Pulmonary Alveolar Proteinosis: An Overview and Emerging Therapeutics

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary syndrome characterized by surfactant accumulation in the alveoli, which results impaired gas exchange. There are three types of PAP: primary, secondary and congenital. Autoimmune PAP, under category primary most common form, caused an impairment granulocyte–macrophage colony-stimulating factor (GM-CSF) signalling, due to presence anti-GM-CSF autoantibodies. While some patients with PAP remain asymptomatic, present progressive dyspnoea insidious onset that, rarely, can progress respiratory failure death. Currently, there no known cure approved therapies for PAP. Whole-lung lavage remains gold-standard treatment; however, several novel promising targeted being actively investigated.