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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 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

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