Goodpasture Syndrome, Pulmonary

Key Features

• Idiopathic recurrent alveolar hemorrhage and rapidly progressive glomerulonephritis

• Mediated by anti-glomerular basement membrane antibodies

• Occurs mainly in men who are in their 30s and 40s

Clinical Findings

• Hemoptysis is the usual presenting symptom

• However, pulmonary hemorrhage may be occult

• Dyspnea, cough, hypoxemia, and diffuse bilateral alveolar infiltrates are typical features

• Iron deficiency anemia and microscopic hematuria are usually present

Diagnosis

• Based on characteristic linear IgG deposits detected by immunofluorescence in glomeruli or alveoli and on the presence of anti-glomerular basement membrane antibody in serum

Treatment

• Combinations of immunosuppressive drugs (initially methylprednisolone, 30 mg/kg intravenously over 20 minutes every other day for three doses, followed by daily oral prednisone, 1 mg/kg/day; with cyclophosphamide, 2 mg/kg by mouth per day) and plasmapheresis have yielded excellent results