Pulmonary Alveolar Proteinosis

Abstract

In the attempt to obtain further information concerning the natural history of this newly elucidated disease entity, one of the patients originally reported by Rosen, Castleman, and Liebow in July 1958, has been followed an additional 4 years to the present. This long observation, the extensive pulmonary function tests, and the cardiac catheterization performed in this case, make it worth publication as an addition to our knowledge of a new and rare disease entity. Progressive weakness, dyspnea, and extensive bilateral pulmonary infiltration initially were accompanied by a restrictive type of respiratory defect with a suggested pulmonary alveolo-capillary blockade. Over the next 4 years, there occurred clinically progressive amelioration, with a returm to full time employment and a nearly asymptomatic status for the last one and one-half years; function tests indicated disappearance of the alveolo-capillary block and conversion to a rather mild obstructive disease during the first two years with nearly complete clearing subsequently. The cardiac catheterization at the end of three years showed only questionable to mild abnormality. The x-rays, however, improved only to a moderate degree and continued to show fairly extensive pathology. The detailed findings of the biopsy, chest x-rays, and function tests are displayed and analyzed in comparison with such data as is available in the literature.