Goodpasture Syndrome

Goodpasture syndrome is a rare autoimmune disease first discovered in 1919. It affects less than one in 500,000 people—according to some estimates, as few as one in two million people. The condition occurs when the immune system attacks the the glomeruli, or lining, of the lungs and kidneys. In the kidneys, the glomerular cells are part of the apparatus responsible for the first stage in filtering the blood, which is the function of the kidneys. When this cell layer is mistaken for an infectious agent, the immune system can cause significant damage.

In particular, this disease can result in patients coughing up blood. They will also experience shortness of breath and chest pain. Once the condition moves to the kidneys—or when it starts there—symptoms include swelling of the limbs, a puffy face, high blood pressure, or blood or protein in the urine, due to the disease harming kidney function. Goodpasture syndrome generally starts in the lungs first, and moves to the kidneys afterward. However, in 10 percent of cases it stays in the lungs, and about three times as often, it never affects the lungs at all.

It is not entirely clear what causes Goodpasture disorder. It is likely that some people are particularly susceptible to the illness, because there is a long list of possible triggers, things that damage the blood vessels in the lungs and draw the attention of the immune system, some of them quite common, but the disease remains rare.

These triggers include cigarette smoke, high oxygen levels, sepsis, cocaine, infections such as flu, metal dust, hydrocarbons, and organic solvents. There appears to be a genetic component, and certain gene mutations are themselves triggers. Recently, scientists found that the element bromine plays an important role in protecting the kidneys from the immune system.

It is important for Goodpasture syndrome to be treated quickly, because it can progress rapidly, and soon become fatal if let alone. Treatment generally means suppressing the immune system long enough for it to become acclimate again to the kidneys. Another treatment is blood purification, in which unwanted antibodies—to the body’s own organs—are removed. However, even once the disease is cured, the damage it has done remains.

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