Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease caused by problems with: surfactant metabolism, alveolar macrophage function, granulocyte-macrophage colony stimulating factor, or an unknown etiology. These can lead to the deposition of surfactant and apoproteins within the alveoli. Symmetric alveolar opacities in a classic “bat-wing” distribution are characteristic chest x-ray findings of PAP which also explains the issues patients have with gas exchange.

Treatment options range from supplemental oxygen for mild symptoms to total lung lavages for severe, intractable symptoms. A total lung lavage requires general anesthesia with lung isolation (e.g., a double-lumen endotracheal tube) to facilitate the instillation of crystalloid solution, mechanical chest wall agitation, and subsequent suctioning of protein-containing fluid. Based on the etiology of PAP, other options include GM-CSF, immunological, and plasma exchange.

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