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 presence of anti-glomerular basement membrane antibody in serum

Treatment

• Plasmapheresis plus immunosuppressive therapy is recommended, rather than immunosuppressive therapy alone

• Immunosuppression with the combination of corticosteroids and cyclophosphamide is recommended