Goodpasture Syndrome:: Causes, Symptoms, Diagnosis, Treatment

Goodpasture Syndrome:: Causes, Symptoms, Diagnosis, Treatment


What is Goodpasture Syndrome?


Goodpasture 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. No one knows why, in Goodpasture syndrome, the immune system makes antibodies that end up attacking the lungs and kidneys. A combination of factors has been implicated, and among these is the presence of an inherited component.

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What Causes Goodpasture Syndrome?


While the exact cause of Goodpasture syndrome is unknown, certain behaviors and environmental factors are believed to put people at higher risk. Certain respiratory infections may trigger the disease. Exposure to hydrocarbon fumes, metallic dust, tobacco smoke, or certain illegal substances such as cocaine may also increase risk.

Scientists believe the immune system attacks lung and kidney tissue because the condition fools body defenses into thinking the parts are foreign to the body itself.

According to the National Institutes of Health (NIH), Goodpasture syndrome affects men eight times more often than women and occurs most commonly in early adulthood. (NIH, 2011) The National Kidney Foundation (NKF) reports that the disease is more common in Caucasians than in other races.

Goodpasture Syndrome Symptoms & Diagnosis


What Are the Symptoms of Goodpasture Syndrome?


Symptoms may start out slowly, gradually affecting the lungs and then the kidneys. Other times they may progress rapidly, becoming severe in a matter of days. Initial symptoms may include:
  • fatigue, weakness, or lethargy
  • nausea and/or vomiting
  • loss of appetite
  • unhealthy, pale appearance
If the disease moves to affect the lungs, the following symptoms may occur:
  • dry cough or coughing up blood
  • shortness of breath or difficulty breathing
Sometimes symptoms affecting the lungs can become life-threatening, particularly if there is a lot of bleeding.
If the disease affects the kidneys, it may cause:
  • burning sensation during urination
  • blood in the urine or foamy urine
  • swelling of the hands and feet
  • high blood pressure
  • back pain below the ribs

These signs are followed by kidney involvement, represented first with small amounts of blood in the urine, protein excretion in the urine, and other clinical and laboratory findings.

To diagnose Goodpasture syndrome, doctors can now use a blood test, but a kidney biopsy may be necessary to check for the presence of the harmful antibody.

A chest X-ray may show the presence of abnormal white patches that indicate bleeding in the lungs.

A kidney biopsy may reveal changes that indicate the presence of Goodpasture syndrome. During this test, a sample of tissue from the kidney is removed and sent to a lab for testing. Lab technicians will look for the presence of antibodies or other abnormal cells to help your doctor make a diagnosis.

How Is Goodpasture Syndrome Treated?


Treatments involve medications that slow down your immune system. These may include one or more of the following:
  • immunosuppressive drugs to keep your immune system from making the antibodies that damage your lungs and kidneys. Cyclophosphamide is one example.
  • corticosteroids like prednisone to help control bleeding in your lungs. These medications also suppress your immune system.
A treatment called plasmapheresis may be used to help filter out harmful antibodies in your blood. During this procedure, blood is withdrawn and the liquid portion (plasma) is removed and replaced. The "cleaned" blood is transferred back into your body.

Other treatments depend on the individual’s age, overall health, and the severity of the disease. Doctors may prescribe additional medications to control fluid build-up and high blood pressure. In addition to medication, dietary changes such as cutting down on salt intake can help control swelling and blood pressure.

Goodpasture 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 syndrome does not usually lead to permanent lung damage. Damage to the kidneys, however, may be long-lasting. If the kidneys fail, kidney transplantation or dialysis therapy to remove waste products and extra fluid from the blood may become necessary.

Prevention


Never sniff glue or siphon gasoline with your mouth, which expose the lungs to hydrocarbon solvents and can cause the disease.


Complications


Acute respiratory failure, acute renal failure and chronic renal failure are the most common complications.

  • Pulmonary haemorrhage with respiratory failure is the most common cause of death.
  • Renal failure in 90%.
  • The circulating antibodies clear within 8 weeks, but an early relapse within 2 months may occur when circulating antibodies are still present. This typically presents as alveolar haemorrhage.
  • The risk factors for relapse include infection, volume overload, and cigarette smoking.
  • Pneumocystis jirovecii pneumonia has an annual incidence of 1% but is a potentially deadly complication of immunosuppressive therapy in patients with Goodpasture's syndrome. Prophylaxis with co-trimoxazole may be useful.
If Goodpasture's syndrome occurs in pregnancy it may produce hypertension and associated intrauterine growth restriction requiring premature delivery. Both mother and baby are at risk.