What is Goodpasture's Syndrome
Goodpasture's Syndrome is a rare autoimmune disorder that affects the lungs and the kidneys. It is also known by the names anti-glomerular basement antibody disease and anti-glomerular basement membrane disease (or anti-GBM disease).
Normally, the body's immune system produces antibodies that fight off infection and illness. With an autoimmune disease such as Goodpasture's Syndrome, the body's antibodies actually attack healthy cells. In the case of Goodpasture's Syndrome, the antibodies attack the lungs and kidneys. Researchers still don't know why.
Specifically, the antibodies attack the alveoli (air sacs) and capillaries of the lungs, and the filtering apparatus of the kidneys. The body reacts to the antibodies by swelling and producing inflammation. This inflammation interferes with normal lung and kidney function.
Research has shown that certain people are genetically predisposed to producing these attacking antibodies. Under certain conditions such as exposure to tobacco smoke, inhalation of gasoline or paint fumes, or the presence of a viral upper respiratory infection, the body will produce antibodies that will not only attack the infection, but also healthy cells.
In Europe it is estimated that one in every millionth person is diagnosed with Goodpasture's Syndrome in a year, primarily in white populations. It is even more rare in other races. Cases have been reported in patients from four to 80 years old, but it is most common in people between 18 to 30 and 50 to 65 years old.
Symptoms of Goodpasture's Syndrome
Over time the lungs (and kidneys) will start to bleed or hemorrhage. While there are other conditions that produce this particular symptom, a Goodpasture's Syndrome diagnosis is usually reserved "when the immune system attacks a particular molecule that is found in the kidney and the lung" known as the Goodpasture antigen.
Usually the lung symptoms will appear before the kidney symptoms. Those symptoms include:
- coughing up blood
- burning sensation when urinating
- fatigue
- nausea
- difficulty breathing
- pale coloring
- blood in the urine
- protein in the urine (on testing)
While diagnosis is usually made through a blood test, lung or kidney biopsy may be necessary to test for the presence of antibodies. Chest X-rays will show white patches in the lungs due to bleeding.
Some patients may experience severe symptoms and require extensive intervention, while others may only experience a dry cough and minor breathlessness. Some patients have reported mild symptoms for years prior to diagnosis. Because of the bleeding whether long or short-term, anemia is also usually present.
How to treat Goodpasture's Syndrome
Treatment combines several methods to address both the lung and the kidney issues with the hope of preventing permanent damage. This treatment includes a cocktail of immunosuppressing drugs (usually prednisone and cyclophosphamide), and a procedure known as plasma exchange or plasmapheresis. In plasmapheresis, blood is drawn and placed in a centrifuge to separate the red and white blood cells from the plasma. The plasma is replaced or substituted by a purified protein solution. Then the blood is returned to the body. Some medical centers use immunoabsorption procedures to remove the antibodies.
Plasmapheresis is preformed for two weeks, while the cyclophosphamide treatment continues for three months. If the full course of treatment is done, recurrence of the disease are rare, though if symptoms are severe enough or not treated in a timely and effective manner, damage to the lungs and kidneys may mean ongoing treatment to maintain quality of life.
Sources:
http://kidney.niddk.nih.gov (National Institute of Diabetes and Digestive and Kidney Diseases)
www.merck.com
www.edren.org (Edinburgh Renal Unit)
www.peoples-health.com