Goodpasture’s Syndrome

In 1919, while studying the influenza pandemic, Ernest Goodpasture, an American pathologist and physician, recognized a disease affecting the lungs and kidneys. His discovery is now known as Goodpasture’s syndrome (or Goodpasture syndrome).

Goodpasture syndrome (GPS) is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. It is thought to attack the alpha-3 subunit of type IV collagen, which has therefore been referred to as Goodpasture’s antigen. Goodpasture syndrome may quickly result in permanent lung and kidney damage, often leading to death. It is treated with drugs that suppress the immune system such as corticosteroids and cyclophosphamide, and with plasmapheresis, in which the antibodies are removed from the blood.

Signs and symptoms of Goodpasture’s syndrome

The antiglomerular basement membrane (GBM) antibodies primarily attack the kidneys and lungs, although, generalized symptoms like malaise, weight loss, fatigue, fever, and chills are also common, as are joint aches and pains. 60 to 80% of those with the condition experience both lung and kidney involvement; 20-40% have kidney involvement alone, and less than 10% have lung involvement alone.

Lung symptoms usually antedate kidney symptoms and usually include: coughing up blood, chest pain (in less than 50% of cases overall), cough, and shortness of breath.

Kidney symptoms usually include blood in the urine, protein in the urine, unexplained swelling of limbs or face, high amounts of urea in the blood, and high blood pressure.

Causes of Goodpasture’s syndrome

Although it’s not fully understood why a person is susceptible to Goodpasture’s syndrome, research has shown the condition affects males more frequently than females and most often affects people between the ages of 18-30 and 50-65. Some possible causes are:

  • Genetics
  • Viral infections
  • Exposure to hydrocarbon solvents or paraquat (a weed killer)

Goodpasture’s syndrome and the kidneys

Each kidney contains a million tiny clusters of blood vessels that filter waste from the body. Each cluster is called a glomerulus (glomeruli when referring to more than one).

Goodpasture’s syndrome causes the immune system to produce anti-glomerular basement membrane antibodies, which attack the glomerular basement membrane — a part of the kidney that assists in filtering waste from the body. The kidney’s glomeruli swell and become inflamed, leading to a condition called glomerulonephritis, the third leading cause of kidney disease.

When the glomeruli are inflamed, they cannot maintain the chemical balance of the body, and waste builds up in the blood. This results in high blood pressure and causes more kidney damage, which may lead to end stage renal disease (ESRD), the final stage of kidney disease.

Kidney failure is barely noticeable until more than 80% of kidney function is lost. Symptoms of kidney failure include:

  • Loss of appetite
  • Malaise
  • Breathlessness
  • Edema
  • High blood pressure
  • Hematuria (blood in the urine)
  • Reduced glomerular filtration rate (GFR)
  • Large amounts of protein in urine
  • Drop in urine production

Goodpasture’s syndrome and the lungs

Symptoms of Goodpasture’s syndrome may be as mild as a dry cough and moments of breathlessness. The lung infection can progress slowly or very rapidly. An extreme symptom is coughing up blood. Depending on the severity of the disease, bleeding of the lungs can last from two weeks to two years. People can develop anemia from blood loss due to lung hemorrhaging. Smokers and those who have lung damage from a previous infection or exposure to fumes are at greater risk of lung hemorrhaging.

Diagnosing Goodpasture’s syndrome

There is a specific blood test that can show if you have harmful antibodies attacking your lungs and kidneys.

A doctor can also test your blood and urine to find out if you have kidney disease. A urine test can check for the amount of protein, blood and other things to indicate kidney damage. A blood test for serum creatinine can be used to calculate glomerular filtration rate (GFR), which tells how well your kidneys are filtering wastes from the blood.

Your doctor may also perform a kidney biopsy to see if the kidney tissue is damaged by the antibody.

Your lungs may also be checked for damage with a chest X-ray and lung biopsy.

A lack of obvious symptoms can make it difficult for a doctor to diagnose Goodpasture’s syndrome. A physical exam, blood tests and urinalysis can reveal high blood pressure, fluid imbalance, abnormal heartbeat, lung distress, or blood and protein in the urine. Further tests include:

  • Anti-glomerular basement membrane test
  • Arterial blood gas test
  • Blood urea nitrogen (BUN) test
  • Chest X-ray
  • Kidney biopsy
  • Lung biopsy

Treating Goodpasture’s syndrome

Early diagnosis and treatment are the best ways to prevent kidney damage. Your doctor may give you medications to help slow the immune system and try to keep it from making harmful antibodies. You may also be given medication to control fluid buildup or high blood pressure. The doctor may suggest that you undergo a special blood filtering process (plasmapheresis) to remove harmful antibodies.

The main focus of treatment is controlling the antibodies in the blood. Goodpasture’s syndrome responds well to cyclophosphamide, an immunosuppressive drug. Corticosteroids may be prescribed to help control bleeding in the lungs, and antibiotics may be prescribed to treat infection.

Plasmapheresis is a procedure that cleanses the blood plasma (the fluid in which the red blood cells circulate) by removing the harmful antibodies. Approximately 300 mL of the patient’s blood is drawn at a time. It’s placed in a centrifuge to separate the red and white blood cells from the plasma. The patient’s blood cells are placed in a plasma substitute and returned to the body.

While treatment for Goodpasture’s syndrome is effective, autoimmune disease treatment suppresses the body’s immune system and can leave a person susceptible to other infections, which can lead to serious illnesses and complications.

Summary

Goodpasture’s syndrome is a rare condition that causes the body’s immune system to attack healthy lung and kidney tissue. In some cases, a person will cough up blood and notice discomfort while urinating. Bleeding in the lungs is extremely dangerous, but with proper treatment the lungs can recover with little damage. Early detection is especially important for the kidneys because they are unable to recover from harm.

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