Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK

doi:10.1183/23120541.00529-2023

. 2024 Jan 15;10(1):00529-2023.

doi: 10.1183/23120541.00529-2023. eCollection 2024 Jan.

Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK

Giles Dixon^{1

2

3

4}, Samuel Hague¹, Sarah Mulholland¹, Huzaifa Adamali¹, Aye Myat Noe Khin², Hannah Thould², Roisin Connon⁵, Paul Minnis⁵, Eoin Murtagh⁵, Fasihul Khan⁶, Sameen Toor⁶, Alexandra Lawrence⁷, Marium Naqvi⁷, Alex West⁷, Robina K Coker⁸, Katie Ward⁸, Leda Yazbeck⁸, Simon Hart⁹, Theresa Garfoot¹⁰, Kate Newman¹⁰, Pilar Rivera-Ortega¹⁰, Lachlan Stranks¹⁰, Paul Beirne¹¹, Jessica Bradley¹¹, Catherine Rowan¹¹, Sarah Agnew¹², Mahin Ahmad¹², Lisa G Spencer¹², Joshua Aigbirior¹³, Ahmed Fahim¹³, Andrew M Wilson^{14

15}, Elizabeth Butcher¹⁶, Sy Giin Chong¹⁶, Gauri Saini¹⁶, Sabrina Zulfikar¹⁷, Felix Chua¹⁸, Peter M George¹⁸, Maria Kokosi¹⁸, Vasileios Kouranos¹⁸, Philip Molyneaux¹⁸, Elisabetta Renzoni¹⁸, Benedetta Vitri¹⁸, Athol U Wells¹⁸, Lisa M Nicol¹⁹, Stephen Bianchi²⁰, Raman Kular²⁰, HuaJian Liu²¹, Alexander John²¹, Sarah Barth²², Melissa Wickremasinghe²², Ian A Forrest²³, Ian Grimes²³, A John Simpson^{23

24}, Sophie V Fletcher^{25

26}, Mark G Jones^{25

26}, Emma Kinsella²⁵, Jennifer Naftel²⁵, Nicola Wood²⁵, Jodie Chalmers²⁷, Anjali Crawshaw²⁷, Louise E Crowley²⁷, Davinder Dosanjh²⁷, Christopher C Huntley^{27

28}, Gareth I Walters^{27

28}, Timothy Gatheral²⁹, Catherine Plum²⁹, Shiva Bikmalla³⁰, Raja Muthusami³⁰, Helen Stone³⁰, Jonathan C L Rodrigues^{4

31}, Krasimira Tsaneva-Atanasova^{32

33

34}, Chris J Scotton³, Michael A Gibbons^{2

3

35}, Shaney L Barratt^{1

35}

Affiliations

¹ Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UK.
² South West Peninsula ILD Network, Royal Devon University Healthcare NHS Foundation Trust, Exeter, UK.
³ Department of Clinical and Biomedical Sciences, University of Exeter, Exeter, UK.
⁴ Royal United Hospitals Bath NHS Foundation Trust, Bath, UK.
⁵ Antrim Area Hospital, Northern Health and Social Care Trust, Antrim, UK.
⁶ Glenfield Hospital, University Hospitals of Leicester NHS Trust, Leicester, UK.
⁷ Guy's and St Thomas' Hospital NHS Foundation Trust, London, UK.
⁸ Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
⁹ Hull University Teaching Hospitals NHS Trust, Hull, UK.
¹⁰ Interstitial Lung Disease Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK.
¹¹ Leeds Teaching Hospitals NHS Trust, Leeds, UK.
¹² Liverpool Interstitial Lung Disease Service, Aintree Hospital, Liverpool University Hospital NHS FT, Liverpool, UK.
¹³ New Cross Hospital, The Royal Wolverhampton NHS Trust, Wolverhampton, UK.
¹⁴ Norfolk and Norwich University Hospital NHS Foundation Trust, UK.
¹⁵ Norwich Medical School, University of East Anglia, Norwich, UK.
¹⁶ Nottingham University Hospitals NHS Trust, Nottingham, UK.
¹⁷ Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
¹⁸ Royal Brompton and Harefield Hospitals, London, UK.
¹⁹ Royal Infirmary of Edinburgh, Edinburgh, UK.
²⁰ Sheffield Teaching Hospital NHS Foundation Trust, Sheffield, UK.
²¹ Southern Health and Social Care Trust, Portadown, UK.
²² St Mary's Hospital, Imperial College Healthcare NHS Trust, London, UK.
²³ The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK.
²⁴ Newcastle University, Newcastle upon Tyne, UK.
²⁵ University Hospital of Southampton NHS Foundation Trust, Southampton, UK.
²⁶ NIHR Southampton Respiratory Biomedical Research Centre and School of Clinical and Experimental Sciences, Faulty of Medicine, University of Southampton, Southampton, UK.
²⁷ University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
²⁸ Institute of Applied Health Research, University of Birmingham, Birmingham, UK.
²⁹ University Hospitals of Morecambe Bay NHS Foundation Trust, Lancashire and South Cumbria ILD Service, Lancaster, UK.
³⁰ University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, UK.
³¹ Department of Health, University of Bath, Bath, UK.
³² Department of Mathematics and Statistics, Faculty of Environment, Science and Economy, University of Exeter, Exeter, UK.
³³ EPSRC Hub for Quantitative Modelling in Healthcare, University of Exeter, Exeter, UK.
³⁴ Living Systems Institute, University of Exeter, Exeter, UK.
³⁵ These authors contributed equally.

PMID: 38226064
PMCID: PMC10789269
DOI: 10.1183/23120541.00529-2023

Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK

Giles Dixon et al. ERJ Open Res. 2024.

. 2024 Jan 15;10(1):00529-2023.

doi: 10.1183/23120541.00529-2023. eCollection 2024 Jan.

Authors

Affiliations

¹ Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UK.
² South West Peninsula ILD Network, Royal Devon University Healthcare NHS Foundation Trust, Exeter, UK.
³ Department of Clinical and Biomedical Sciences, University of Exeter, Exeter, UK.
⁴ Royal United Hospitals Bath NHS Foundation Trust, Bath, UK.
⁵ Antrim Area Hospital, Northern Health and Social Care Trust, Antrim, UK.
⁶ Glenfield Hospital, University Hospitals of Leicester NHS Trust, Leicester, UK.
⁷ Guy's and St Thomas' Hospital NHS Foundation Trust, London, UK.
⁸ Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, UK.
⁹ Hull University Teaching Hospitals NHS Trust, Hull, UK.
¹⁰ Interstitial Lung Disease Unit, Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK.
¹¹ Leeds Teaching Hospitals NHS Trust, Leeds, UK.
¹² Liverpool Interstitial Lung Disease Service, Aintree Hospital, Liverpool University Hospital NHS FT, Liverpool, UK.
¹³ New Cross Hospital, The Royal Wolverhampton NHS Trust, Wolverhampton, UK.
¹⁴ Norfolk and Norwich University Hospital NHS Foundation Trust, UK.
¹⁵ Norwich Medical School, University of East Anglia, Norwich, UK.
¹⁶ Nottingham University Hospitals NHS Trust, Nottingham, UK.
¹⁷ Oxford University Hospitals NHS Foundation Trust, Oxford, UK.
¹⁸ Royal Brompton and Harefield Hospitals, London, UK.
¹⁹ Royal Infirmary of Edinburgh, Edinburgh, UK.
²⁰ Sheffield Teaching Hospital NHS Foundation Trust, Sheffield, UK.
²¹ Southern Health and Social Care Trust, Portadown, UK.
²² St Mary's Hospital, Imperial College Healthcare NHS Trust, London, UK.
²³ The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK.
²⁴ Newcastle University, Newcastle upon Tyne, UK.
²⁵ University Hospital of Southampton NHS Foundation Trust, Southampton, UK.
²⁶ NIHR Southampton Respiratory Biomedical Research Centre and School of Clinical and Experimental Sciences, Faulty of Medicine, University of Southampton, Southampton, UK.
²⁷ University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
²⁸ Institute of Applied Health Research, University of Birmingham, Birmingham, UK.
²⁹ University Hospitals of Morecambe Bay NHS Foundation Trust, Lancashire and South Cumbria ILD Service, Lancaster, UK.
³⁰ University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, UK.
³¹ Department of Health, University of Bath, Bath, UK.
³² Department of Mathematics and Statistics, Faculty of Environment, Science and Economy, University of Exeter, Exeter, UK.
³³ EPSRC Hub for Quantitative Modelling in Healthcare, University of Exeter, Exeter, UK.
³⁴ Living Systems Institute, University of Exeter, Exeter, UK.
³⁵ These authors contributed equally.

PMID: 38226064
PMCID: PMC10789269
DOI: 10.1183/23120541.00529-2023

Abstract

Background: Nintedanib slows progression of lung function decline in patients with progressive fibrosing (PF) interstitial lung disease (ILD) and was recommended for this indication within the United Kingdom (UK) National Health Service in Scotland in June 2021 and in England, Wales and Northern Ireland in November 2021. To date, there has been no national evaluation of the use of nintedanib for PF-ILD in a real-world setting.

Methods: 26 UK centres were invited to take part in a national service evaluation between 17 November 2021 and 30 September 2022. Summary data regarding underlying diagnosis, pulmonary function tests, diagnostic criteria, radiological appearance, concurrent immunosuppressive therapy and drug tolerability were collected via electronic survey.

Results: 24 UK prescribing centres responded to the service evaluation invitation. Between 17 November 2021 and 30 September 2022, 1120 patients received a multidisciplinary team recommendation to commence nintedanib for PF-ILD. The most common underlying diagnoses were hypersensitivity pneumonitis (298 out of 1120, 26.6%), connective tissue disease associated ILD (197 out of 1120, 17.6%), rheumatoid arthritis associated ILD (180 out of 1120, 16.0%), idiopathic nonspecific interstitial pneumonia (125 out of 1120, 11.1%) and unclassifiable ILD (100 out of 1120, 8.9%). Of these, 54.4% (609 out of 1120) were receiving concomitant corticosteroids, 355 (31.7%) out of 1120 were receiving concomitant mycophenolate mofetil and 340 (30.3%) out of 1120 were receiving another immunosuppressive/modulatory therapy. Radiological progression of ILD combined with worsening respiratory symptoms was the most common reason for the diagnosis of PF-ILD.

Conclusion: We have demonstrated the use of nintedanib for the treatment of PF-ILD across a broad range of underlying conditions. Nintedanib is frequently co-prescribed alongside immunosuppressive and immunomodulatory therapy. The use of nintedanib for the treatment of PF-ILD has demonstrated acceptable tolerability in a real-world setting.

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Conflict of interest statement

Conflict of interest: A.J. Simpson has received funding to his institution from Boehringer Ingelheim (BI) to undertake an educational meeting. A. West has received support from BI for speaking at or chairing educational events, and attendance and travel to educational meetings; and is part of an advisory board for BI and Avalyn Pharmaceuticals. A. John has received funding from BI to attend an educational event. A.M. Wilson has received grants from Aseptika, Brainomix and BASF, has received speakers’ fees from BI, has received support for attending meetings by Chiesi, and has institutional interests with Celgene Corporation, GSK and Insmed Inc. A. Crawshaw has received speakers’ fees from BI and AstraZeneca (AZ). A.U. Wells has undertaken advisory board activity and consultant work for BI, Roche and Veracyte. C.C. Huntley has received an honorarium for educational content from BI and sponsorship for conference attendance. D. Dosanjh has received a speaker's fee from BI, meeting attendance costs from AZ and is part of the advisory board for AZ, Gilead, BI and Synairgen. E. Renzoni has received institutional funding, honoraria for educational events and funding for conference attendance from BI, and is member of the advisory board for BI and Roche. F. Chua has received consulting fees, honoraria, support for conference attendance and is an advisory board member for BI. G. Saini has received institutional payment for educational presentation from BI. G. Dixon, H. Stone, L.M. Nicol and I.A. Forrest have received support for educational event attendance from BI. J.C.L. Rodrigues has received grant funding from NIHR, consulting fees from NHSx and HeartFlow, honoraria from Sanofi, Aidence and 4-C Research market research, meeting attendance support from Aidence and HeartFlow, leadership role in Heart and Lung Imaging LTD (HLH), stock in Radnet and shares in HLH. K. Tsaneva-Atanasova has financial support from EPSRC grant. M. Naqvi has received a grant from NHS Digital, honoraria from BI, AZ and Roche, support for meeting attendance from BI and advisory board membership for BI, and is ILD Pharmacist Network Chair and ILD-IN Co-chair. M.G. Jones has received grants from Royal Society, BI, NC3Rs, MRC, AAIR Charity and the British Lung Foundation. P.M. George has received an institutional grant from BI, honoraria from BI, Roche, Teva, Cipla and Brainomix, meeting attendance support from BI and Roche and has stock in Brainomix. P. Molyneaux has grant funding from AZ, consulting fees from Roche, BI, AZ, Trevi and Qureight, and honoraria from BI and Roche; and is an associate editor of this journal. P. Rivera-Ortega has received grant funding from MRC, institutional grant funding from BI, Roche, CSL Behring, Fibrogen, Vicore Pharma AB, Gilead Sciences and Galecto, consulting fees from BI and Roche, honoraria from BI, Roche and Respiratory Effectiveness Group (REG), support for meeting attendance from BI and REG, is a chair of the REG and member of the Global Writing Group Committee for REMAP-ILD. R.K. Coker has received honoraria from BI. S. Agnew has received honoraria from BI, support for meeting attendance from BI and is member of the BTS ILD registry advisory board. S.L. Barratt has received consulting fees and honoraria from BI. S. Hart has received research grant from BI, consulting fees from Trevi Therapeutics, honoraria and support for meeting attendance from BI and Chiesi, was Chair of the BTS Standard of Care Committee 2019–2022, and is a Trustee of Action for Pulmonary Fibrosis and an associate editor of this journal. S. Barth received honoraria from BI for educational meeting facilitating. T. Garfoot received support to attend the ILD IN annual conference. T. Gatheral has received speakers’ fees from BI. Conflict of interest: The remaining authors have no competing interests.

Figures

**FIGURE 1**
Primary diagnosis of 1120 patients with an interstitial lung disease (ILD) multidisciplinary team decision to commence nintedanib between 17 November 2021 and 30 September 2022. RA: rheumatoid arthritis; NSIP: nonspecific interstitial pneumonia; SSc: systemic sclerosis; MCTD: mixed connective tissue disease; CPFE: combined pulmonary fibrosis and emphysema syndrome; PF: pulmonary fibrosis. ^#: the predominant diagnoses included in “other ILD” were pleuroparenchymal fibroelastosis in 19 (1.7%) out of 1120, other connective tissue disease-related ILD in 11 (1.0%) out of 1120, fibrotic organising pneumonia in seven (0.6%) out of 1120, interstitial pneumonia with autoimmune features in six (0.5%) out of 1120, asbestosis in five (0.4%) out of 1120, desquamative interstitial pneumonia in four (0.4%) out of 1120, post-coronavirus disease 2019 ILD in three (0.3%) out of 1120 and sarcoidosis in two (0.2%) out of 1120.

**FIGURE 2**
Diagnostic criteria for progressive fibrosing interstitial lung disease (ILD) in 1120 patients with an ILD multidisciplinary team decision to commence nintedanib between 17 November 2021 and 30 September 2022. FVC: forced vital capacity; HRCT: high-resolution computed tomography.

**FIGURE 3**
Percentage predicted forced vital capacity (FVC) and transfer capacity of the lung for carbon monoxide (D_LCO) for 1120 patients with an interstitial lung disease multidisciplinary team decision decision to commence nintedanib between 17 November 2021 and 30 September 2022.

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References

1. Cottin V, Hirani NA, Hotchkin DL, et al. . Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27: 180076. doi:10.1183/16000617.0076-2018 - DOI - PMC - PubMed
1. Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med 2020; 383: 958–968. doi:10.1056/NEJMra2005230 - DOI - PubMed
1. Hambly N, Farooqi MM, Dvorkin-Gheva A, et al. . Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry. Eur Respir J 2022; 60: 2102571. doi:10.1183/13993003.02571-2021 - DOI - PubMed
1. Flaherty KR, Wells AU, Cottin V, et al. . Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019; 381: 1718–1727. doi:10.1056/NEJMoa1908681 - DOI - PubMed
1. Richeldi L, du Bois RM, Raghu G, et al. . Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071–2082. doi:10.1056/NEJMoa1402584 - DOI - PubMed

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[1] Cottin V, Hirani NA, Hotchkin DL, et al. . Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27: 180076. doi:10.1183/16000617.0076-2018 - DOI - PMC - PubMed

[2] Cottin V, Hirani NA, Hotchkin DL, et al. . Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 2018; 27: 180076. doi:10.1183/16000617.0076-2018 - DOI - PMC - PubMed

[3] Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med 2020; 383: 958–968. doi:10.1056/NEJMra2005230 - DOI - PubMed

[4] Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med 2020; 383: 958–968. doi:10.1056/NEJMra2005230 - DOI - PubMed

[5] Hambly N, Farooqi MM, Dvorkin-Gheva A, et al. . Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry. Eur Respir J 2022; 60: 2102571. doi:10.1183/13993003.02571-2021 - DOI - PubMed

[6] Hambly N, Farooqi MM, Dvorkin-Gheva A, et al. . Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry. Eur Respir J 2022; 60: 2102571. doi:10.1183/13993003.02571-2021 - DOI - PubMed

[7] Flaherty KR, Wells AU, Cottin V, et al. . Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019; 381: 1718–1727. doi:10.1056/NEJMoa1908681 - DOI - PubMed

[8] Flaherty KR, Wells AU, Cottin V, et al. . Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med 2019; 381: 1718–1727. doi:10.1056/NEJMoa1908681 - DOI - PubMed

[9] Richeldi L, du Bois RM, Raghu G, et al. . Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071–2082. doi:10.1056/NEJMoa1402584 - DOI - PubMed

[10] Richeldi L, du Bois RM, Raghu G, et al. . Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 2014; 370: 2071–2082. doi:10.1056/NEJMoa1402584 - DOI - PubMed

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Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK

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Real-world experience of nintedanib for progressive fibrosing interstitial lung disease in the UK

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Abstract

Conflict of interest statement

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