Defective Lung Macrophages in Pulmonary Alveolar Proteinosis

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Abstract
Lung macrophages obtained by segmental lavage from 3 patients with pulmonary alveolar proteinosis were studied in vitro. The macrophages exhibited morphologic abnormalities including excess lipid accumulation and giant secondary lysosome formation. These cells survived poorly in tissue culture, showed imparied chemotactic activity and had decreased adhesiveness to glass. They phagocytized normally but had substantially decreased capacity to kill ingested Candida pseudotropicalis. Evidence was obtained that the macrophage defect was acquired and probably related to ingestion of the proteinaceous alveolar fluid. Peripheral blood monocyte function was normal in 1 patient and morphologic abnormalities were produced in normal monocyte-derived macrophages cultured with proteinaceous lavage material. The lung macrophage in alveolar proteinosis is probably a defective cell as a consequence of an abnormal pulmonary environment.

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