Goodpasture's Syndrome

In Goodpasture's syndrome, hemoptysis and rapidly progressive glomerulonephritis result from the deposition of antibodies against alveolar and glomerular basement membranes (GBM). This disorder can occur at any age but most commonly strikes men between ages 20 and 30. The prognosis improves with aggressive immunosuppressant and antibiotic therapy and with dialysis or kidney transplantation.

Causes

The cause of Goodpasture's syndrome is unknown. Although some cases have been associated with exposure to hydrocarbons or with type II hypersensitivity reaction, many patients have no precipitating events. The high incidence of human leukocyte antigen DR2 in patients with this disorder suggests a genetic predisposition.

Abnormal production and deposition of antibodies against GBM and alveolar basement membrane activate the complement and inflammatory responses, resulting in glomerular and alveolar tissue damage.

Symptoms

Symptoms include foamy, bloody, or dark colored urine, decreased urine output, cough with bloody sputum, difficulty breathing after exertion, weakness, fatigue , nausea or vomiting, weight loss, nonspecific chest pain and/or pale skin.

Diagnostic tests

Doctors will take a blood test and/or a kidney biopsy to diagnose Goodpasture's syndrome.

Treatment

Plasmapheresis can be used to remove antibodies, and immunosuppressants can be prescribed to suppress antibody production. Patients with renal failure can benefit from dialysis or kidney transplantation. Aggressive ultrafiltration helps to relieve pulmonary edema that may aggravate pulmonary hemorrhage. High-dose I.V. corticosteroids also help to control pulmonary hemorrhage.

Prevention

No known prevention of Goodpasture's syndrome exists. People should avoid glue sniffing and the siphoning gasoline. Stopping smoking, if a family history of renal failure exists, may prevent some cases. Early diagnosis and treatment may slow progression of the disorder.

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