E091 Progressive pulmonary fibrosis in systemic autoimmune rheumatic diseases and other types of interstitial lung disease: real-world patient care in the UK

Background/Aims Progressive pulmonary fibrosis (PPF) is a chronic form of interstitial lung disease (ILD), characterised by inflammation and fibrosis of the lungs. Patients with PPF experience worsening of respiratory symptoms over time and a decline in lung function that can ultimately result in death. The condition may arise as a complication of various underlying causes, including common systemic autoimmune rheumatic diseases (SARDs) such as rheumatoid arthritis, systemic sclerosis, polymyositis/dermatomyositis and Sjögren’s syndrome. UK and international guidelines recommend the use of antifibrotic medications for patients with PPF regardless of the underlying cause. This study describes demographics, disease management, and health-related quality of life for patients with PPF in the UK. Methods Data were drawn from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey of physicians and their patients in the UK from February to October 2024. Physicians provided patient demographics and treatment history data, and patients voluntarily completed the EQ-5D and King’s Brief-ILD (KBILD) questionnaires. Analyses were descriptive. Results Overall, 50 physicians reported data on 208 patients with PPF, 117 of whom had SARD-PPF and 91 had PPF associated with other underlying conditions (non-SARD-PPF). For patients with SARD-PPF, mean (SD) age was 61 (12) years, 68% were female, and the mean pulmonary function, as measured by forced vital capacity % predicted, was 58.1 (17.2). Medical treatment was prescribed for 84% of patients with SARD-PPF. The most common therapies were corticosteroids (56%) and immunosuppressants (56%), in particular mycophenolate (25%) and methotrexate (15%). Biologics were prescribed for 34% of patients, with rituximab being the most common (29%). A small proportion (6%) were treated with disease-modifying anti-rheumatic drugs. Less than half (42%) of SARD-PPF patients were treated with antifibrotics: 38% with nintedanib and 3% with pirfenidone. Antifibrotics were combined with corticosteroids and/or immunosuppressants in some patients. Side effects of antifibrotic treatment, including diarrhoea and nausea, were common (22% of patients treated [11/49]). Physicians reported that 76% of patients with SARD-PPF and 77% of those with non-SARD-PPF had moderate or severe disease progression in the 12 months preceding the survey. However, they expected that 62% of patients with SARD-PPF and 50% of those with non-SARD-PPF would have moderate or severe disease progression within the next 12 months. Patients with SARD-PPF and non-SARD-PPF reported a mean EQ-5D score of 0.44 (0.33) and 0.73 (0.19), respectively. Patients with SARD-PPF and non-SARD-PPF reported a mean KBILD score of 46.2 (11.0) and 52.9 (8.9), respectively. Conclusion Despite guideline recommendations, many patients with SARD-PPF in the UK are not prescribed antifibrotic medications - treatments proven to slow the decline of lung function. Patients continue to experience disease progression, worsening of symptoms and a reduced quality of life compared to people of similar age in the general population. Disclosure B. Torlinska: Other; Employed by Boehringer Ingelheim. R. Hamad: Other; Employed by Boehringer Ingelheim. M. Frysz: Other; Employed by Boehringer Ingelheim. L. Lee: Other; Employed by Adelphi Real World. M. Small: Other; Employed by Adelphi Real World. R. Moore: Other; Employed by Boehringer Ingelheim Ltd.