Pulmonary Alveolar Proteinosis

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

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

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

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