Pulmonary alveolar proteinosis syndrome

T Suzuki, BC Trapnell - Clinics in chest medicine, 2016 - chestmed.theclinics.com
Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by the accumulation
of surfactant in alveolar macrophages and alveoli resulting in hypoxemic respiratory failure.
In 1958, Rosen and colleagues 1 first reported PAP as a disorder consisting of filling of
alveoli by a periodic acid–Schiff (PAS)-positive proteinaceous material, rich in lipid. The
accumulated material is now known to be comprised primarily of pulmonary surfactant and
smaller amounts of cell debris. PAP is often reported in the medical literature as a disease …