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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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11/14/2010

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