TY  - JOUR
A1  - Günther, Andreas
A1  - Krauss, Ekaterina
A1  - Tello, Silke
A1  - Wagner, Jasmin
A1  - Paul, Bettina
A1  - Kuhn, Stefan
A1  - Maurer, Olga
A1  - Heinemann, Sabine
A1  - Costabel, Ulrich
A1  - Nieto Barbero, María Asunción
A1  - Müller, Veronika
A1  - Bonniaud, Philippe
A1  - Vancheri, Carlo
A1  - Wells, Athol
A1  - Vasakova, Martina
A1  - Pesci, Alberto
A1  - Sofia, Matteo
A1  - Klepetko, Walter
A1  - Seeger, Werner
A1  - Drakopanagiotakis, Fotios
A1  - Crestani, Bruno
T1  - The European IPF registry (eurIPFreg) : baseline characteristics and survival of patients with idiopathic pulmonary fibrosis
T2  - Respiratory research
N2  - 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).
KW  - Idiopathic pulmonary fibrosis (IPF)
KW  - European registry for idiopathic pulmonary fibrosis (eurIPFreg)
KW  - Interstitial lung diseases (ILD)
Y1  - 2018
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/48458
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-484586
SN  - 1465-993X
SN  - 1465-9921
N1  - 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.
VL  - 19
IS  - 1, Art. 141
SP  - 1
EP  - 10
PB  - BioMed Central
CY  - London
ER  -