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 ).
- Guenther, A.
- Krauss, E.
- Tello, S.
- Wagner, J.
- Paul, B.
- Kuhn, S.
- Maurer, O.
- Heinemann, S.
- Costabel, U.
- Barbero, M. A. N.
- Muller, V.
- Bonniaud, P.
- Vancheri, C.
- Wells, A.
- Vasakova, M.
- Pesci, A.
- Sofia, M.
- Klepetko, W.
- Seeger, W.
- Drakopanagiotakis, F.
- Crestani, B.
Keywords
- *European registry for idiopathic pulmonary fibrosis (eurIPFreg)
- *Idiopathic pulmonary fibrosis (IPF)
- *Interstitial lung diseases (ILD)
