Background
For patients with interstitial lung diseases (ILD) with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of disease characteristics at diagnosis, patient journey, and treatment is limited. This study aimed to describe demographics and clinical experiences of patients presenting with PPF in a European real-world setting.
Methods
Data were analysed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey of pulmonologists and rheumatologists in five European countries (France, Germany, Italy, Spain, United Kingdom) and internal medicine specialists (France) from April to October 2022. Physicians provided data for up to 12 consecutive patients with physician-confirmed ILD with a progressive phenotype other than idiopathic pulmonary fibrosis. Analyses were descriptive.
Results
Overall, 265 physicians reported on 1,335 patients. Mean (standard deviation [SD]) age at survey date was 60.4 (11.6) years, 91.2% were white, 58.1% female, 44.0% non-smokers. The mean age (SD) at first healthcare professional (HCP) visit was 57.6 (12.1) years, with 63.3% patients first consulting a primary care physician. There was a mean delay of 7.8 (22.7) months between first ILD symptom and HCP visit, and another 7.7 (12.8) months to ILD diagnosis. There were 47.7% patients with physician-reported moderate ILD at survey date, 42.3% with mild and 10.0% with severe ILD. Disease progression was reported in the 12 months prior to the survey for 19.5% of patients; of these, progression was based on worsening symptom severity in 27.3% and on lung function decline in 25.8%. For patients experiencing symptoms prior to ILD diagnosis (72.8%), the most common symptoms were dyspnoea on exertion (80.5%) and cough (57.8%). Overall, 17.4% of patients were misdiagnosed prior to ILD diagnosis, with chronic obstructive pulmonary disease suspected in 39.2% of them. The most frequent comorbidities were anxiety (16.9%) and gastroesophageal reflux (15.5%). Although 77.8% of patients were receiving treatment for ILD at survey date, 15.6% of patients had never been prescribed treatment for ILD.
Conclusions
This real-world study expands our understanding of patients, diagnostic delays and treatment gaps experienced by patients diagnosed with PPF in Europe. Given the progressive nature of PPF, diagnostic delay may lead to poor outcomes, including shorter survival.
Trial registration:
n/a