Real world idiopathic pulmonary fibrosis in the EMPIRE registry
Bildiriler > Özet Bildiri · 2018
Yazarlar: Martina Vasakova, Martina Sterclova, Nesrin MOĞULKOÇ BISHOP, Jan Kus, Veronika Mueller, Marta Hajkova, Dragana Jovanovic, Jasna Tekavec-Trkanjec, Mordechai Kremer, Michal Svoboda, Marketa Janotova
Idiopathic interstitial pneumonias, Fransa, Uluslararası, Sözlü Sunum, Özet Bildiri, 2018-2018, Wos, cilt 52, ss PA2203-PA2203
DOI: 10.1183/13993003.congress-2018.pa2203
Özet
Aims: Idiopathic pulmonary fibrosis (IPF) clinical behaviour is mostly known only from clinical trials and from single center or country experiences. We aim to show real world of IPF in Central and Eastern Europe on the data from European Multipartner IPF registry (EMPIRE) which currently involves 8 European countries. Patients and methods: The analysis was performed on 2048 patients (626 F; 1422 M; 834 (40.7%) non-smokers; 817 from the Czech republic, 377 Turkey, 366 Poland, 182 Hungary, 159 Slovakia, 84 Serbia, 45 Croatia, 18 Israel). Baseline demographic data, lung function, and HRCT patterns were collected and their relations to survival were statistically evaluated. Results: Median age was 67.7 years (range 50.1–81.9), symptom duration 11.0 (range 1.0-50.0) months. Median forced vital capacity (FVC) was 76.2% (range 47.1-111.6) and transfer factor (TLCO) 47% (range 22.2-78.6). Typical HRCT pattern (definite UIP) was seen in 1550 patients (75.5%), and atypical (possible or inconsistent with UIP) in 491 (24.5%). 662 patients were on pirfenidone and 481 on nintedanib. Survival was significantly longer in females (p ˂0.001), in the patients with higher FVC and TLCO (p ˂0.001), non-smokers (p=0.021) and the patients with atypical HRCT presentation (p=0.011). Also pirfenidone (p=0.033) and nintedanib (p ˂0.001) treatment offered survival advantage compared to any other or no therapy. Conclusion: Better outcome was observed in females and in the patients with better initial FVC and TLCO, with atypical HRCT pattern and in those on anti-fibrotic treatment. The EMPIRE registry can show IPF in real world and prove effectivity of anti-fibrotic treatment across the diversity of IPF compared to randomized clinical trials.