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 ).

1st Faculty of Medicine and Thomayer Hospital Prague Czech Republic

AGAPLESION Lung Clinic Waldhof Elgershausen Greifenstein Germany

Clinical Research Unit Pulmonary Fibrosis University of Giessen and Marburg Lung Center Klinikstrasse 36 35392 Giessen Germany

Competence Center for Rare Pulmonary Diseases Hopital Bichat Paris France

Department of Clinical and Molecular Biomedicine Università degli Studi di Catania Catania Italy

Department of Pulmonology Semmelweis University Budapest Hungary

Department of Thoracic Surgery Vienna University Hospital Vienna Austria

European IPF Registry and Biobank Giessen Germany

Excellence Cluster Cardiopulmonary System Justus Liebig University Giessen and Johann Wolfgang Goethe University Frankfurt Frankfurt Germany

Hospital Clinico San Carlos Madrid Spain

Interstitial Lung Disease Unit Royal Brompton Hospital London UK

Ospedale San Gerardo Monza Italy

Reference Center for Rare Pulmonary Diseases Centre Hospitalier Universitaire Dijon Bourgogne INSERMU1231 Université Bourgogne Franche Comté Dijon France

Ruhrlandklinik University Hospital Essen Germany

Università degli Studi di Napoli Federico 2 Naples Italy

Universities of Giessen and Marburg Lung Center Giessen Germany

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ClinicalTrials.gov
NCT02951416