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

  • 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).

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Author:Andreas Günther, Ekaterina Krauss, Silke Tello, Jasmin Wagner, Bettina Paul, Stefan Kuhn, Olga Maurer, Sabine Heinemann, Ulrich Costabel, María Asunción Nieto Barbero, Veronika Müller, Philippe Bonniaud, Carlo Vancheri, Athol Wells, Martina Vasakova, Alberto Pesci, Matteo Sofia, Walter Klepetko, Werner SeegerORCiDGND, Fotios Drakopanagiotakis, Bruno Crestani
URN:urn:nbn:de:hebis:30:3-484586
DOI:https://doi.org/10.1186/s12931-018-0845-5
ISSN:1465-993X
ISSN:1465-9921
Pubmed Id:https://pubmed.ncbi.nlm.nih.gov/30055613
Parent Title (English):Respiratory research
Publisher:BioMed Central
Place of publication:London
Document Type:Article
Language:English
Year of Completion:2018
Date of first Publication:2018/07/28
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2018/12/06
Tag:European registry for idiopathic pulmonary fibrosis (eurIPFreg); Idiopathic pulmonary fibrosis (IPF); Interstitial lung diseases (ILD)
Volume:19
Issue:1, Art. 141
Page Number:10
First Page:1
Last Page:10
Note:
Open Access: This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
HeBIS-PPN:440895499
Institutes:Medizin / Medizin
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Sammlungen:Universitätspublikationen
Licence (German):License LogoCreative Commons - Namensnennung 4.0