Journalartikel

Jahr der Veröffentlichung: 2018

Zeitschrift: Respiratory Research

Bandnummer: 19

ISSN: 1465-993X

eISSN: 1465-9921

Open Access Status: Gold

DOI Link: https://doi.org/10.1186/s12931-018-0845-5

Verlag: BioMed Central

Abstract: 

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 (532%). 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.

Harvard-Zitierstil: Guenther, A., Krauss, E., Tello, S., Wagner, J., Paul, B., Kuhn, S., et al. (2018) The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis, Respiratory Research, 19, Article 141. https://doi.org/10.1186/s12931-018-0845-5

APA-Zitierstil: Guenther, A., Krauss, E., Tello, S., Wagner, J., Paul, B., Kuhn, S., Maurer, O., Heinemann, S., Costabel, U., Nieto Barbero, M., Mueller, V., Bonniaud, P., Vancheri, C., Wells, A., Vasakova, M., Pesci, A., Sofia, M., Klepetko, W., Seeger, W., ...Crestani, B. (2018). The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respiratory Research. 19, Article 141. https://doi.org/10.1186/s12931-018-0845-5