TY - CHAP M1 - Book, Section TI - Pulmonary Alveolar Proteinosis A1 - Fitzpatrick, Meghan E. A1 - Prendergast, Niall T. A1 - Rivera-Lebron, Belinda A2 - Papadakis, Maxine A. A2 - McPhee, Stephen J. A2 - Rabow, Michael W. A2 - McQuaid, Kenneth R. PY - 2022 T2 - Current Medical Diagnosis & Treatment 2022 AB - Pulmonary alveolar proteinosis is a rare disease in which periodic acid-Schiff (PAS)-positive phospholipids accumulate within alveolar spaces. The condition may be primary (idiopathic) or secondary (occurring in immunodeficiency; hematologic malignancies; inhalation of mineral dusts; or following lung infections, including tuberculosis and viral infections). Progressive dyspnea is the usual presenting symptom, and chest radiograph shows bilateral alveolar infiltrates suggestive of pulmonary edema (eFigure 9–16). The diagnosis is based on demonstration of characteristic findings on BAL (milky appearance and PAS-positive lipoproteinaceous material) in association with typical clinical and radiographic features. In secondary disease, an elevated anti-GM-CSF (anti-granulocyte-macrophage colony-stimulating factor) titer in serum or BAL fluid is highly sensitive and specific. In some cases, transbronchial or surgical lung biopsy (revealing amorphous intra-alveolar phospholipid) is necessary. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1186012944 ER -