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

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. Char...

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Bibliographic Details
Published in:Canadian respiratory journal 2016-01, Vol.2016 (2016), p.1-8
Main Authors: Li, Huiping, Cao, Weijun, Gao, Beilan, Yang, WenLan, Xu, JinFu, Bai, JiuWu, Liang, Shuo
Format: Article
Language:English
Subjects:
Adult
Biopsy
Care and treatment
China - epidemiology
Cross-Sectional Studies
Disease
Female
Hospitals
Humans
Immunoglobulins
Male
Middle Aged
Mortality
Patients
Prognosis
Proteins
Pulmonary alveolar proteinosis
Pulmonary Alveolar Proteinosis - diagnosis
Pulmonary Alveolar Proteinosis - mortality
Retrospective Studies
Severity of Illness Index
Surfactants
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Summary: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.
ISSN:1198-2241
1916-7245
DOI:10.1155/2016/3412836