Primary Alveolar Proteinosis and Review of the Literature

Pulmonary alveolar proteinosis is a rare lung disease characterized by the accumulation of lipoproteinaceous material within the alveoli. Analysis of the lipoproteinaceous materials accumulating in the airspaces demonstrates that they represent an abnormal deposition of the normal constituents of surfactant. This deposi­tion is due to an increased secretion or a decreased clearance from the alveoli. We present the diagnosis of primary pulmonary alve­olar proteinosis of a 26 year old female. She was admitted to our department with a dry cough and dyspnea with a slowly progres­sive course. The patient, who was started antituberculous therapy one year ago, had persistent symmetrical airspace consolidation in the chest x-ray. The diagnosis was made by open lung biopsy. Out­patient showed radiological and clinical remission during the time she was hospitalized.