The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry

Michael Kreuter,Silke Geier,Jürgen Behr,Sven Gläser,Jeff Swigris,Marion Frankenberger,Tobias Welte,Thomas Bahmer,Hubert Wirtz,Dirk Skowasch,Christian Grohé ,Antje Prasse,David Pittrow,H Wilkens ,Claus Neurohr,Matthias Held,Nicolas Kahn,J Kirschner ,Martin Claussen,Tim Oqueka,Joachim Meyer ,Martin Schwaiblmair,Lars Hagmeyer,Stefan Andreas,Dirk Koschel,Jens Klotsche

doi:10.1186/s12931-019-1020-3

Abstract

BackgroundQuality of life (QoL) is profoundly impaired in patients with idiopathic pulmonary fibrosis (IPF). However, data is limited regarding the course of QoL. We therefore analysed longitudinal data from the German INSIGHTS-IPF registry.MethodsClinical status and QoL were assessed at enrollment and subsequently at 6- to 12-months intervals. A range of different QoL questionnaires including the St. George’s Respiratory Questionnaire (SGRQ) were used.ResultsData from 424 patients were included; 76.9% male; mean age 68.7 ± 9.1 years, mean FVC% predicted 75.9 ± 19.4, mean DLCO% predicted 36.1 ± 15.9. QoL worsened significantly during follow-up with higher total SGRQ scores (increased by 1.47 per year; 95% CI: 1.17 to 1.76; p < 0.001) and higher UCSD-SOBQ scores and lower EQ-5D VAS and WHO-5 scores. An absolute decline in FVC% predicted of > 10% was associated with a significant deterioration in SGRQ (increasing by 9.08 units; 95% CI: 2.48 to 15.67; p = 0.007), while patients with stable or improved FVC had no significantly change in SGRQ. Patients with a > 10% decrease of DLCO % predicted also had a significant increase in SGRQ (+ 7.79 units; 95% CI: 0.85 to 14.73; p = 0.028), while SQRQ was almost stable in patients with stable or improved DLCO. Patients who died had a significant greater increase in SGRQ total scores (mean 11.8 ± 18.6) at their last follow-up visit prior to death compared to survivors (mean 4.2 ± 18.9; HR = 1.03; 95% CI: 1.01 to 1.04; p < 0.001). All QoL scores across the follow-up period were significantly worse in hospitalised patients compared to non-hospitalised patients, with the worst scores reported in those hospitalised for acute exacerbations.ConclusionsQoL assessments in the INSIGHTS-IPF registry demonstrate a close relationship between QoL and clinically meaningful changes in lung function, comorbidities, disease duration and clinical course of IPF, including hospitalisation and mortality.

Highlights

Idiopathic pulmonary fibrosis (IPF) is a severe disease characterised by debilitating symptoms such as cough, fatigue, increasing and immobilising dyspnoea and is associated with a limited survival [1, 2]
Most patients were in New York Heart Association functional class (NYHA) functional class II/III at enrolment, with a mean Six-minute walk distance (6MWD) of 288 ± 200 m
Mean values for respiratory parameters were; forced vital capacity (FVC) % predicted (75.9% ± 19.4), forced expiratory volume in 1 s (FEV1)% (68.3% ± 17.4), and DLCO % predicted (36.1% ± 15.9); 53.0% were in GAP index stage 2 and 28.2% in stage 3

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a severe disease characterised by debilitating symptoms such as cough, fatigue, increasing and immobilising dyspnoea and is associated with a limited survival [1, 2]. Recent reports have shown that in IPF patients health-related quality of life (QoL) is associated with clinical symptoms, duration of disease, physical activity, comorbidities, and disease severity [6,7,8,9,10] and may be prognostic [11]. We report longitudinal data (with up to 36 months follow-up) for a range of QoL measures and examine changes in QoL outcomes and their relationship to clinical parameters. We analysed longitudinal data from the German INSIGHTS-IPF registry

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Conclusion