Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage
Objective. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and id...
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| Format: | Article |
| Language: | English |
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Wiley
2018-01-01
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| Series: | Canadian Respiratory Journal |
| Online Access: | http://dx.doi.org/10.1155/2018/6043053 |
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| author | Makoto Shioya Mitsuo Otsuka Gen Yamada Yasuaki Umeda Kimiyuki Ikeda Hirotaka Nishikiori Koji Kuronuma Hirofumi Chiba Hiroki Takahashi |
| author_facet | Makoto Shioya Mitsuo Otsuka Gen Yamada Yasuaki Umeda Kimiyuki Ikeda Hirotaka Nishikiori Koji Kuronuma Hirofumi Chiba Hiroki Takahashi |
| author_sort | Makoto Shioya |
| collection | DOAJ |
| description | Objective. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and idiopathic pulmonary fibrosis (IPF). Methods. We performed a retrospective cohort study on 375 consecutive idiopathic interstitial pneumonia patients between April 2004 and December 2014. Among them, we diagnosed IPPFE and IPF patients using high-resolution computed tomography radiological criteria. Results. Twenty-nine IPPFE patients (9 males, 20 females) and 67 IPF patients (54 males, 13 females) were enrolled. IPPFE patients were significantly more likely to be females and nonsmokers and had lower body mass index, lower values of predicted percentage of forced vital capacity (%FVC), and a higher residual volume-to-total lung capacity ratio than IPF patients. Survival analysis revealed that they had significantly poorer prognosis than IPF patients in GAP (gender, age, and physiology) stages II + III. %FVC and GAP index independently predict mortality in patients with IPPFE. Conclusions. Patients with IPPFE showed poorer prognosis in the advanced stage than patients with IPF. %FVC and GAP index are independent predictors of survival in patients with IPPFE. |
| format | Article |
| id | doaj-art-a5d800c911264d3db08db380835c5364 |
| institution | Kabale University |
| issn | 1198-2241 1916-7245 |
| language | English |
| publishDate | 2018-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Canadian Respiratory Journal |
| spelling | doaj-art-a5d800c911264d3db08db380835c53642025-08-20T03:38:48ZengWileyCanadian Respiratory Journal1198-22411916-72452018-01-01201810.1155/2018/60430536043053Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced StageMakoto Shioya0Mitsuo Otsuka1Gen Yamada2Yasuaki Umeda3Kimiyuki Ikeda4Hirotaka Nishikiori5Koji Kuronuma6Hirofumi Chiba7Hiroki Takahashi8Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanDepartment of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, South-1, West-16, Sapporo 060-8543, JapanObjective. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease characterized by predominant upper lobe pulmonary fibrosis of unknown etiology. However, the prognosis of IPPFE has not been discussed. We investigated the clinical characteristics and prognostic factors of IPPFE and idiopathic pulmonary fibrosis (IPF). Methods. We performed a retrospective cohort study on 375 consecutive idiopathic interstitial pneumonia patients between April 2004 and December 2014. Among them, we diagnosed IPPFE and IPF patients using high-resolution computed tomography radiological criteria. Results. Twenty-nine IPPFE patients (9 males, 20 females) and 67 IPF patients (54 males, 13 females) were enrolled. IPPFE patients were significantly more likely to be females and nonsmokers and had lower body mass index, lower values of predicted percentage of forced vital capacity (%FVC), and a higher residual volume-to-total lung capacity ratio than IPF patients. Survival analysis revealed that they had significantly poorer prognosis than IPF patients in GAP (gender, age, and physiology) stages II + III. %FVC and GAP index independently predict mortality in patients with IPPFE. Conclusions. Patients with IPPFE showed poorer prognosis in the advanced stage than patients with IPF. %FVC and GAP index are independent predictors of survival in patients with IPPFE.http://dx.doi.org/10.1155/2018/6043053 |
| spellingShingle | Makoto Shioya Mitsuo Otsuka Gen Yamada Yasuaki Umeda Kimiyuki Ikeda Hirotaka Nishikiori Koji Kuronuma Hirofumi Chiba Hiroki Takahashi Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage Canadian Respiratory Journal |
| title | Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage |
| title_full | Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage |
| title_fullStr | Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage |
| title_full_unstemmed | Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage |
| title_short | Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage |
| title_sort | poorer prognosis of idiopathic pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis in advanced stage |
| url | http://dx.doi.org/10.1155/2018/6043053 |
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