Skip to main content

Main menu

  • Home
  • Current issue
  • Early View
  • Archive
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Institutional open access agreements
    • Peer reviewer login
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • Early View
  • Archive
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Institutional open access agreements
    • Peer reviewer login
  • Alerts
  • Subscriptions

Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia

Keishi Sugino, Hirotaka Ono, Hiroshige Shimizu, Takeyuki Kurosawa, Keiko Matsumoto, Masahiro Ando, Kiyoshi Mori, Eiyasu Tsuboi, Sakae Homma, Kazuma Kishi
ERJ Open Research 2021 7: 00196-2020; DOI: 10.1183/23120541.00196-2020
Keishi Sugino
1Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
2Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: ks142129_ikusou@ybb.ne.jp
Hirotaka Ono
1Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hiroshige Shimizu
2Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Takeyuki Kurosawa
2Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Keiko Matsumoto
3Dept of Radiology, Toho University Omori Medical Center, Tokyo, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Masahiro Ando
1Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kiyoshi Mori
1Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Eiyasu Tsuboi
1Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sakae Homma
4Dept of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, Tokyo, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kazuma Kishi
2Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Figures

  • Tables
  • FIGURE 1
    • Download figure
    • Open in new tab
    • Download powerpoint
    FIGURE 1

    The Kaplan–Meier survival curve in patients with typical idiopathic pulmonary fibrosis (IPF) (solid line) (n=195) and idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP) (dashed line) (n=64) (median survival time 62.3 months versus 34.0 months, p<0.0001).

  • FIGURE 2
    • Download figure
    • Open in new tab
    • Download powerpoint
    FIGURE 2

    Change in forced vital capacity (FVC) before and after treatments with antifibrotic agents (idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP)/typical idiopathic pulmonary fibrosis (IPF)in 32 out of 58 cases). There were no differences between the groups in FVC value 6 months before treatment with antifibrotic agents (IPPFE with UIP versus typical IPF = −0.21±0.16 L versus −0.20±0.19 L; p=0.95). However, more significantly reduced FVC values 6 months after treatment with antifibrotic agents were found in patients with IPPFE with UIP than in those with typical IPF (IPPFE with UIP versus typical IPF = −0.15±0.17 L versus −0.004±0.18 L; p=0.0002; IPPFE with UIP: before 6 months versus at the onset of treatments versus after 6 months = 1.96±0.66 L versus 1.75±0.64 L versus 1.61±0.61 L; p<0.0001, p<0.0001; typical IPF: before 6 months versus at the onset of treatments versus after 6 months = 2.25±0.61 L versus 2.05±0.62 L versus 2.05±0.64 L, p<0.0001, p=1.00). Two-way repeated measure ANOVA with Bonferroni's multiple comparison.

  • FIGURE 3
    • Download figure
    • Open in new tab
    • Download powerpoint
    FIGURE 3

    Change in % predicted forced vital capacity (FVC) during follow-up. a) Linear mixed model (LMM) analysis showed that the decline in the slope of FVC during follow-up was significantly different between the two groups (p=0.0003). Patients with typical idiopathic pulmonary fibrosis (IPF) had a significantly higher baseline FVC than those with idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP) (77.4±18.7% versus 63.9±15.7%, p<0.0001). b) Patients with IPPFE with UIP during follow-up who were treated with antifibrotic agents had a significantly lower baseline FVC and a more rapid decline in FVC compared with that in those with typical IPF treated with antifibrotic agents (p=0.0002). Patients with typical IPF had a significantly higher baseline FVC than those with IPPFE with UIP (73.8±17.8% versus 64.6±14.9%, p=0.003).

Tables

  • Figures
  • TABLE 1

    Demographic and baseline patient characteristics

    VariableIPPFE with UIPTypical IPFp-value
    Subjects64195
    Males/females43/21162/330.012
    Age years72.7±7.172.7±7.10.972
    BMI kg·m−217.6±2.922.7±3.5<0.0001
    Current/former/never smokers6/34/2426/136/330.003
    mMRC score 0/I/II/III/IV5/25/23/6/522/61/60/40/120.248
    Severity of IPF GAP stage I/II/III28/24/1299/68/280.555
    Primary lung cancer0 (0%)12 (6.2%)0.042
    Pneumothorax20 (31.3%)3 (1.5%)<0.0001
    Acute exacerbation19 (29.7%)64 (32.8%)0.757
    Nintedanib8 (12.5%)38 (19.4%)0.06
    Pirfenidone26 (40.6%)53 (27.1%)0.06
    Histological UIP diagnosis10 (15.6%)42 (21.5%)0.370
    Histological PPFE diagnosis10 (15.6%)0 (0%)<0.0001
    Observation period months30.7±19.738.9±34.20.07

    Data are presented as n, mean±sd or n (%), unless otherwise stated. IPPFE: idiopathic pleuroparenchymal fibroelastosis; UIP: usual interstitial pneumonia; IPF: idiopathic pulmonary fibrosis; BMI: body mass index; mMRC: modified Medical Research Council; GAP: gender, age and lung physiology; PPFE: pleuroparenchymal fibroelastosis.

    • TABLE 2

      Comparison of pulmonary function tests, serum markers and chest computed tomography (CT) findings between patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP) and typical idiopathic pulmonary fibrosis (IPF)

      VariableIPPFE with UIPTypical IPFp-value
      Subjects n64195
      FVC % predicted66.7±16.978.5±18.80.0001
      FEV1 % predicted86.1±24.997.2±47.30.073
      TLC % predicted74.4±15.777.9±16.60.153
      RV % predicted90.6±27.382.9±21.90.026
      DLCO % predicted66.2±25.260.3±20.40.072
      CPI41.5±18.243.0±21.70.621
      KL-6 U·mL−1823±4851123±7430.003
      SP-D ng·mL−1283±162293±2340.742
      PaO2 mmHg84.1±13.480.8±13.10.087
      PaCO2 mmHg43.6±5.839.4±3.9<0.0001
      Fibrosis score1.7±0.71.6±0.60.953
      Extent of bronchiectasis1.8±0.81.6±0.70.106
      CT pattern: UIP/possible UIP/inconsistent with UIP169/22/4

      Data are presented as mean±sd unless otherwise stated. FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; TLC: total lung capacity; RV: residual volume; DLCO: diffusing capacity of the lung for carbon monoxide; CPI: composite physiological index; KL-6: Krebs von den Lungen-6; SP-D: surfactant protein D; PaO2: arterial oxygen tension; PaCO2: arterial carbon dioxide tension.

      • TABLE 3

        Fine–Gray univariable and multivariable competing risks models demonstrating prognostic factors for survival in patients with idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP) (n=64)

        VariableUnivariateMultivariate
        SHR (95% CI)p-valueSHR (95% CI)p-value
        Age1.000 (0.945–1.058)0.991
        Female sex1.659 (0.753–3.651)0.209
        BMI0.757 (0.632–0.907)0.0030.806 (0.659–0.987)0.037
        Ever-smokers0.754 (0.350–1.625)0.472
        mMRC score0.996 (0.687–1.445)0.985
        SpO2 <90%1.284 (0.601–2.742)0.518
        FVC % predicted0.955 (0.929–0.982)0.0010.971 (0.946–0.995)0.019
        Prednisolone use0.502 (0.223–1.132)0.0970.228 (0.072–0.727)0.012
        Pneumothorax3.029 (1.366–6.714)0.006

        SHR: subdistribution hazard ratio; BMI: body mass index; mMRC: modified Medical Research Council; SpO2: peripheral oxygen saturation; FVC: forced vital capacity.

        • TABLE 4

          Comparison of causes of death between idiopathic pleuroparenchymal fibroelastosis (IPPFE) with usual interstitial pneumonia (UIP) and typical idiopathic pulmonary fibrosis (IPF)

          VariableIPPFE with UIPTypical IPFp-value
          Subjects n64195
          Lung cancer0 (0%)6 (3.0%)0.341
          Pneumonia12 (18.7%)16 (8.2%)0.034
          Acute exacerbation9 (14.0%)32 (16.4%)0.843
          Chronic deterioration20 (31.2%)15 (7.6%)<0.0001
          Others3 (4.6%)11 (5.6%)1.000
          Unknown4 (6.2%)2 (1.0%)0.034

          Data are presented as n (%) unless otherwise stated.

          • TABLE 5

            Multivariate logistic regression analysis of predictive factors for efficacy of antifibrotic agents (n=125)

            VariableOR (95% CI)p-value
            Age1.013 (0.948–1.086)0.699
            Male sex0.799 (0.268–2.587)0.697
            BMI1.079 (0.918–1.277)0.354
            GAP stage1.644 (0.845–3.218)0.142
            IPPFE with UIP7.096 (2.018–28.270)0.002

            BMI: body mass index; GAP: gender, age and lung physiology; IPPFE: idiopathic pleuroparenchymal fibroelastosis; UIP: usual interstitial pneumonia.

            PreviousNext
            Back to top
            Vol 7 Issue 1 Table of Contents
            ERJ Open Research: 7 (1)
            • Table of Contents
            • Index by author
            Email

            Thank you for your interest in spreading the word on European Respiratory Society .

            NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

            Enter multiple addresses on separate lines or separate them with commas.
            Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia
            (Your Name) has sent you a message from European Respiratory Society
            (Your Name) thought you would like to see the European Respiratory Society web site.
            CAPTCHA
            This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
            Print
            Citation Tools
            Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia
            Keishi Sugino, Hirotaka Ono, Hiroshige Shimizu, Takeyuki Kurosawa, Keiko Matsumoto, Masahiro Ando, Kiyoshi Mori, Eiyasu Tsuboi, Sakae Homma, Kazuma Kishi
            ERJ Open Research Jan 2021, 7 (1) 00196-2020; DOI: 10.1183/23120541.00196-2020

            Citation Manager Formats

            • BibTeX
            • Bookends
            • EasyBib
            • EndNote (tagged)
            • EndNote 8 (xml)
            • Medlars
            • Mendeley
            • Papers
            • RefWorks Tagged
            • Ref Manager
            • RIS
            • Zotero
            Share
            Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia
            Keishi Sugino, Hirotaka Ono, Hiroshige Shimizu, Takeyuki Kurosawa, Keiko Matsumoto, Masahiro Ando, Kiyoshi Mori, Eiyasu Tsuboi, Sakae Homma, Kazuma Kishi
            ERJ Open Research Jan 2021, 7 (1) 00196-2020; DOI: 10.1183/23120541.00196-2020
            del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
            Full Text (PDF)

            Jump To

            • Article
              • Abstract
              • Abstract
              • Introduction
              • Methods
              • Results
              • Discussion
              • Acknowledgements
              • Footnotes
              • References
            • Figures & Data
            • Info & Metrics
            • PDF

            Subjects

            • Interstitial and orphan lung disease
            • Pulmonary pharmacology and therapeutics
            • Tweet Widget
            • Facebook Like
            • Google Plus One

            More in this TOC Section

            Original articles

            • Effect of β-blockers on the risk of COPD exacerbations
            • Recurrence of symptoms after childhood LRTI
            • BMI and in-hospital mortality in patients with AE-IPF
            Show more Original articles

            Interstitial lung disease

            • Weak correlation of quantitative HRCT with lung function change
            • No effect of danazol treatment in patients with advanced IPF
            • High prevalence of ILAs in middle-aged never-smokers
            Show more Interstitial lung disease

            Related Articles

            Navigate

            • Home
            • Current issue
            • Archive

            About ERJ Open Research

            • Editorial board
            • Journal information
            • Press
            • Permissions and reprints
            • Advertising

            The European Respiratory Society

            • Society home
            • myERS
            • Privacy policy
            • Accessibility

            ERS publications

            • European Respiratory Journal
            • ERJ Open Research
            • European Respiratory Review
            • Breathe
            • ERS books online
            • ERS Bookshop

            Help

            • Feedback

            For authors

            • Instructions for authors
            • Publication ethics and malpractice
            • Submit a manuscript

            For readers

            • Alerts
            • Subjects
            • RSS

            Subscriptions

            • Accessing the ERS publications

            Contact us

            European Respiratory Society
            442 Glossop Road
            Sheffield S10 2PX
            United Kingdom
            Tel: +44 114 2672860
            Email: journals@ersnet.org

            ISSN

            Online ISSN: 2312-0541

            Copyright © 2023 by the European Respiratory Society