Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 9-23: Pulmonary Alveolar Proteinosis + Key Features Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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