Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage
Joint Authors
Shioya, Makoto
Otsuka, Mitsuo
Yamada, Gen
Umeda, Yasuaki
Ikeda, Kimiyuki
Nishikiori, Hirotaka
Kuronuma, Koji
Chiba, Hirofumi
Takahashi, Hiroki
Source
Issue
Vol. 2018, Issue 2018 (31 Dec. 2018), pp.1-7, 7 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2018-08-13
Country of Publication
Egypt
No. of Pages
7
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Shioya, Makoto& Otsuka, Mitsuo& Yamada, Gen& Umeda, Yasuaki& Ikeda, Kimiyuki& Nishikiori, Hirotaka…[et al.]. 2018. Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. Canadian Respiratory Journal،Vol. 2018, no. 2018, pp.1-7.
https://search.emarefa.net/detail/BIM-1151815
Modern Language Association (MLA)
Shioya, Makoto…[et al.]. Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. Canadian Respiratory Journal No. 2018 (2018), pp.1-7.
https://search.emarefa.net/detail/BIM-1151815
American Medical Association (AMA)
Shioya, Makoto& Otsuka, Mitsuo& Yamada, Gen& Umeda, Yasuaki& Ikeda, Kimiyuki& Nishikiori, Hirotaka…[et al.]. Poorer Prognosis of Idiopathic Pleuroparenchymal Fibroelastosis Compared with Idiopathic Pulmonary Fibrosis in Advanced Stage. Canadian Respiratory Journal. 2018. Vol. 2018, no. 2018, pp.1-7.
https://search.emarefa.net/detail/BIM-1151815
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1151815