Pulmonary Alveolar Proteinosis

Abstract

In 1958, Rosen, Castleman, and Liebow (1) first described pulmonary alveolar proteinosis, a disease in which a homogeneous proteinaceous material filled the alveoli and small bronchi. These authors felt that this was a new disease entity because such striking pathological findings could hardly have been overlooked by previous observers. The course of the disease may be interrupted by spontaneous remission (2, 3), but death often occurs. Rosen listed 15 deaths among the 50 cases he had collected by 1960 (4). Other authors have reported 8 additional deaths (5-11). In all, 23 deaths have been reported among the approximately 85 cases