A Case of Acquired Pulmonary Alveolar Proteinosis Successfully Treated with Whole Lung Lavage
AIR FORCE MEDICAL WING (59TH) LACKLAND AFB TX LACKLAND United States
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Pulmonary alveolar proteinosis PAP is a rare disorder characterized by the accumulation of surfactant within the alveoli. Surfactant phospholipids and proteins are produced by type II alveolar epithelial cells, and subsequently cleared by the alveolar macrophages. Cell signaling initiated by the granulocyte-macrophage colony-stimulating factor GM-CSF receptor is essential to the breakdown of surfactant. The acquired form of PAP most commonly involves circulating anti-bodies to GM-CSF, with resultant accumulation of surfactant.