Alveolar phospholipids in pulmonary alveolar proteinosis.

The biochemical compositions of lung lavage fluid from three patients and that of the lung tissue obtained by a biopsy from a patient with alveolar proteinosis were analyzed. The lung lavage fluid was separated to the supernatant and the insoluble precipitate fractions by a brief centrifugation and the lung tissue was separated to surfactant and residual fractions by sucrose density gradient centrifugation. The supernatant of lung washing contained larger quantities of protein and less lipid which was mainly composed of lecithin and sphingomyelin. In contrast, the insoluble material of lung washing contained 4--8 fold of lipid as compared with the supernatant, and lecithin was a predominant phospholipid. From the analysis of fatty acid and individual molecular species of major phospholipids, the higher contents of dipalmitoyllecithin and sphingomyelin containing palmitate were found in the insoluble material. The phospholipid composition and fatty acid composition of lecithin were similar between the precipitate fraction of lung washing and the surfactant fraction of lung tissue. The ratio of protein to phospholipid in the precipitate fraction was similar to that of lung washing. These analytical data suggest that the materials accumulated in the alveoli of the patients may be derived mainly from the lung surfactant fraction. The metabolic studies showed that lecithin synthesis through de novo and lysolecithin pathways works in human lung with this disease as actively as in rat lung. The secretion of lung lecithin to the surfactant fraction also appeared to be normal. From these analytical and metabolic findings, the causes for the accumulation of the materials which cover alveolar surface and impair gaseous exchange in the alveoli of pulmonary alveolar proteinosis, were discussed.