Guide CidpUsa
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Autoimmune role in Renal Diseases
Goodpasture 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.
What are the symptoms of Goodpasture's syndrome?
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.
How is Goodpasture's syndrome diagnosed?
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.
How is
Goodpasture's syndrome treated?
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
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 .Rituximab is a B-cell depleting agent that has been used with success in a variety of autoimmune diseases. There are now a small number of cases that have been reported in the literature describing the use of rituximab for the treatment of anti-GBM disease,
transplantation, may become necessary.