Goodpasture syndrome is a disorder with an autoimmune component that can cause bleeding in the lungs and long-term damage to the kidneys. If untreated, it can quickly progress to kidney failure and other complications. The condition is most common in people between ages 20 and 30 and those over 60. Anyone can develop this syndrome, but it is more often diagnosed in men.
Glomerulonephritis is one component of Goodpasture syndrome. This kidney disease causes inflammation in the tiny filter cells in the called glomeruli. Glomerulonephritis can result from many things, including strep throat, lupus, and Wegener's disease. Early diagnosis is essential to determine the cause and provide prompt and proper treatment.
Another component to Goodpasture syndrome is a bleeding in the lungs called pulmonary alveolar hemorrhage. This condition causes widespread damage to small vessels in the lungs, which causes the air sacs or alveoli to fill with blood. If enough alveoli are affected, the lungs cannot exchange oxygen effectively. Many things can cause alveolar hemorrhage, including adenovirus, cytomegalovirus, malaria, H1N1, and staph infections, especially in those with weakened immune systems. Quick diagnosis and treatment are essential.
Goodpasture syndrome and Goodpasture disease are used interchangeably, but there is a slight distinction between them. Goodpasture disease refers to cases with pulmonary hemorrhage, glomerulonephritis, and anti-GBM antibodies. Goodpasture syndrome refers to cases with pulmonary hemorrhage and glomerulonephritis, but it is not caused by anti-GBM antibodies. Collectively, these conditions are known as anti-GBM antibody disease.
The causes of Goodpasture syndrome are not fully understood. It seems to develop due to a combination of genetic predisposition and environmental factors. Anti-bodies attack the collagen in the kidneys and lungs, but many environmental factors increase the risk of developing Goodpasture syndrome, including smoking, infection, inhaling hydrocarbons, and inhaling cocaine.
Many early symptoms of Goodpasture syndrome are non-specific, including fatigue, weakness, nausea, and vomiting. Signs that the lungs are affected are difficulty breathing and coughing, often coughing up blood. Kidney involvement presents with bloody or foamy urine, high blood pressure, or ankle swelling. The symptoms of Goodpasture syndrome usually progress quickly.
People with Goodpasture syndrome initially present with signs of kidney damage or rapidly progressing glomerulonephritis. These symptoms are non-specific as they have many causes, so the doctor will do a complete workup. Pulmonary symptoms may be present along with kidney symptoms or shortly after. Younger individuals usually develop lung and kidney symptoms simultaneously, while people over 50 often present with a milder case involving glomerulonephritis with no lung issues.
A kidney biopsy will provide a definitive diagnosis because it identifies the cause and extent of the damage and provides an idea of how long the kidneys have been involved. Although confirming the diagnosis with a biopsy is necessary, treatment can and should begin before this, for the best results.
Other diagnostic tests help determine the extent of the damage caused by Goodpasture syndrome. Chest x-rays and pulmonary function tests provide information about the condition of the lungs, while renal function tests and urinalysis evaluate kidney function. A complete blood cell count may reveal low red blood cells resulting from alveolar hemorrhage or increased white blood cells, which indicate an inflammatory response.
Treatment varies depending on timing and severity. Medication that suppresses the immune system can curb the production of anti-GBM antibodies and reduce inflammation. Treatment also includes plasmapheresis or plasma exchange, a therapy that uses a machine to filter the blood and remove antibodies. Plasmapheresis is usually done for a few weeks. Depending on how the patient responds, they may need to take immunosuppressive drugs for several months after treatment.
Goodpasture syndrome may reoccur in some cases, even after successful treatment. With effective therapies and early treatment, the five-year survival rate is more than 80 percent. Less than 30 percent of those who have recovered from Goodpasture syndrome need long-term dialysis. In severe cases, the patient may need a kidney transplant.
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