Abstract
Objectives
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease (ILD) and the most common idiopathic interstitial pneumonia. The UK IPF Registry was established in 2013 to collect data pertaining to clinical features, therapeutic approaches and outcomes. From February 2023, the Registry expanded to include any ILD with evidence of fibrosis.
Design
The UK IPF Registry is a national, multicentre observational registry, including both prospective and retrospective data of patients with IPF in secondary or tertiary care. Cases eligible for inclusion were those with a diagnosis of IPF, presenting at participating centres from January 2013.
Results
Between January 2013 and February 2023, 5052 IPF cases were registered from 64 participating centres. There was a male preponderance (77.8%) with mean±SD age of 74±8.1 years, 66% were ex-smokers and 76% had at least one comorbidity. Over a third (36.7%) experienced symptoms for more than 24 months prior to their first clinic visit. The majority of cases were discussed at a multidisciplinary team (MDT) meeting and the most common radiological patterns at presentation were probable (54.6%) and definite (42.7%) usual interstitial pneumonia. There was a reduction in surgical lung biopsies from 14% in 2013 to 5.5% in 2022. Antifibrotic therapy prescription rose from 36.0% in 2013 to 55.9% in 2023. The use of nintedanib (approved by National Institute of Clinical Excellence in January 2016) rose from 6.7% in 2013 to 31.5% in 2022 and pirfenidone (approved in April 2013) was initially used in around a third of cases before dropping to between 16.8% and 24.9% after nintedanib was approved.
Conclusion
These data reflect clinical practice across the UK and it is intended the data will have a role in informing the future of IPF care and providing a model for benchmarking, ultimately increasing knowledge and improving clinical care for this devastating disease.
Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease (ILD) and the most common idiopathic interstitial pneumonia. The UK IPF Registry was established in 2013 to collect data pertaining to clinical features, therapeutic approaches and outcomes. From February 2023, the Registry expanded to include any ILD with evidence of fibrosis.
Design
The UK IPF Registry is a national, multicentre observational registry, including both prospective and retrospective data of patients with IPF in secondary or tertiary care. Cases eligible for inclusion were those with a diagnosis of IPF, presenting at participating centres from January 2013.
Results
Between January 2013 and February 2023, 5052 IPF cases were registered from 64 participating centres. There was a male preponderance (77.8%) with mean±SD age of 74±8.1 years, 66% were ex-smokers and 76% had at least one comorbidity. Over a third (36.7%) experienced symptoms for more than 24 months prior to their first clinic visit. The majority of cases were discussed at a multidisciplinary team (MDT) meeting and the most common radiological patterns at presentation were probable (54.6%) and definite (42.7%) usual interstitial pneumonia. There was a reduction in surgical lung biopsies from 14% in 2013 to 5.5% in 2022. Antifibrotic therapy prescription rose from 36.0% in 2013 to 55.9% in 2023. The use of nintedanib (approved by National Institute of Clinical Excellence in January 2016) rose from 6.7% in 2013 to 31.5% in 2022 and pirfenidone (approved in April 2013) was initially used in around a third of cases before dropping to between 16.8% and 24.9% after nintedanib was approved.
Conclusion
These data reflect clinical practice across the UK and it is intended the data will have a role in informing the future of IPF care and providing a model for benchmarking, ultimately increasing knowledge and improving clinical care for this devastating disease.
| Original language | English |
|---|---|
| Article number | e002773 |
| Pages (from-to) | 1-9 |
| Number of pages | 9 |
| Journal | BMJ Open Respiratory Research |
| Volume | 12 |
| Issue number | 1 |
| Early online date | 19 Feb 2025 |
| DOIs | |
| Publication status | Published (in print/issue) - 19 Feb 2025 |
Bibliographical note
© Author(s) (or their employer(s)) 2025. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group.Data Access Statement
Data may be obtained from a third party and are not publicly available. Researchers may apply to access UK Interstitial Lung Disease Registry data directly from the British Thoracic Society through the Society’s data access request process. For further information please visit https://www.brit- thoracic.org.uk/quality-improvement/bts-clinical-data-policy-and-data-access/.Keywords
- Idiopathic Pulmonary Fibrosis
- Interstitial Fibrosis
- Anti-Inflammatory Agents, Non-Steroidal
- Prospective Studies
- Humans
- Lung
- Middle Aged
- Pyridones
- Male
- United Kingdom
- idiopathic pulmonary fibrosis
- Interstitial fibrosis
- Aged, 80 and over
- Female
- Registries
- Aged
- Retrospective Studies
- Indoles
- Anti-Inflammatory Agents, Non-Steroidal/therapeutic use
- Pyridones/therapeutic use
- Indoles/therapeutic use
- United Kingdom/epidemiology
- Lung/pathology
- Idiopathic Pulmonary Fibrosis/therapy