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Pulmonary alveolar proteinosis (PAP) is a rare disease, certainly underdiagnosed, characterised by the intra-alveolar accumulation of a milky fluid rich in phospholipids and lipoproteins derived from alveolar surfactant, positive in periodic acid-Schiff staining. The alveolar macrophage plays a major role in the pathogenesis of PAP, and its role in the turn-over of alveolar surfactant is being altered by various mechanisms. More than 90% of cases of PAP are primary autoimmune, characterised bydoi:10.2478/pneum-2019-0029 fatcat:gc55v62in5hulgakrryx6royci