Publication year: 2022
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Pulmonary alveolar proteinosis (PAP) is a rare respiratory syndrome characterized by the accumulation of surfactant lipoproteins within the alveoli. Primary PAP is led by a granulocyte-macrophage colony-stimulating factor (GM-CSF) signaling disruption. Secondary PAP is associated with various diseases causing a reduction in function and/or the number of alveolar macrophages. Congenital PAP emerges as a consequence of corrupted surfactant production, due to mutations affecting surfactant transporter or lung development.