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For further information, see CMDT Part 9-28: Alveolar Hemorrhage Syndromes

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

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