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DESCRIPTION
Goodpasture's Syndrome is an uncommon disease that affects both the kidneys and the lungs. Also called anti-glomerular basement antibody disease, it is an autoimmune disease - a condition in which the body's own defense system reacts against some part of the body itself. When the immune system is working normally, it creates antibodies to fight off germs. In Goodpasture's syndrome, the immune system makes antibodies that attack the lungs and kidneys. Why this happens is not fully understood.
SYMPTOMS
If you have the disease, you will usually experience these symptoms:
- Bleeding from the lungs.
- Coughing up of blood.
- Inflammation of the kidney (glomerulonephritis).
- A burning sensation when urinating.
The first signs may be vague, such as fatigue, nausea, difficulty breathing, or paleness. These signs are followed by kidney involvement with the above symptoms of blood in the urine, protein in the urine, and other clinical and laboratory findings.
CAUSES
Researchers have identified a number of possible causes, among them the presence of an inherited component; exposure to certain chemicals, including hydrocarbon solvents and the weed killer Paraquat; and viral infections.
Usually, you have signs and symptoms because your body is making antibodies that hurt the lining of your lungs and kidneys. It is not known why your antibodies begin attacking your own body. Usually, they only do helpful things such as fight infections.
This problem is most common in people between the ages of 15 and 35 or after 55. It is not contagious and it is more common in men and Caucasians. Sometimes, you will have these signs and symptoms with other diseases, such as lupus erythematosus or Wegener's granulomatosis.
COMPLICATIONS
The lung disease may cause life threatening bleeding but does not usually cause long-term harm to your lungs. However, the harm done to your kidneys can result in kidney failure. You may need either dialysis or a new kidney (kidney transplant). If your kidney is being harmed, you may:
- Look pale and feel tired.
- Have blood or protein in your urine.
- Have back pain below your ribs.
- Go to the bathroom more often, especially at night.
- Have burning or difficulty when going to the bathroom.
- Have swelling of your hands and feet.
- Have high blood pressure.
DIAGNOSIS
Your health care provider will check you thoroughly. There is a specific blood test that can show if you have this harmful antibody. Your health care provider may also do a lung or kidney biopsy (take a small piece of kidney tissue to look at under a microscope) to see if the antibody is present in the tissue.
TREATMENTS
Early diagnosis and treatment are the best ways to prevent kidney damage. Your health care provider will give you medicines that will fight the harmful antibodies. Sometimes, the health care provider may suggest that you undergo a special blood filtering process (plasmapheresis) to remove harmful antibodies.
Goodpasture's syndrome is treated with oral immunosuppressive drugs, such as cyclophosphamide and corticosteroids, to keep the immune system from making antibodies. Corticosteroid drugs may be given intravenously to control bleeding in the lungs. A process called plasmapheresis may be helpful and necessary to remove the harmful antibodies from the blood. In plasmapheresis, a patient's blood is drawn, about 300 ml at a time, and placed in a centrifuge to separate the red and white blood cells from the plasma. The cells are then placed in a plasma substitute and returned to the body. This procedure is usually done in combination with immunosuppressive drug treatment.
Goodpasture's syndrome may last only a few weeks or as long as 2 years. Bleeding in the lungs can be very serious and even fatal in some cases. But Goodpasture's syndrome does not usually lead to permanent lung damage. Damage to the kidneys, however, may be long-lasting. If the kidneys fail, dialysis to remove waste products and extra fluid from the blood, or kidney transplantation, may become necessary.
PROGNOSIS
Usually, your body will only make the antibodies for a short time anywhere from a few weeks to two years. Once this stops, you should not have any more problems with your lungs. However, your kidneys may have been harmed a little or a lot.
Unfortunately, by the time you see your health care provider, your kidneys may already be damaged badly. It is possible that you may need dialysis or a kidney transplant. Before you can get a new kidney, your health care provider will check to be sure you do not still have any of the harmful antibodies. The medicines you need to take when you get a new kidney also help to prevent your body from making the harmful antibodies.
DIET & NUTRITION
Sometimes you will be asked to change your diet to slow down the loss of your kidney function. Your health care provider may also want you to lower your cholesterol level and/or add fiber to your diet. Your health care provider and your dietitian will help you with these changes.
SELF-CARE MEASURES
Other measures that may help to keep you healthy are exercise and reducing stress in your life. It is important not to smoke because smoking can increase bleeding into your lungs.
GENETICS & FAMILY
Goodpasture's Syndrome usually does not run in families. There have been a few cases where two members of a family had the disease.
RESEARCH
Currently, there is a lot of interest in finding out what causes the making of harmful antibodies. This work may lead to new drugs that are more helpful.
Research is being done to discover genetic factors that are important in the disease. Also, research is looking at better ways to treat the bleeding that happens with the lung disease.
MORE INFORMATION
National Kidney Foundation Affiliate Office:
Massachusetts, Rhode Island, New Hampshire, & Vermont11 Vanderbilt Avenue
If you would like to become a volunteer and find out more about what's happening where you live, contact your local NKF Affiliate Offices.
Norwood, MA 02062
Phone: (781) 278-0222
Fax: (781) 278-0333
Website: www.kidneyhealth.org
Email: tthompson@kidneyhealth.org
If you would like more information, please call 1-800-622-9010 or write the National Kidney Foundation, 30 East 33rd Street, New York, NY 10016.
American Kidney Fund
6110 Executive Boulevard, Suite 1010
Rockville, MD 20852
Phone: 1-800-638-8299
Email: helpline@kidneyfund.org
Internet: www.kidneyfund.org
National Kidney Foundation, Inc.
30 East 33rd Street
New York, NY 10016
Phone: 1-800-622-9010 or 212-889-2210
Internet: www.kidney.org
National Kidney and Urologic Diseases Information Clearinghouse
3 Information Way
Bethesda, MD 20892-3580
Phone: 1-800-891-5390
Fax: 703-738-4929
Email: nkudic@info.niddk.nih.gov
Internet: www.kidney.niddk.nih.gov/
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