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Holistic management of patients with progressive pulmonary fibrosis

Ana Oliveira, Gaia Fabbri, Thomas Gille, Elena Bargagli, Boris Duchemann, Rachel Evans, Hilary Pinnock, Anne E. Holland, Elisabetta Renzoni, Magnus Ekström, Steve Jones, Marlies Wijsenbeek, Anh Tuan Dinh-Xuan, Guido Vagheggini
Breathe 2023 19: 230101; DOI: 10.1183/20734735.0101-2023
Ana Oliveira
1Lab 3R Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), IBMED Aveiro PT, Aveiro, Portugal
2School of Rehabilitation Science, Faculty of Health Science, McMaster University, Hamilton, ON, Canada
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  • ORCID record for Ana Oliveira
Gaia Fabbri
3Respiratory Diseases and Lung Transplantation Unit, Department of Medical and Surgical Sciences and Neuro-Sciences, University of Siena, Siena, Italy
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Thomas Gille
4Inserm U1272 “Hypoxia & the Lung”, UFR SMBH Léonard de Vinci, Université Sorbonne Paris Nord, Bobigny, France
5Service Physiologie et Explorations Fonctionnelles, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Assistance Publique – Hôpitaux de Paris, Bobigny, France
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Elena Bargagli
3Respiratory Diseases and Lung Transplantation Unit, Department of Medical and Surgical Sciences and Neuro-Sciences, University of Siena, Siena, Italy
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Boris Duchemann
4Inserm U1272 “Hypoxia & the Lung”, UFR SMBH Léonard de Vinci, Université Sorbonne Paris Nord, Bobigny, France
6Service d'oncologie médicale et thoracique, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Assistance Publique – Hôpitaux de Paris, Bobigny, France
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Rachel Evans
7Department of Respiratory Sciences, University of Leicester, Glenfield Hospital, Leicester, UK
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Hilary Pinnock
8Allergy and Respiratory Research Group, Usher Institute, The University of Edinburgh, Edinburgh, UK
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Anne E. Holland
9Central Clinical School, Monash University and Department of Physiotherapy, Alfred Health, Melbourne, Australia
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Elisabetta Renzoni
10Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas’ NHS Foundation Trust, London, UK
11Margaret Turner Warwick Centre for Fibrosing Lung Diseases, NHLI, Imperial College, London, UK
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Magnus Ekström
12Lund University, Faculty of Medicine, Department of Clinical Sciences Lund, Respiratory Medicine, Allergology and Palliative Medicine, Lund, Sweden
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Steve Jones
13European Pulmonary Fibrosis Federation, Brussels, Belgium
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Marlies Wijsenbeek
14Centre for Expertise for Interstitial Lung Disease and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands
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Anh Tuan Dinh-Xuan
15Service de Physiologie-Explorations Fonctionnelles, Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Cité, Paris, France
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Guido Vagheggini
16Department of Internal Medicine and Medical Specialties, Respiratory Failure Pathway, Azienda USL Toscana Nordovest, Pisa, Italy
17Fondazione Volterra Ricerche ONLUS, Volterra (PI), Italy
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  • For correspondence: guidovagheggini@libero.it
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  • FIGURE 1
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    FIGURE 1

    Illustration of the different types of interstitial lung disease (ILD) associated with progressive pulmonary fibrosis. ILDs can be broadly categorised into connective tissue disease (CTD)-ILDs, other ILDs, and unclassifiable ILDs (uILDs). The CTD-ILDs are further sub-categorised based on the type of CTD, including rheumatoid arthritis-associated ILD, systemic sclerosis-associated ILD, mixed CTD-associated ILD, and other autoimmune ILDs. Other ILDs include exposure related ILDs (e.g. asbestosis and silicosis), non-idiopathic pulmonary fibrosis (such as genetic and/or familial pulmonary fibrosis (g/f PF)), idiopathic interstitial pneumonias (such as desquamative interstitial pneumonia), and others. HP: hypersensitivity pneumonitis; iNSIP: idiopathic nonspecific interstitial pneumonia; IPAF: interstitial pneumonia with autoimmune features; IPF: idiopathic pulmonary fibrosis.

  • FIGURE 2
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    FIGURE 2

    Summary of the current knowledge, gaps and opportunities for supportive care in needs identified by patients with pulmonary fibrosis. f-ILD: interstitial lung diseases associated with progressive pulmonary fibrosis; PR: pulmonary rehabilitation.

  • FIGURE 3
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    FIGURE 3

    Illustration of potential effects of supplemental oxygen in a patient with pulmonary fibrosis and hypoxaemia during exertion. Green lines represent potential responses with supplemental oxygen, and blue and red lines the potential responses without supplemental oxygen. Responses are not observed, the data for illustrational purposes only. Adapted from the presentation given by Magnus Ekström (Lund, Sweden) at the 2022 European Respiratory Society International Congress, with permission from the author.

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Vol 19 Issue 3 Table of Contents
Breathe: 19 (3)
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Holistic management of patients with progressive pulmonary fibrosis
Ana Oliveira, Gaia Fabbri, Thomas Gille, Elena Bargagli, Boris Duchemann, Rachel Evans, Hilary Pinnock, Anne E. Holland, Elisabetta Renzoni, Magnus Ekström, Steve Jones, Marlies Wijsenbeek, Anh Tuan Dinh-Xuan, Guido Vagheggini
Breathe Sep 2023, 19 (3) 230101; DOI: 10.1183/20734735.0101-2023

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Holistic management of patients with progressive pulmonary fibrosis
Ana Oliveira, Gaia Fabbri, Thomas Gille, Elena Bargagli, Boris Duchemann, Rachel Evans, Hilary Pinnock, Anne E. Holland, Elisabetta Renzoni, Magnus Ekström, Steve Jones, Marlies Wijsenbeek, Anh Tuan Dinh-Xuan, Guido Vagheggini
Breathe Sep 2023, 19 (3) 230101; DOI: 10.1183/20734735.0101-2023
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