| Item type |
学術雑誌論文 / Journal Article(1) |
| 公開日 |
2021-10-01 |
| タイトル |
|
|
タイトル |
Disease course and prognosis of pleuroparenchymal fibroelastosis compared with idiopathic pulmonary fibrosis |
|
言語 |
en |
| 言語 |
|
|
言語 |
eng |
| キーワード |
|
|
主題 |
pleuroparechymal fibroelastosis |
| キーワード |
|
|
主題 |
clinical diagnosis |
| キーワード |
|
|
主題 |
acute exacerbation |
| キーワード |
|
|
主題 |
prognosis |
| キーワード |
|
|
主題 |
idiopathic pulmonary fibrosis |
| 資源タイプ |
|
|
資源タイプ識別子 |
http://purl.org/coar/resource_type/c_6501 |
|
資源タイプ |
journal article |
| その他のタイトル |
|
|
その他のタイトル |
Disease course and prognosis of iPPFE |
| 著者 |
Suzuki, Yuzo
Fujisawa, Tomoyuki
Sumikawa, Hiromitsu
Tanaka, Tomonori
Sugimoto, Chikatoshi
Kono, Masato
Hozumi, Hironao
Karayama, Masato
Furuhashi, Kazuki
Enomoto, Noriyuki
Nakamura, Yutaro
Inui, Naoki
Suda, Takafumi
|
| 書誌情報 |
Respiratory Medicine
巻 171,
p. 106078,
発行日 2020-09
|
| 出版者 |
|
|
出版者 |
Elsevier |
| 権利 |
|
|
権利情報 |
Copyright 2020. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/ |
| 権利 |
|
|
権利情報 |
"""This is the accepted manuscript version. The formal published version is available at """"https://doi.org/10.1016/j.rmed.2020.106078"""".""" |
| 抄録 |
|
|
内容記述タイプ |
Abstract |
|
内容記述 |
Background: Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare interstitial lung disease characterised by unique radiological and pathological findings. However, pathological evaluations are available only in a limited number of patients. Therefore, several clinical diagnostic criteria have been proposed. Nevertheless, the applicability of these criteria has not yet been validated. Moreover, the clinical course of iPPFE and its prognosis have not yet been completely elucidated.
Methods: The present study assessed previously proposed clinical diagnostic criteria by comparing the clinical features between pathologically diagnosed iPPFE (p-iPPFE) and clinically diagnosed iPPFE (c-iPPFE). Subsequently, the clinical features of iPPFE were characterized and compared with those of idiopathic pulmonary fibrosis (IPF, n=323).
Results: Clinical characteristics of c-iPPFE (n=27) and p-iPPFE (n=35) were similar. No significant difference was observed in terms of prognosis between c-iPPFE and p-iPPFE. The number of patients with iPPFE (both c-iPPFE and p-iPPFE) who developed lung cancer was significantly lower than that of patients with IPF. However, acute exacerbation (AE) showed similar incidence in patients with iPPFE and IPF. Survival of patients with iPPFE was significantly worse than that of patients with IPF (5-year survival rate: 38.5% vs. 63.5%, p<0.0001), and the most common cause of death was chronic respiratory failure (73.8%), followed by AE (14.3%). Male gender was the only poor prognostic factor of iPPFE.
Conclusion: The present study demonstrated efficiency of clinical diagnosis and also revealed clinically important characteristics of iPPFE that should be considered for management of iPPFE. |
| ISSN |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
0954-6111 |
| EISSN |
|
|
収録物識別子タイプ |
ISSN |
|
収録物識別子 |
1532-3064 |
| PubMed番号 |
|
|
関連タイプ |
isVersionOf |
|
|
識別子タイプ |
PMID |
|
|
関連識別子 |
32917352 |
| 出版社DOI |
|
|
関連タイプ |
isVersionOf |
|
|
識別子タイプ |
DOI |
|
|
関連識別子 |
10.1016/j.rmed.2020.106078 |
| 著者版フラグ |
|
|
出版タイプ |
AM |
|
出版タイプResource |
http://purl.org/coar/version/c_ab4af688f83e57aa |
Respir Med.-171-106078.pdf (1.2 MB)
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