Goodpasture's syndrome
Definition of Goodpasture's syndrome
Goodpasture's syndrome is a rare disease that can affect the lungs and kidneys. 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 uncertain. A combination
of factors has been implicated, among them the presence of an inherited component and
exposure to certain chemicals.
Goodpasture's syndrome can cause people to cough up blood or feel a burning sensation
when urinating. But its first signs may be vague, like fatigue, nausea, dyspnea
(difficulty breathing), or pallor. 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.
To diagnose Goodpasture's syndrome, doctors use a blood test, but a kidney biopsy (or a
lung biopsy) may be necessary to check for the presence of the harmful antibody.
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 (PLAZ-ma-fer-REE-sis) may be helpful and necessary to remove the harmful
antibodies from the blood; this is usually done in combination with the 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 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.
Description of Goodpasture's syndrome
The disorder is characterized by deposits of antibodies in the membranes of both the
lung and kidneys, causing both inflammation of kidney glomerulus (glomerulonephritis) and
lung bleeding. It is typically a disease of young males.
Causes & symptoms of Goodpasture's syndrome
The exact cause is unknown. It is an autoimmune disorder; that is, the immune system is
fighting the body's own normal tissues. Sometimes the disorder is triggered by a viral
infection, or by the inhalation of gasoline or other hydrocarbon solvents. An association
also exists between cigarette smoking and the syndrome.
Symptoms include foamy, bloody, or dark colored urine, decreased urine output, cough
with bloody sputum, difficulty breathing after exertion, weakness, fatigue, nausea or
vomiting, weight loss, nonspecific chest pain and/or pale skin.
Diagnosis of Goodpasture's syndrome
The clinician will perform a battery of tests to confirm a diagnosis. These tests
include a complete blood count (CBC) to confirm anemia, iron levels to check for blood
loss and blood urea nitrogen (BUN) and creatinine levels to test the kidney function. A
urinalysis will be done to check for damage to the kidneys. A sputum test will be done to
look for antibodies. A chest x ray will be done to assess the amount of fluid in the lung
tissues. A lung needle biopsy and a kidney biopsy will show immune system deposits.
Treatment of Goodpasture's syndrome
Treatment is focused on slowing the progression of the disease. Treatment is most
effective when begun early, before kidney function has deteriorated to a point where the
kidney is permanently damaged, and dialysis is necessary. Corticosteroids, such as
prednisone, or other anti-inflammatory medications may be used to reduce the immune
response. Immune suppressants such as cyclophosphamide or azathioprine are used
aggressively to reduce immune system effects.
A procedure whereby blood plasma, which contains antibodies, is removed from the body
and replaced with fluids or donated plasma (plasmapheresis) may be performed daily for two
or more weeks to remove circulating antibodies. It is fairly effective in slowing or
reversing the disorder. Dialysis to clean the blood of wastes may be required if kidney
function is poor. A kidney transplant may be successful, especially if performed after
circulating antibodies have been absent for several months.
Prognosis of Goodpasture's syndrome
The probable outcome is variable. Most cases progress to severe renal failure and
end-stage renal disease within months. Early diagnosis and treatment makes the probable
outcome more favorable.
Prevention of Goodpasture's syndrome
No known prevention of Goodpasture's syndrome exists. People should avoid glue sniffing
and the siphoning gasoline. Stopping smoking, if a family history of renal failure exists,
may prevent some cases. Early diagnosis and treatment may slow progression of the disorder
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