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\u2019s syndrome (or Goodpasture syndrome).<\/p>\n
Goodpasture\u2019s 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\u2019s 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\u2019 blood vessels may occur because the immune system mistakes these healthy tissues as harmful.<\/p>\n
Signs of Goodpasture\u2019s 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:<\/p>\n
If the kidneys are affected, symptoms include:<\/p>\n
Although it\u2019s not fully understood why a person is susceptible to Goodpasture\u2019s 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:<\/p>\n
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\u2019s syndrome causes the immune system to produce anti-glomerular basement membrane antibodies, which attack the glomerular basement membrane \u2014 a part of the kidney that assists in filtering waste from the body. The kidney\u2019s glomeruli swell and become inflamed, leading to a condition called glomerulonephritis, the third leading cause of kidney disease.<\/p>\n
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.<\/p>\n
Symptoms of Goodpasture\u2019s 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.<\/p>\n
A lack of obvious symptoms can make it difficult for a doctor to diagnose Goodpasture\u2019s 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:<\/p>\n
The main focus of treatment is controlling the antibodies in the blood. Goodpasture\u2019s 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.<\/p>\n
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\u2019s blood is drawn at a time. It\u2019s placed in a centrifuge to separate the red and white blood cells from the plasma. The patient\u2019s blood cells are placed in a plasma substitute and returned to the body.<\/p>\n
While treatment for Goodpasture\u2019s syndrome is effective, autoimmune disease treatment suppresses the body\u2019s immune system and can leave a person susceptible to other infections, which can lead to serious illnesses and complications.<\/p>\n
Goodpasture\u2019s syndrome is a rare condition that causes the body\u2019s 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.<\/p>\n
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