Wednesday, December 30, 2009

Goodpasture's Syndrome

Goodpasture’s syndrome is a disease which affects the kidneys and lungs. It is characterized by glomerulonephritis and hemorrhaging of the lungs. The Goodpasture's syndrome is also called anti-glomerular basement membrane disease, which consists of progressive kidney failure which develops in days to weeks along with lung disease with cough, shortness of breath, and blood in the sputum. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease triggered when the patient’s immune system attacks Goodpasture antigen (a type II hypersensitivity reaction), which is found in the kidney and lung, and in time, causing damage to these organs. The disease bears the name of the American pathologist Dr. Ernest Goodpasture, whose 1919 description is regarded as the first report on the existence of the condition.

People who suffer from Goodpasture syndrome may feel a burning sensation when urinating. Other symptoms include fatigue or listlessness, nausea, difficulty breathing, or paleness. These symptoms are followed by small amounts of blood and protein in the urine. Aside from the presence of an inherited component, other possible causes are exposure to certain chemicals, including hydrocarbon solvents and the weed killer Paraquat, and viral infections. In the cases arising out of breathing hydrocarbon solvents, the immune system may attack organs or tissues because it mistakes them for viruses or foreign chemicals. This immune system's faulty response provokes bleeding in the air sacs and inflammation in the kidney's filtering units.

Goodpasture’s syndrome responds well to treatment with corticosteroids and immunosuppressants. These drugs dampen the body's normal immune response and the patient may become more susceptible to infections. The concentration of anti-GBM antibodies in the blood may be reduced by apheresis to remove blood plasma and replace a portion of the plasma with an isotonic salt and protein solution. This course of treatment usually lasts between three and six months.