Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterised by intra-alveolar accumulation of surfactant components and cellular debris, with minimal interstitial inflammation or fibrosis.
Since surfactant accumulates abnormally, a disturbance in the normal pathway of surfactant production, metabolism, recycling or degradation has been postulated.
This disease has a variable clinical course: from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infections.
PAP leading to pulmonary fibrosis is rarely seen. (18309702)
References
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