The UK IPF Registry has provided new insights into the evolving landscape of idiopathic pulmonary fibrosis (IPF) management, showing significant shifts in diagnosis and treatment approaches over the last decade.
Established in 2013, the national, multicentre registry includes both prospective and retrospective data of patients diagnosed with IPF across 64 centres in the UK. Eligible cases were those with a diagnosis of IPF, presenting at secondary or tertiary care centres from January 2013. The registry was expanded in February 2023 to include interstitial lung diseases with evidence of fibrosis.
Between January 2013–February 2023, 5,052 cases of IPF were registered. The cohort had a clear male predominance, with 77.8% of patients being male, and the mean age was 74±8.1 years. The majority of patients were ex-smokers (66%) and had at least one comorbidity (76%). Over a third of patients (36.7%) had experienced symptoms for more than 24 months before their first clinic visit. Most cases were discussed at a multidisciplinary team meeting, and the most common radiological patterns at presentation were probable (54.6%) and definite (42.7%) usual interstitial pneumonia.
A notable shift occurred in diagnostic practices, with the percentage of patients undergoing surgical lung biopsies falling from 14% in 2013 to 5.5% in 2022. The prescription of antifibrotic therapy increased substantially from 36.0% in 2013 to 55.9% in 2023. The use of nintedanib, approved by the National Institute of Clinical Excellence in 2016, grew from 6.7% in 2013 to 31.5% in 2022, while pirfenidone usage fluctuated between 16.8% and 24.9% after nintedanib’s approval.
The data reflect ongoing clinical practice across the UK and are expected to inform future IPF care, providing a benchmark to improve both clinical knowledge and patient outcomes.
Ada Enesco, EMJ
Reference
Fahim A et al.; British Thoracic Society. Idiopathic pulmonary fibrosis in the UK: findings from the British Thoracic Society UK Idiopathic Pulmonary Fibrosis Registry. BMJ Open Respir Res. 2025;12(1):e002773.
