Idiopathic pulmonary fibrosis in the United Kingdom: findings from the first decade of the largest single-country IPF registry

Fahim, Ahmed, Loughenbury, Maria and Agnew, Sarah , et al. (2024) Idiopathic pulmonary fibrosis in the United Kingdom: findings from the first decade of the largest single-country IPF registry. European Respiratory Journal, 64. (doi:10.1183/13993003.congress-2024.PA5147).

Abstract

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is the most common progressive interstitial lung disease (ILD) and has a dismal prognosis. We reviewed data from the UK ILD Registry and present changes over time.

Methods: participating centres entered data onto a central electronic database between 1 January 2013 and 19 February 2023. Informed consent was obtained by all participants. A descriptive analysis of these data was undertaken.

Results: data were available from 5052 cases from 87 centres. There was a male preponderance (77.8%) with mean age of 74.0 years (± 8.1). Overall, 66% of patients were ex-smokers and 76% had at least one co-morbidity. Over a third (36.7%) experienced a delay of more than 24 months before first clinic visit, and the average wait time from referral to first clinic visit was 96 days (for the last 12 months of data collection this rose to 100 days). At first clinic visit almost all patients had GAP Stage I (63%) or Stage II (36%) disease, with only 1% having Stage III. Pirfenidone (approved for use in April 2013) was initially used in around a third of cases, with its use dropping by around 10 percentage points after nintedanib was approved in January 2016. As pirfenidone use dropped nintedanib use rose from 6.7% in 2013 to 31.5% in 2022. At presentation 7.5% were recruited to a clinical trial, rising to 21.7% of cases at any time during follow up.

Conclusion: these data reflect clinical practice across ILD centres in the UK and show changes of care over the last decade. We have shown increasing use of antifibrotic therapy but a persistent delay in referral to clinic review. Greater service provision is required for these patients.

This record has no associated files available for download.

Contributors

Author: Sophie Fletcher

Download statistics