers can do a blood test to check for the HLA-DR15 antigen. If you know you have the HLA-DR15 antigen, ask your provider about regular checkups to monitor your health. If you smoke, talk to your provider about how to quit smoking.

If you’re frequently exposed to gasoline, kerosene, tar or asphalt, you have a higher risk of developing the condition. You should avoid these hydrocarbons if possible.

TYPE OF DOCTOR DEPARTMENT :- Nephrologist Specialist can diagnoses Goodpasture syndrome.