Pulmonary alveolar proteinosis was first described in 1958 on the basis of 27 biopsy and autopsy cases.1 Individuals in industrialized nations appear to be more susceptible to the disease, but no occupational relation has been clearly established. Most cases have been observed in the United States; a few have been seen in Great Britain, France, Germany, and Italy. Two cases have been reported from Japan and one from New Zealand.
Although an additional 80 cases have been added to the world literature since the original description, the cause of the disease remains unknown and its clinical course has not been well delineated. Lipid-rich proteinaceous material may remain in the lung for months or years without showing any apparent change as observed roentgenographically, or it may disappear rapidly, leaving few or no tissue changes and no residual impairment of respiratory function. In the absence of liver dysfunction, a persistent elevation
PULMONARY ALVEOLAR PROTEINOSIS. JAMA. 1967;199(6):418. doi:10.1001/jama.1967.03120060116023
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