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

Guenther, Andreas ; Krauss, Ekaterina ; Tello, Silke ; Wagner, Jasmin ; Paul, Bettina ; Kuhn, Stefan ; Maurer, Olga ; Heinemann, Sabine ; Costabel, Ulrich ; Barbero, María Asunción Nieto ; Müller, Veronika ; Bonniaud, Philippe ; Vancheri, Carlo ; Wells, Athol ; Vasakova, Martina ; Pesci, Alberto ; Sofia, Matteo ; Klepetko, Walter ; Seeger, Werner ; Drakopanagiotakis, Fotios ; Crestani, Bruno

Originalveröffentlichung: (2018) Respiratory Research 19, 141 doi: 10.1186/s12931-018-0845-5
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URN: urn:nbn:de:hebis:26-opus-153414

Freie Schlagwörter (Englisch): idiopathic pulmonary fibrosis (IPF) , European registry for idiopathic pulmonary fibrosis (eurIPFreg) , interstitial lung diseases (ILD)
Sammlung: Open Access - Publikationsfonds
Universität Justus-Liebig-Universität Gießen
Institut: Medizinische Klinik II
Fachgebiet: Medizin
DDC-Sachgruppe: Medizin
Dokumentart: Aufsatz
Sprache: Englisch
Erstellungsjahr: 2018
Publikationsdatum: 03.08.2020
Kurzfassung auf Englisch: 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
Lizenz: Lizenz-Logo  Creative Commons - Namensnennung 4.0