The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis

Andreas Guenther (1, 2, 3, 4, 5, 6) , Ekaterina Krauss (1, 2) , Silke Tello (1, 2) , Jasmin Wagner (1, 2) , Bettina Paul (1, 2) , Stefan Kuhn (1, 2) , Olga Maurer (1, 5) , Sabine Heinemann (1, 2) , Ulrich Costabel (1, 7) , María Asunción Nieto Barbero (1, 8) , Veronika Müller (1, 9) , Philippe Bonniaud (1, 10) , Carlo Vancheri (11, 1) , Athol U. Wells (1, 12) , Martina Vasakova (1, 13) , Alberto Pesci (1, 14) , Matteo Sofia (1, 15) , Walter Klepetko (1, 16) , Werner Seeger (1, 2, 3, 4) , Fotios Drakopanagiotakis (1, 2) , Bruno Crestani (1, 17)

1 eurIPFreg/bank - European IPF Registry and Biobank
2 UGMLC - University of Giessen and Marburg Lung Center
3 JLU - Justus-Liebig-Universität Gießen = Justus Liebig University
4 Johann Wolfgang Goethe University
5 Agaplesion Pneumologische KliniK Waldhof-Elgershausen
6 German Center for Lung Research
7 AöR - University Hospital Essen
8 Hospital Clinico San Carlos (Madrid)
9 Semmelweis University [Budapest]
10 LNC - Lipides - Nutrition - Cancer [Dijon - U1231]
11 Unict - Università degli studi di Catania = University of Catania
12 Department of Respiratory Medicine
13 1st Faculty of Medicine and Thomayer University Hospital
14 San Gerardo Hospital
15 University of Naples Federico II = Università degli studi di Napoli Federico II
16 University Hospital Vienna
17 Centre de compétences maladies pulmonaires rares [CHU Bichat]

BACKGROUND:Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research. METHODS:The registry data are based on patient and physician baseline and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term objectives of the registry are to provide clues for a better understanding of IPF phenotype sub-clusters, triggering factors and aggravating conditions, regional and environmental characteristics, and of disease behavior and management. RESULTS:This paper describes baseline data of 525 IPF subjects recruited from 11/2009 until 10/2016. IPF patients had a mean age of 68.1 years, and seeked medical advice due to insidious dyspnea (90.1%), fatigue (69.2%), and dry coughing (53.2%). A surgical lung biopsy was performed in 32% in 2009, but in only 8% of the cases in 2016, possibly due to increased numbers of cryobiopsy. At the time of inclusion in the eurIPFreg, FVC was 68.4% ± 22.6% of predicted value, DLco ranged at 42.1% ± 17.8% of predicted value (mean value ± SD). Signs of pulmonary hypertension were found in 16.8%. Steroids, immunosuppressants and N-Acetylcysteine declined since 2009, and were replaced by antifibrotics, under which patients showed improved survival (p = 0.001). CONCLUSIONS:Our data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time. TRIAL REGISTRATION:The eurIPFreg and eurIPFbank are listed in ClinicalTrials.gov( NCT02951416 ).

Mots clés: Idiopathic pulmonary fibrosis (IPF); Interstitial lung diseases (ILD); European registry for idiopathic pulmonary fibrosis (eurIPFreg)

Domaines: Pneumologie et système respiratoire

Andreas Guenther, Ekaterina Krauss, Silke Tello, Jasmin Wagner, Bettina Paul, et al.. The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respiratory Research, 2018, 19 (1), pp.141. ⟨10.1186/s12931-018-0845-5⟩. ⟨hal-02006205⟩