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’s syndrome is an unusual autoimmune disorder that can cause lung disease and chronic kidney disease (CKD). A healthy immune system produces antibodies to fight off germs and bacteria. In Goodpasture’s syndrome, the immune system makes antibodies that attack healthy protein called collagen in the air sacs of the lungs and the glomerular basement membrane of the kidneys. The attacking substances are called anti-glomerular basement membrane antibodies. Bleeding in the lungs and inflammation in the kidneys’ blood vessels may occur because the immune system mistakes these healthy tissues as harmful.

Signs and symptoms of Goodpasture’s syndrome

Signs of Goodpasture’s syndrome can occur over a few days or a few years. The first signs may be paleness, fatigue, loss of appetite, nausea and weakness. As the disease advances, the lung symptoms are:

Dry cough
Coughing up blood
Difficulty breathing

If the kidneys are affected, symptoms include:

Burning sensation during urination
Blood in the urine
High blood pressure
Edema (swollen body parts)
Low urine production

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

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

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.