Pulmonary alveolar proteinosis secondary to chronic chlorine occupational inhalation

Abstract

Pulmonary Alveolar Proteinosis (PAP) is one of the “orphan diseases” of the lung, whose peculiarity consists in an intra-alveolar accumulation of a lipo-proteinaceous material related to surfactant, which interferes with gas exchange, presenting with symptoms and signs varying in intensity. Initially described by Rosen, Castleman and Liebow in 1958, its frequency is very low: both Diksen and Ben Dov estimate an incidence in 0.37 cases/100,000 people and prevalence in 3.7/1,000,000 inhabitants.1‒3