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

Giles Dixon; Samuel Hague; Sarah Mulholland; Huzaifa Adamali; Aye Myat Noe Khin; Hannah Thould; Roisin Connon; Paul Minnis; Eoin Murtagh; Fasihul Khan; Sameen Toor; Alexandra Lawrence; Marium Naqvi; Alex West; Robina K Coker; Katie Ward; Leda Yazbeck; Simon Hart; Theresa Garfoot; Kate Newman; Pilar Rivera-Ortega; Lachlan Stranks; Paul Beirne; Jessica Bradley; Catherine Rowan; Sarah Agnew; Mahin Ahmad; Lisa G Spencer; Joshua Aigbirior; Ahmed Fahim; Andrew M Wilson; Elizabeth Butcher; Sy Giin Chong; Gauri Saini; Sabrina Zulfikar; Felix Chua; Peter M George; Maria Kokosi; Vasileios Kouranos; Philip Molyneaux; Elisabetta Renzoni; Benedetta Vitri; Athol U Wells; Lisa M Nicol; Stephen Bianchi; Raman Kular; HuaJian Liu; Alexander John; Sarah Barth; Melissa Wickremasinghe; Ian A Forrest; Ian Grimes; A John Simpson; Sophie V Fletcher; Mark G Jones; Emma Kinsella; Jennifer Naftel; Nicola Wood; Jodie Chalmers; Anjali Crawshaw; Louise E Crowley; Davinder Dosanjh; Christopher C Huntley; Gareth I Walters; Timothy Gatheral; Catherine Plum; Shiva Bikmalla; Raja Muthusami; Helen Stone; Jonathan C L Rodrigues; Krasimira Tsaneva-Atanasova; Chris J Scotton; Michael A Gibbons; Shaney L Barratt

doi:10.1183/23120541.00529-2023

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

ERJ Open Res. 2024 Jan 15;10(1):00529-2023. doi: 10.1183/23120541.00529-2023. eCollection 2024 Jan.

Authors

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.

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.

Grants and funding

WT_/Wellcome Trust/United Kingdom