Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China
BackgroundThe high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient.MethodsWe retrospectively evaluated HRCT...
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Frontiers Media S.A.
2023-02-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2023.1058001/full |
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| author | Jiu-Wu Bai Jian-nan Huang Shen-yun Shi Ai Ge Hai-wen Lu Xiao-li Sun Shu-yi Gu Shuo Liang Ke-bin Cheng Xin-lun Tian Yong-long Xiao Kai-feng Xu Jin-Fu Xu |
| author_facet | Jiu-Wu Bai Jian-nan Huang Shen-yun Shi Ai Ge Hai-wen Lu Xiao-li Sun Shu-yi Gu Shuo Liang Ke-bin Cheng Xin-lun Tian Yong-long Xiao Kai-feng Xu Jin-Fu Xu |
| author_sort | Jiu-Wu Bai |
| collection | DOAJ |
| description | BackgroundThe high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient.MethodsWe retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the curative effect was assessed after 3 months.ResultsThe HRCT overall density and extent scores in our retrospective analysis were higher than the extent scores in all patients and every original extent score severity group, as well as higher related to arterial partial oxygen pressure (PaO2) than extent scores. The mild patients accounted for 61.9% based on DSS 1–2, 20.3% based on SPSP 1–3, and 20.8% based on SPSPII 1–3. Based on SPSP or SPSPII, the number of severe patients deteriorating was higher in the mild and moderate groups. When applied prospectively, arterial PaO2 differed between any two SPSPII severity groups. The alveolar-arterial gradient in PaO2 (P[A-a]O2), % predicted carbon monoxide diffusing capacity of the lung (DLCO), and HRCT score were higher in the severe group than in the mild and moderate groups. After diagnosis, mild patients received symptomatic treatment, moderate patients received pure whole lung lavage (WLL) or granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy, and severe patients received WLL and GM-CSF therapy. Importantly, the SPSPII in mild and severe groups were lower than baseline after 3 months.ConclusionThe HRCT density and extent scores of patients with APAP were better than the extent score. The SPSPII score system based on smoking status, symptoms, PaO2, predicted DLCO, and overall HRCT score was better than DSS and SPSP for assessing the severity and efficacy and predicting the prognosis.Trial registrationClinicalTrial.gov, identifier: NCT04516577. |
| first_indexed | 2024-04-10T16:54:35Z |
| format | Article |
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| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-04-10T16:54:35Z |
| publishDate | 2023-02-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Medicine |
| spelling | doaj.art-c0bee96c55034868aa7c29416f4fa87e2023-02-07T08:35:37ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2023-02-011010.3389/fmed.2023.10580011058001Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in ChinaJiu-Wu Bai0Jian-nan Huang1Shen-yun Shi2Ai Ge3Hai-wen Lu4Xiao-li Sun5Shu-yi Gu6Shuo Liang7Ke-bin Cheng8Xin-lun Tian9Yong-long Xiao10Kai-feng Xu11Jin-Fu Xu12Department of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, ChinaDepartment of Respiratory and Critical Care Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Jiangsu, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaDepartment of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, ChinaDepartment of Respiratory and Critical Care Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, Jiangsu, ChinaDepartment of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, ChinaDepartment of Respiratory and Critical Care Medicine, Shanghai Pulmonary Hospital, School of Medicine, Tongji University, Shanghai, ChinaBackgroundThe high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient.MethodsWe retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the curative effect was assessed after 3 months.ResultsThe HRCT overall density and extent scores in our retrospective analysis were higher than the extent scores in all patients and every original extent score severity group, as well as higher related to arterial partial oxygen pressure (PaO2) than extent scores. The mild patients accounted for 61.9% based on DSS 1–2, 20.3% based on SPSP 1–3, and 20.8% based on SPSPII 1–3. Based on SPSP or SPSPII, the number of severe patients deteriorating was higher in the mild and moderate groups. When applied prospectively, arterial PaO2 differed between any two SPSPII severity groups. The alveolar-arterial gradient in PaO2 (P[A-a]O2), % predicted carbon monoxide diffusing capacity of the lung (DLCO), and HRCT score were higher in the severe group than in the mild and moderate groups. After diagnosis, mild patients received symptomatic treatment, moderate patients received pure whole lung lavage (WLL) or granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy, and severe patients received WLL and GM-CSF therapy. Importantly, the SPSPII in mild and severe groups were lower than baseline after 3 months.ConclusionThe HRCT density and extent scores of patients with APAP were better than the extent score. The SPSPII score system based on smoking status, symptoms, PaO2, predicted DLCO, and overall HRCT score was better than DSS and SPSP for assessing the severity and efficacy and predicting the prognosis.Trial registrationClinicalTrial.gov, identifier: NCT04516577.https://www.frontiersin.org/articles/10.3389/fmed.2023.1058001/fullautoimmune pulmonary alveolar proteinosisdisease severity scoregranulocyte-macrophage colony-stimulating factorseverity and prognosis scorewhole lung lavage |
| spellingShingle | Jiu-Wu Bai Jian-nan Huang Shen-yun Shi Ai Ge Hai-wen Lu Xiao-li Sun Shu-yi Gu Shuo Liang Ke-bin Cheng Xin-lun Tian Yong-long Xiao Kai-feng Xu Jin-Fu Xu Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China Frontiers in Medicine autoimmune pulmonary alveolar proteinosis disease severity score granulocyte-macrophage colony-stimulating factor severity and prognosis score whole lung lavage |
| title | Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China |
| title_full | Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China |
| title_fullStr | Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China |
| title_full_unstemmed | Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China |
| title_short | Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China |
| title_sort | updated severity and prognosis score of pulmonary alveolar proteinosis a multi center cohort study in china |
| topic | autoimmune pulmonary alveolar proteinosis disease severity score granulocyte-macrophage colony-stimulating factor severity and prognosis score whole lung lavage |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2023.1058001/full |
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