title: A New Scale to Assess the Severity and Prognosis of Pulmonary Alveolar Proteinosis.
abstract: 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, PaO2, lung function parameters, and HRCT score of 101 patients with PAP were retrospectively analyzed. Many indexes were explored and integrated into a scale. Results. PaO2 was lower among smokers than among never-smokers. PaO2 differed between each pair of patient groups stratified according to HRCT score or DLCO, % predicted, which differed between any two groups stratified according to PaO2. The PAP patients who died presented with more symptoms, a higher HRCT score, and lower DLCO, % predicted, than survivors. Smoking status, symptoms, PaO2, HRCT score, and DLCO, % predicted, were integrated into a scale (severity and prognosis score of PAP (SPSP)). SPSP correlated positively with PaO2, FVC, % predicted, FEV1, % predicted, and DLCO, % predicted, and negatively with HRCT score. The patients who died displayed a higher SPSP than survivors. Conclusion. Smoking status, symptoms, PaO2, 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.
authors.affiliation: Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: JiuWu
authors.initials: J
authors.lastname: Bai
authors.affiliation: Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: JinFu
authors.identifier: 0000-0002-8039-8973
authors.initials: J
authors.lastname: Xu
authors.affiliation: Department of Pulmonary Function Test, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: WenLan
authors.initials: W
authors.lastname: Yang
authors.affiliation: Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: Beilan
authors.initials: B
authors.lastname: Gao
authors.affiliation: Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: Weijun
authors.initials: W
authors.lastname: Cao
authors.affiliation: Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: Shuo
authors.initials: S
authors.lastname: Liang
authors.affiliation: Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, 507 Zheng Min Road, Shanghai 200433, China.
authors.forename: Huiping
authors.identifier: 0000-0002-6998-9828
authors.initials: H
authors.lastname: Li
mesh_terms: D000328:Adult; D002681:China / Q000453:epidemiology; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000401:mortality; D012189:Retrospective Studies; D012720:Severity of Illness Index