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Multicenter Study
. 2018 Jul 28;19(1):141.
doi: 10.1186/s12931-018-0845-5.

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

Andreas Guenther  1   2   3   4   5 , Ekaterina Krauss  6   7 , Silke Tello  6   7 , Jasmin Wagner  6   7 , Bettina Paul  6   7 , Stefan Kuhn  6   7 , Olga Maurer  6   8 , Sabine Heinemann  6   7 , Ulrich Costabel  6   9 , María Asunción Nieto Barbero  6   10 , Veronika Müller  6   11 , Philippe Bonniaud  6   12 , Carlo Vancheri  6   13 , Athol Wells  6   14 , Martina Vasakova  6   15 , Alberto Pesci  6   16 , Matteo Sofia  6   17 , Walter Klepetko  6   18 , Werner Seeger  6   7   19 , Fotios Drakopanagiotakis  6   7 , Bruno Crestani  6   20
Affiliations
Multicenter Study

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

Andreas Guenther et al. Respir Res. .

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 (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 ).

Keywords: European registry for idiopathic pulmonary fibrosis (eurIPFreg); Idiopathic pulmonary fibrosis (IPF); Interstitial lung diseases (ILD).

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Conflict of interest statement

Ethics approval and consent to participate

Both, eurIPFreg and eurIPFbank have also been reviewed and approved from institutional review boards in Germany (e.g. Ethics Committee of Justus-Liebig-University of Giessen; 111/08), France, Italy, Austria, Spain, Czech Republic, Hungary and the UK. The research was conducted strictly according to the principles of the Declaration of Helsinki. Patients were included into the registry upon having signed the informed consent. The eurIPFreg and eurIPFbank are listed in ClinicalTrials.gov (NCT02951416).

Consent for publication

Not applicable

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Distribution and diversity of ILD diagnoses in the eurIPFreg cohort. Data are presented as patients numbers per diagnosis. IBD: inflammatory bowel diseases; DPLD: diffuse parenchymal lung diseases
Fig. 2
Fig. 2
Distribution of self-reported symptoms of IPF patients. Data are presented as percentage of all patients with reported symptom
Fig. 3
Fig. 3
Change in biopsy procedures in IPF over time. Data are given as percentage of the respective procedure undertaken in IPF subjects in the year of first diagnosis
Fig. 4
Fig. 4
Spectrum of co-morbidities in the IPF cohort. Data are given as percentage of all patients. Multiple co-morbidities could be reported
Fig. 5
Fig. 5
Change in IPF treatment over time. The graph shows various therapeutic regime (acetylcysteine, azathioprine, prednisolone, mycophenolic acid and anti-fibrotic drugs) in percentage of all treated patients
Fig. 6
Fig. 6
Overall survival of IPF patients upon first diagnosis depending on treatment. Given are Kaplan-Meier curves for cumulative survival, based on definite outcome data (survival status definitely known as per end of 2016) and on last visit data. A statistically significant difference in survival was encountered between patients receiving anti-fibrotic treatment and those not receiving antifibrotics, significance level p was 0.001. Within the group of patients receiving antifibrotic treatment, 83% of patients received pirfenidone and 17% received nintedanib
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References

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