Goodpasture’s Syndrome
What is Goodpasture’s syndrome?
Goodpasture’s syndrome is a rare disease that can affect the lungs and kidneys. 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. 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.
Signs and Symptoms:
Goodpasture’s syndrome can cause people to cough up blood or feel a burning sensation when urinating. But its first signs may be vague, such as fatigue, nausea, difficulty breathing, or paleness. These signs are followed by kidney involvement, represented first by small amounts of blood in the urine, protein in the urine, and other clinical and laboratory findings.
Diagnosis:
To diagnose Goodpasture’s syndrome, doctors use a blood test, but a kidney or lung biopsy may be necessary to check for the presence of the harmful antibodies.
Treatment:
Goodpasture’s syndrome is treated with oral immunosuppressive drugs—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.
Source: National Kidney and Urologic Diseases Information Clearinghouse. Goodpasture's Syndrome. National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK). National Institutes of Health. NIH Publication No. 07–4558 : April 2007
