Goodpasture’s Syndrome
In Goodpasture’s syndrome, hemoptysis and rapidly progressive glomerulonephritis result from the deposition of antibodies against the alveolar and glomerular
basement membranes (GBMs). Goodpasture’s syndrome may occur at any age but most commonly strikes males between ages 20 and 30. The prognosis improves with aggressive immunosuppressant and antibiotic therapy and with dialysis or kidney transplantation.
basement membranes (GBMs). Goodpasture’s syndrome may occur at any age but most commonly strikes males 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 type 2 influenza, many 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 the GBM and alveolar basement membrane activate the complement and inflammatory responses, resulting in glomerular and alveolar tissue damage.
Complications
Renal failure, requiring dialysis or transplantation, and severe pulmonary complications, such as pulmonary edema and hemorrhage, may occur.
Assessment
Initially, the patient with Goodpasture’s syndrome may complain of malaise, fatigue, and pallor—signs and symptoms associated with severe iron deficiency anemia.
Your assessment may reveal hematuria and signs of peripheral edema associated with renal involvement. You may also note signs of pulmonary involvement, such as dyspnea and hemoptysis, ranging from a cough with blood-tinged sputum to frank pulmonary hemorrhage. The patient may have had subclinical pulmonary bleeding for months or years before developing overt hemorrhage and signs of renal disease.
Diagnostic tests
Measurement of circulating anti-GBM antibodies by radioimmunoassay, as well as linear staining of GBM and alveolar basement membrane by immunofluorescence, confirms the diagnosis.
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