[A case of pulmonary alveolar proteinosis with invasion of macrophages in alveolar interstitial region]

Abstract

A 24-year-old woman was admitted to our hospital on November 2, 1989 for investigation and treatment of abnormal shadows detected in her routine chest radiograph on July 19, 1989. The chest X-ray film on admission showed diffuse infiltrative shadows in the peripheral regions of the bilateral middle and lower lung fields. In addition centrilobular shadows in the subpleural regions with increases irregular densities were found on chest CT. We therefore suspected pulmonary alveolar proteinosis. Bronchoalveolar lavage from the right middle bronchus and transbronchial lung biopsy of the right upper and lower lobes were performed. Electron microscopic examination of a specimen of bronchoalveolar lavage fluid revealed many multilamellar bodies, characteristic of pulmonary alveolar proteinosis. In addition, proteinaceous material containing multilamellar bodies was observed within the alveolar lumina. We diagnosed this case as pulmonary alveolar proteinosis. Electron microscopy also revealed many macrophages in the alveolar walls, some of which contained a few or numerous multilamellar bodies within the secondary lysosomes. We treated this patient with oral and inhaled Ambroxol, with improvement of her clinical condition.