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

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.Electronic supplementary materialThe online version of this article (10.1186/s12931-019-1020-3) contains supplementary material, which is available to authorized users.

Responsiveness and minimal clinically important difference of SGRQ-I and K-BILD in idiopathic pulmonary fibrosis

“…The association between low HRQL and increased mortality has been investigated in other studies using for instance SGRQ and SOBQ. In concordance with the IPF-specific SGRQ-I, both baseline SGRQ score and changes in SGRQ and SOBQ scores were found to be prognostic factors in patients with IPF [4,34]. The small number of mortalities and less advanced disease in our cohort might explain why only a trend was observed between K-BILD and mortality.…”

“…Along with a wide range of comorbidities, patients with IPF often experience impaired health-related quality of life (HRQL) [2,3]. As the disease progresses, the symptom burden increases resulting in decreasing HRQL, and in the terminal phase of the disease, HRQL plummets considerably [4]. Antifibrotic treatments successfully slow down lung function decline, but do not improve HRQL convincingly [5,6].…”

Efficacy and safety of nintedanib in patients with advanced idiopathic pulmonary fibrosis

“…Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease associated with decline in lung function, worsening dyspnoea and quality of life, and considerable mortality [1,2]. While IPF progresses in all patients, the pattern of disease progression is variable and remains a challenge to predict [3,4].…”

Impact of Depression on Patients With Idiopathic Pulmonary Fibrosis

“…Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive fibrotic lung disease exerting a dramatic impact on patients' quality of life (1). The Food and Drug Administration approval of two novel anti-fibrotic compounds has finally shifted the therapeutic dial of IPF (2)(3)(4)(5).…”

“…The Food and Drug Administration approval of two novel anti-fibrotic compounds has finally shifted the therapeutic dial of IPF (2)(3)(4)(5). While the emergence of drugs that slow down disease progression represents a major breakthrough in the management of IPF, pharmacological therapy does not address patient reported outcomes including cough, dyspnea and quality of life while at the same time comes with side-effects that have a net reduction in quality of life (1,6). Importantly, IPF is an age-related disease and thus presents with major comorbidities that significantly impact survival and quality of life (7)(8)(9).…”