Pulmonary Alveolar Proteinosis

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Abstract
Eighty-five cases of pulmonary alveolar proteinosis, including 6 new cases, are reviewed. Several important clinical features are confirmed and need re-emphasis. Superimposed pulmonary infection is frequent and hazardous. The wide variety of pathogenic organisms, especially fungi, that have been isolated from these patients emphasizes the importance of cultural studies in guiding treatment. Specific therapy of infections and nonspecific methods of promoting the elimination of the alveolar material have been successful in keeping the majority of patients alive and in good condition. The use of adrenal steroids would appear to be contraindicated. A new theory of pathogenesis has been proposed. The chemical similarity between the alveolar filling material in pulmonary alveolar proteinosis and the normal surface-active agent secreted by the alveolar epithelial cells has been pointed out. Certain diseases such as the respiratory distress syndrome of newborns have been attributed to a deficiency of this surface-active material. By anaology to other conditions, it would seem plausible that a disease corresponding to hypersecretion would also be found. It is proposed that pulmonary alveolar proteinosis might be such a disease. Although this theory does not go much further in explaining the etiology of the disease, it may serve as a useful concept for future investigation.

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