A recent publication by the British Thoracic Society (BTS) offers the first long-term analysis of the UK Idiopathic Pulmonary Fibrosis (IPF) Registry.
IPF is a progressive interstitial lung disease marked by a significant symptom burden and a poor prognosis. While disease progression is variable, accurately identifying patients at risk for more rapid decline remains difficult.
The IPF Registry collects real-world data on disease progression, clinical features, therapeutic approaches, and patient outcomes. Established in 2013, it is a national, multicentre, observational registry that includes both prospective and retrospective IPF cases across the UK.
Data on 5,052 IPF cases across 64 UK hospitals, including 13 specialist centres were analysed. The cohort had a mean age of 74, the majority (77.8%) were male and 76% presented with at least one comorbidity. Two thirds (66%) were ex-smokers, 93.9% had no family history of IPF and 10% had pulmonary hypertension.
Key IPF findings
The researchers found that referral trends shifted from primary to secondary care over time, with the mean wait from referral to first clinic visit at just over 13 weeks; 60% of patients reported symptoms for over a year before the first clinic visit.
Diagnosis of IPF was primarily through high-resolution computed tomography, with 97% showing interstitial pneumonia patterns. At presentation, 48% of patients were prescribed antifibrotic treatments, with nintedanib use surpassing pirfenidone from 2017 onwards.
Mortality was 31% and, over time, increasing numbers were deemed ineligible for lung transplantation. Long wait times for diagnosis and treatment were noted as a challenge for optimal IPF management within the NHS.
Reflecting on the impact of their research, the researchers wrote: ‘It is paramount to have large-scale, longitudinal data sets for patients with IPF because, without such data, it would be challenging to identify and quantify unmet needs.’
Lead author Dr Ahmed Fahim added: ‘It is exciting to present a decade of data from the BTS Registry and highlighting the importance of workforce expansion to deliver high-quality care and improve clinical outcomes.’
Study authors emphasised that delayed diagnosis, timely access to treatment and insufficient involvement in clinical research are important factors impacting care quality for this patient population.
They anticipate that their analysis will help shape the future of IPF care by providing a benchmarking model and improving both knowledge and clinical management.
Reference
Fahim A et al. Idiopathic pulmonary fibrosis in the UK: findings from the British Thoracic Society UK Idiopathic Pulmonary Fibrosis Registry. BMJ Open Respir Res 2025;12:e002773.
