Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of surfactant in the alveoli in the lungs

Classically it is associated with smoking and occurs in a young / middle aged adult

Types

Primary PAP: Also known as autoimmune PAP

Secondary PAP: This occurs secondary to conditions that affect the lungs’ ability to clear surfactant, such (IE malignancy, infection, or pneumoconiosis).

Congenital PAP: A rare form of the disease that is present at birth due to genetic mutations involved in surfactant production.

Chest Radiograph

1.     Perihilar lung opacification - “bat-wing” configuration without signs of left heart failure

2.     Diffuse pulmonary opacities

3.     No lymphadenopathy, no cardiomegaly, no pleural effusion