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Background . Pulmonary Alveolar Proteinosis (PAP) is a syndrome characterized by pulmonary surfactant accumulation. Small proportion of PAP patients experienced spontaneous remission.  Objective . The aim of this study was to assess the severity and prognosis of PAP using various indexes.  Methods . Characteristics, PaO 2 , lung function parameters, and HRCT score of 101 patients with PAP were retrospectively analyzed. Many indexes were explored and integrated into a scale.  Results . PaO 2  was lower among smokers than among never-smokers. PaO 2  differed between each pair of patient groups stratified according to HRCT score or DLCO, % predicted, which differed between any two groups stratified according to PaO 2 . The PAP patients who died presented with more symptoms, a higher HRCT score, and lower DLCO, % predicted, than survivors. Smoking status, symptoms, PaO 2 , HRCT score, and DLCO, % predicted, were integrated into a scale (severity and prognosis score of PAP (SPSP)). SPSP correlated positively with PaO 2 , FVC, % predicted, FEV 1 , % predicted, and DLCO, % predicted, and negatively with HRCT score. The patients who died displayed a higher SPSP than survivors.  Conclusion . Smoking status, symptoms, PaO 2 , HRCT score, and DLCO, % predicted, were integrated into a scale (SPSP) that can be used to assess the severity and prognosis of PAP to some degree.

A New Scale to Assess the Severity and Prognosis of Pulmonary Alveolar Proteinosis