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.
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 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.