Demographics and Baseline Characteristics of Patients with Idiopathic Pulmonary Fibrosis (IPF) in a Real-World Setting: Results of 847 Patients Enrolled in the Radico-ILD Cohort in France

International audienceRationale: Idiopathic pulmonary fibrosis (IPF) is a rare condition and few epidemiological dataare available in France. This specific research project aims to describe characteristics of treatedIPF patients and the impact of antifibrotic treatments in terms of morbidity and mortality in theFrench real-life setting. Methods: The French RaDiCo (Rare Disease Cohort)-ILD (idiopathicInterstitial Lung Diseases) registry is an ongoing observational study initiated in June 2017, witha sub-analysis of IPF patients. This longitudinal long-term cohort includes pediatric and adultpatients with ILD and is supported by the national network of reference and competence centersfor rare pulmonary diseases. IPF was diagnosed using international ATS/ERS 2011 criteria witha diagnosis of IPF or working diagnosis of IPF by multidisciplinary discussion. Here, we presentthe baseline data of IPF patients. Results: Between June 15th 2017 and September 4th 2019,1246 ILD patients were enrolled in the RaDiCo-ILD registry from 18 centers, including 847 withIPF (68%). IPF patients were mostly male (82.7%), with a mean age of 72.5 ± 9 years at inclusionand a mean BMI of 26.8 ± 4.3; 44.6 % of IPF patients included were incident cases, with amedian length between diagnosis and inclusion of 8.9 months (Q1=0.9 and Q3=26.4); 25.3% hada biopsy. The mean FVC at IPF diagnosis was 73.4 ± 25.0 % of predicted value (n=561), and themean DLCO at IPF diagnosis was 39.6 ± 18.2 % predicted value (n=498). Among patients withavailable information on anti-fibrotic treatment, 347 had been treated (at least one dose) withnintedanib, and 312 with pirfenidone; among patients treated with antifibrotics, 113 were treatedwith both treatments sequentially. Conclusions: The RaDiCo-ILD registry provides accurate real-world data on the demographics of patients with IPF in France. It will generate a long-term follow-up and will be an invaluable tool to describe the natural history and progression of patients withIPF in real-life conditions