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Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

Vincent Cottin, Nikhil A. Hirani, David L. Hotchkin, Anoop M. Nambiar, Takashi Ogura, María Otaola, Dirk Skowasch, Jong Sun Park, Hataya K. Poonyagariyagorn, Wim Wuyts, Athol U. Wells
European Respiratory Review 2018 27: 180076; DOI: 10.1183/16000617.0076-2018
Vincent Cottin
1Louis Pradel Hospital, Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, UMR 754, Université Claude Bernard Lyon 1, Lyon, France
11Co-lead authors of this paper
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Nikhil A. Hirani
2Edinburgh Lung Fibrosis Clinic and MRC Centre for Inflammation Research, The Queen's Medical Research Centre, The University of Edinburgh, Edinburgh, UK
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David L. Hotchkin
3Division of Pulmonary and Critical Care Medicine, Oregon Clinic, Portland, OR, USA
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Anoop M. Nambiar
4Division of Pulmonary and Critical Care Medicine, Dept of Medicine, University of Texas Health Science Center San Antonio and the South Texas Veterans Health Care System, San Antonio, TX, USA
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Takashi Ogura
5Kanagawa Cardiovascular and Respiratory Center, Kanagawa, Japan
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María Otaola
6Fundación FUNEF, Instituto de Rehabilitacion Psicofísica (IREP Hospital), Buenos Aires, Argentina
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Dirk Skowasch
7Dept of Internal Medicine II, Cardiology, Pneumology and Angiology, University Hospital Bonn, Bonn, Germany
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Jong Sun Park
8Division of Pulmonary and Critical Care Medicine, Dept of Internal Medicine and Lung Institute of Medical Research Center, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea
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Hataya K. Poonyagariyagorn
3Division of Pulmonary and Critical Care Medicine, Oregon Clinic, Portland, OR, USA
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Wim Wuyts
9Unit for Interstitial Lung Diseases, University Hospitals Leuven, Leuven, Belgium
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Athol U. Wells
10Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK
11Co-lead authors of this paper
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Figures

  • FIGURE 1
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    FIGURE 1

    Types of interstitial lung disease (ILD) most likely to have a progressive-fibrosing phenotype (indicated in bold). IIPs: idiopathic interstitial pneumonias. #: stage IV sarcoidosis only; ¶: not an established clinical diagnosis; +: e.g. asbestosis, silicosis.

  • FIGURE 2
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    FIGURE 2

    Diagnosis of fibrosing interstitial lung diseases (ILD) that may present a progressive phenotype. PFTs: pulmonary function test; HCRT: high-resolution computed tomography; BAL: bronchoalveolar lavage; MDD: multidisciplinary diagnosis; PF-ILD: progressive-fibrosing ILD.

  • FIGURE 3
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    FIGURE 3

    Nonspecific interstitial pneumonia: high-resolution computed tomography images from a 46-year-old male patient who underwent lung transplantation. a) The initial scan, taken at first admission, shows ground-glass attenuation and consolidation with reticulation and traction bronchiectasis along with a bronchovascular bundle, sparing the subpleural lung. b) A follow-up scan 3 years later showed increased ground-glass opacity and consolidation despite corticosteroids and immunosuppressive therapy. c) After another 3 years, a decrease in ground-glass opacity and consolidation was evident, together with increased traction bronchiectasis and cysts.

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Vol 27 Issue 150 Table of Contents
European Respiratory Review: 27 (150)
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Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
Vincent Cottin, Nikhil A. Hirani, David L. Hotchkin, Anoop M. Nambiar, Takashi Ogura, María Otaola, Dirk Skowasch, Jong Sun Park, Hataya K. Poonyagariyagorn, Wim Wuyts, Athol U. Wells
European Respiratory Review Dec 2018, 27 (150) 180076; DOI: 10.1183/16000617.0076-2018

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Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases
Vincent Cottin, Nikhil A. Hirani, David L. Hotchkin, Anoop M. Nambiar, Takashi Ogura, María Otaola, Dirk Skowasch, Jong Sun Park, Hataya K. Poonyagariyagorn, Wim Wuyts, Athol U. Wells
European Respiratory Review Dec 2018, 27 (150) 180076; DOI: 10.1183/16000617.0076-2018
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • Diagnostic methods
    • Presentation and clinical course of fibrosing ILDs that may present a progressive phenotype, other than IPF
    • Conclusion
    • Acknowledgements
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
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  • Interstitial and orphan lung disease
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