Current Thoracic Surgery

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by accumulation of material in the alveoli of the lungs. It is generally diagnosed in middle-aged men. The most common complaints are cough and dyspnea. Characteristic radiological findings of PAP include nodular infiltration with a tendency to merge and ground glass density, septal thickening and resemblance to paving stone. Typical findings include periodic acid-Schiff (PAS) stain positive materials in the alveolar proteinosis, milky appearance of bronchoalveolar lavage (BAL), interstitial opacities, thickened interlobular septa, and patchy central or peripheral ground-glass opacities in the lung parenchyma in the chest CT. The most effective proved treatment is whole lung lavage. The aim of the treatment in PAP cases is to restore the alveolar gas exchange by removing excess surfactant accumulated in the alveoli.