Goodpastures syndrome affects the lungs and kidneys. Coughing up blood and rapidly progressive glomerulonephritis follow the deposition of antibody against the alveolar (lung) and glomerular (kidney) basement membranes. This syndrome may occur at any age but is most common in men between ages 20 and 30. The prognosis improves with aggressive immunosuppressants and antibiotic therapy and with dialysis or kidney transplantation.
What Causes it?
The cause of Goodpastures syndrome is unknown. Although some cases have been associated with exposure to hydrocarbons or type 2 influenza, many have no precipitating events. Evidence suggests a genetic predisposition to this syndrome. Abnormal production and deposition of the antibody against glomerular and alveolar basement membranes damages alveolar tissues.
What are its Symptoms?
Goodpastures syndrome may initially cause malaise, fatigue, and pallor associated with severe iron deficiency anemia. Pulmonary findings range from slight shortness of breath and cough with blood-tinged sputum to frank pulmonary hemorrhage. Sub clinical pulmonary bleeding may precede overt hemorrhage and kidney disease by months or years. Usually, such findings are more subtle, although some people note blood in the urine and peripheral swelling.
How is it Diagnosed?
Confirmation of Goodpastures syndrome requires tests to distinguish Goodpastures from other lung-kidney syndromes.
Chest X-rays and removal and analysis of lung and kidney tissue. along with blood and urine tests, can help confirm the diagnosis.
How is it Treated?
Treatment aims to remove antibody by plasmapheresis (processing of formed blood elements) and to suppress antibody production with immunosuppressants. A person with kidney failure may benefit from dialysis or transplantation. Aggressive ultrafiltration helps relieve pulmonary edema that may aggravate pulmonary hemorrhage. Highdose intravenous corticosteroids also help control pulmonary hemorrhage .
What can a person with Goodpastures syndrome do?
• Take steps to conserve your energy. • Learn what signs and symptoms to expect and how to relieve them.
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