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Review
. 2023 Sep;19(3):230101.
doi: 10.1183/20734735.0101-2023. Epub 2023 Sep 12.

Holistic management of patients with progressive pulmonary fibrosis

Ana Oliveira  1   2 , Gaia Fabbri  3 , Thomas Gille  4   5 , Elena Bargagli  3 , Boris Duchemann  4   6 , Rachel Evans  7 , Hilary Pinnock  8 , Anne E Holland  9 , Elisabetta Renzoni  10   11 , Magnus Ekström  12 , Steve Jones  13 , Marlies Wijsenbeek  14 , Anh Tuan Dinh-Xuan  15 , Guido Vagheggini  16   17
Affiliations
Review

Holistic management of patients with progressive pulmonary fibrosis

Ana Oliveira et al. Breathe (Sheff). 2023 Sep.

Abstract

Progressive pulmonary fibrosis (PF) is a complex interstitial lung disease that impacts substantially on patients' daily lives, requiring personalised and integrated care. We summarise the main needs of patients with PF and their caregivers, and suggest a supportive care approach. Individualised care, education, emotional and psychological support, specialised treatments, and better access to information and resources are necessary. Management should start at diagnosis, be tailored to the patient's needs, and consider end-of-life care. Pharmacological and non-pharmacological interventions should be individualised, including oxygen therapy and pulmonary rehabilitation, with digital healthcare utilised as appropriate. Further research is needed to address technical issues related to oxygen delivery and digital healthcare.

Educational aims: To identify the main needs of patients with PF and their caregivers.To describe the components of a comprehensive approach to a supportive care programme for patients with PF.To identify further areas of research to address technical issues related to the management of patients with PF.

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Conflict of interest statement

Conflict of interest: T. Gille reports personal fees from Boehringer Ingelheim, personal fees from Roche, non-financial support from Oxyvie (oxygen provider), non-financial support from LVL Medical (oxygen provider), non-financial support from Vitalaire (oxygen provider), non-financial support from Asten (oxygen provider), outside the submitted work. B. Duchemann reports personal payments for lectures, presentations, speakers bureaus, manuscript writing or educational events for Roche, Pfizer, AstraZeneca, Chiesi, Amgen, Lilly, Medscape, MSD and Sanofi, outside the submitted work; and support for attending meetings and/or travel from AZ, Pfizer, Oxyvie and MSD, outside the submitted work. R. Evans reports grants or contract from NIHR, UKRI, Wolfson Foundation, Genentec, and Roche, outside the submitted work; consulting fees from AstraZeneca, and Evidera for Long COVID, outside the submitted work; speaker fees from Boehringer and Moderna, outside the submitted work; support received from Chiesi to attend BTS virtually 2021, outside the submitted work; and ERS Group 01.02 Pulmonary Rehabilitation and Chronic Care Secretary and ATS Pulmonary Rehabilitation Assembly Chair, both unpaid, outside the submitted work. H. Pinnock reports speaker fees from TEVA pharmaceuticals, and Sandoz, outside the submitted work. A.E. Holland is President, Thoracic Society of Australia and New Zealand, unpaid position, outside the submitted work. E. Renzoni reports grants or contracts from Boehringer Ingelheim, outside the submitted work; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim and Chiesi, outside the submitted work; support for attending meetings and/or travel from Boehringer Ingelheim, outside the submitted work; and advisory boards for Boehringer Ingelheim and Roche, outside the submitted work. S. Jones reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from various pharmaceutical companies for tasks and participation in patient organisation advisory groups. All payments made to either the European Pulmonary Fibrosis Federation (EU-PFF) or the UK charity Action for Pulmonary Fibrosis, outside the submitted work; participation on a Data Safety Monitoring Board or Advisory Board for Galecto, outside the submitted work; and President of European Pulmonary Fibrosis Federation, and Chair of Trustees for Action for Pulmonary Fibrosis, outside the submitted work. M. Wijsenbeek reports grants or contracts from Boehringer Ingelheim, Hoffman la Roche, The Netherlands Organisation for Health Research and Development, The Dutch Lung Foundation, The Dutch Pulmonary Fibrosis patient association, the sarcoidosis patient association, and Astra Zeneca/Daiichi, outside the submitted work; consulting fees from AstraZeneca, Bristol Myers Squibb, Boehringer Ingelheim, CSL Behring, Galapagos, Galecto, Hoffman La Roche, Horizon Therapeutics, Kinevant Sciences, Molecure, Nerre Therapeutics, Novartis, PureTech Health, Respivant, Thyron, Trevi and Vicore, outside the submitted work; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim, CSL Behring, Galapagos, and Hoffman la Roche, outside the submitted work; support for attending meetings and/or travel from Boehringer Ingelheim, Galapagos and Hoffman la Roche, outside the submitted work; Monitoring Board or Advisory Board for Galapagos, outside the submitted work; Chair of the Idiopathic Interstitial Pneumonia group of the European Respiratory Society; Member of the board of the Netherlands Respiratory Society; Member of the scientific advisory board of the European Idiopathic Pulmonary Fibrosis and related disorders federation; and Chair of the educational committee of the European Reference Network for Rare Lung Diseases, unpaid positions, outside the submitted work. G. Vagheggini reports unrestricted educational grants from Chiesi Farmaceutici S.p.A and from A. Menarini Industrie Farmaceutiche Riunite srl. The remaining authors have nothing to disclose.

Figures

FIGURE 1
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
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
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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