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For further information, see CMDT Part 9-23: Pulmonary Alveolar Proteinosis

Key Features

  • Lipoproteinaceous material accumulates within alveolar spaces

  • May be primary (idiopathic) or secondary to

    • Immunodeficiency

    • Hematologic malignancies

    • Inhalation of mineral dusts

    • Lung infection (eg, tuberculosis, viral infection)

Clinical Findings

  • Progressive dyspnea

  • Chest radiograph shows bilateral alveolar infiltrates and chest CT features ground-glass opacities

  • Spontaneous remission occurs in some; progressive respiratory insufficiency develops in others

  • Pulmonary infection with Nocardia or fungi may occur


  • While chest radiograph shows bilateral alveolar infiltrates, chest CT features characteristic "crazy-paving," which refers to ground-glass opacities with superimposed interlobular and intralobular septal thickening

  • Based on characteristic findings on bronchoalveolar lavage (BAL)

    • A milky appearance

    • Periodic acid-Schiff–positive lipoproteinaceous material, with typical clinical and radiographic features

  • In secondary disease, an elevated anti-granulocyte-macrophage colony-stimulating factor (anti-GM-CSF) titer in serum or BAL fluid is highly sensitive and specific

  • Biopsy is necessary in some cases


  • Therapy consists of periodic whole-lung lavage

  • Inhalational or subcutaneous GM-CSF may benefit patients who cannot tolerate or who do not respond to whole-lung lavage

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