Pulmonary Alveolar Proteinosis

Abstract

PULMONARY alveolar proteinosis is a disease of unknown etiology characterized by the deposition of periodic-acidSchiff-(PAS)-positive granular and floccular proteinaceous material, rich in lipids, within the lumens of the distal air spaces.¹ The proteinaceous material is believed to be derived from the granular transformation of proliferated large mononuclear cells in the walls of the alveoli ("septal cells") and their subsequent sloughing and degeneration. Since the initial report of this condition in 1958¹ additional cases have been described with increasing frequency.^{2 3 4 5 6 7 8} The treatment of pulmonary alveolar proteinosis has been principally symptomatic. Therapeutic trials have included adrenal steroids,^{1 , 3 , 7} antibiotics,¹ potassium iodide in large . . .