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Key Features

  • Phospholipid accumulation within alveolar spaces

  • May be primary (idiopathic) or secondary to immunodeficiency, hematologic malignancies, or inhalation of mineral dusts, or may occur after lung infections

Clinical Findings

  • Progressive dyspnea

  • Chest radiograph shows bilateral alveolar infiltrates suggestive of pulmonary edema

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

  • Pulmonary infection with Nocardia or fungi may occur

Diagnosis

  • Based on characteristic findings on alveolar lavage, a milky appearance, and periodic acid-Schiff–positive lipoproteinaceous material, with typical clinical and radiographic features

  • In secondary disease, an elevated anti-GM-CSF titer in serum or bronchoalveolar lavage (BAL) fluid is highly sensitive and specific

  • Biopsy is necessary in some cases

Treatment

  • 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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