The clinical course of idiopathic pulmonary fibrosis and its association to quality of life over time: longitudinal data from the INSIGHTS-IPF registry
Open Access
- 15 March 2019
- journal article
- research article
- Published by Springer Science and Business Media LLC in Respiratory Research
- Vol. 20 (1), 1-13
- https://doi.org/10.1186/s12931-019-1020-3
Abstract
Quality 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. Clinical 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. Data 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. QoL 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.Keywords
Funding Information
- Boehringer Ingelheim
This publication has 30 references indexed in Scilit:
- Efficacy and Safety of Nintedanib in Idiopathic Pulmonary FibrosisNew England Journal of Medicine, 2014
- A Phase 3 Trial of Pirfenidone in Patients with Idiopathic Pulmonary FibrosisNew England Journal of Medicine, 2014
- Cross-Atlantic modification and validation of the A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis (ATAQ-IPF-cA)BMJ Open Respiratory Research, 2014
- Investigating significant health trends in idiopathic pulmonary fibrosis (INSIGHTS-IPF): rationale, aims and design of a nationwide prospective registryBMJ Open Respiratory Research, 2014
- Interventions to improve symptoms and quality of life of patients with fibrotic interstitial lung disease: a systematic review of the literatureThorax, 2012
- Charakteristika und Versorgung der idiopathischen LungenfibroseDeutsche Medizinische Wochenschrift (1946), 2012
- The UCSD shortness of breath questionnaire has longitudinal construct validity in idiopathic pulmonary fibrosisRespiratory Medicine, 2012
- An Official ATS/ERS/JRS/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and ManagementAmerican Journal of Respiratory and Critical Care Medicine, 2011
- Development and validity testing of an IPF-specific version of the St George's Respiratory QuestionnaireThorax, 2010
- A Controlled Trial of Sildenafil in Advanced Idiopathic Pulmonary FibrosisNew England Journal of Medicine, 2010