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

Andreas Güenther ,María Asunción Nieto Barbero,Veronika Müller,Olga Maurer,Martina Vašáková ,S Heinemann ,Werner Seeger,Alberto Pesci,Athol U Wells ,Silke Tello,Carlo Vancheri,Ekaterina Krauss,Walter Klepetko,Bettina Paul,Fotios Drakopanagiotakis,Jasmin Wagner,Ulrich Costabel,Sofia Miceli ,Stefan Kühn ,Philippe Bonniaud,Bruno Crestani

doi:10.1186/s12931-018-0845-5

Abstract

BackgroundSince 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research.MethodsThe 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.ResultsThis 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).ConclusionsOur data provide important insights into baseline characteristics, diagnostic and management changes as well as outcome data in European IPF patients over time.Trial registrationThe eurIPFreg and eurIPFbank are listed in ClinicalTrials.gov(NCT02951416).

Highlights

Since 2009, Idiopathic pulmonary fibrosis (IPF) patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research
One thousand eighty-six of these patients had suspected or proven Interstitial lung diseases (ILD), among them 525 patients with IPF. The data of another 50 IPF patients, who had been recruited into the registry, could not be taken into consideration, as they were still under final assessment
Demographics Patients with interstitial lung diseases other than IPF In the period between 2009 and 2016, a total of 1086 patients with ILDs were recruited into the eurIPFreg

Summary

Introduction

Since 2009, IPF patients across Europe are recruited into the eurIPFreg, providing epidemiological data and biomaterials for translational research. As the natural course of IPF is quite heterogeneous, and as the response to the novel anti-fibrotic drugs has been reported to show great variability, it is essential to identify reliable predictive factors indicating the risk of deterioration and the response to medical treatment, as well as side effects in a broad non-selected patient cohort [12, 13]. Data collected in the frame of the numerous controlled clinical trials undertaken so far in IPF, here especially in the placebo arms, have provided important insights into the clinical course of IPF It should, be kept in mind that patients recruited into these studies represent a rigorously selected population and do not necessarily reflect the characteristics of IPF subjects seen in clinical routine

Methods

Results

Discussion

Conclusion