Source: International Congress 2014 – ILDs 6
Disease area: Interstitial lung diseases

Abstract

Introduction: Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and generally fatal disease. Pirfenidone (PFD) is approved for the treatment of mild-to-moderate IPF in the European Union. An observational registry of IPF patients (pts) (the PROOF registry) was initiated to collect data from routine clinical visits and monitor IPF development. Interim data from the PROOF registry are presented.Aims and objectives: The PROOF registry aims to describe the disease course and outcomes in IPF pts in a real-world setting.Methods: The registry is on-going in 6 centres in Belgium and Luxemburg, and will be extended to the Netherlands. Data from IPF pts are collected according to the normal course of pts care and include demographic information, medical history, pulmonary function data, high-resolution computed tomography and previous/current treatments.Results: Since October 2013, 35 pts (aged 70.2 ± 8.4 years, 89% male) have been enrolled. IPF diagnosis was made during a multidisciplinary discussion and was definite in 91% of cases. IPF symptoms were breathlessness, cough and velcro rales in 77%, 63% and 49% of pts respectively. Surgical lung biopsy was performed in 23% of pts. Pulmonary function tests showed a mean FVC of 80.0 ± 23.6% and a mean FEV1/FVC of 88.4 ± 11.9. Time to IPF diagnosis ranged from 5 days to 8 years. 88% of pts had relevant previous/ongoing co-morbidities and 9% of pts had family history of IPF. 73% of pts received previous IPF treatment; 57% of pts received PFD.Conclusions: The PROOF registry will provide real-world information and insights on the clinical course of IPF.

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W. Wuyts, C. Dahlqvist, M. Schlesser, C. Compere, H. Slabbynck, B. Bondue, M. Berrens, C. Giot, P. DeVuyst (Leuven, Yvoir, Liège, Antwerpen, Brussels, Belgium; Luxembourg, Luxembourg; Nieuwegein, Netherlands; Muttenz, Switzerland). PROOF-registry: A prospective observational registry to describe the disease course and outcomes of idiopathic pulmonary fibrosis patients in a real-world clinical setting. Eur Respir J 2014; 44: Suppl. 58, 3785

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