Health related quality of life in patients with idiopathic pulmonary fibrosis in clinical practice: insights-IPF registry

Abstract

BackgroundThe INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline.MethodsSix hundred twenty-three IPF patients with available QoL data (St George’s Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed. Mean age was 69.6 ± 8.7 years, 77% were males, mean disease duration 2.0 ± 3.3 years, FVC pred was 67.5 ± 17.8%, DLCO pred 35.6 ± 17%.ResultsMean points were SGRQ total 48.3, UCSD SoB 47.8, EQ-5D VAS 66.8, and WHO-5 13.9. These instruments had a high or very high correlation (exception WHO-5 to EQ-5D VAS with moderate correlation). On bivariate analysis, QoL by SGRQ total was statistically significantly associated with clinical symptoms (NYHA; p < 0.001), number of comorbidities (p < 0.05), hospitalisation rate (p < 0.01) and disease severity (as measured by GAP score, CPI, FVC and 6-min walk test; p < 0.05 each). Multivariate analyses showed a significant association between QoL (by SGRQ total) and IPF duration, FVC, age, NYHA class and indication for long-term oxygen treatment.ConclusionsOverall, IPF patients under real-life conditions have lower QoL compared to those in clinical studies. There is a meaningful relationship between QoL and various patient characteristics.Trial registrationThe INSIGHTS-IPF registry is registered at Clinicaltrials.gov (NCT01695408).Electronic supplementary materialThe online version of this article (doi:10.1186/s12931-017-0621-y) contains supplementary material, which is available to authorized users.

Real-World Experience with Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

“…The average life expectancy following diagnosis is approximately 3 years, as this is the worst of all idiopathic interstitial pneumonias. The disease significantly affects health-related quality of life [1] and is also associated with a large number of comorbidities, which further add to the burden of disease associated with the condition [2, 3]. The requirement of those comorbidities for concomitant treatment may also affect the course of the illness [4-6].…”

Statin Therapy and Outcomes in Trials of Nintedanib in Idiopathic Pulmonary Fibrosis

“…Idiopathic pulmonary fibrosis (IPF) is a specific form of interstitial lung disease (ILD) characterised by progressive decline in lung function, worsening dyspnoea, and impaired health-related quality of life [1, 2]. The pathogenesis of IPF is believed to involve epithelial injury and impaired wound healing.…”

Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions

“…Although the same methodological problems are present with real-life data, national registries are already implementing the measurement of QoL in patients with IPF [45,86,[93][94][95], and this approach will allow the collection of prospective data on the clinical history of the disease and will offer the possibility to explore the long-term effect of several interventions on QoL. One example is the recent report on QoL in the German INSIGHTS-IPF registry [86]. Statistically significant associations of QoL with clinical symptoms, number of comorbidities, hospitalisation rate and disease severity (including functional data) have been reported [86].…”

“…One example is the recent report on QoL in the German INSIGHTS-IPF registry [86]. Statistically significant associations of QoL with clinical symptoms, number of comorbidities, hospitalisation rate and disease severity (including functional data) have been reported [86]. The life of a patient with IPF is influenced by respiratory (dyspnoea, cough, limited exercise capacity) and systemic (weight loss, anxiety/depression) symptoms/conditions [80] that must be properly recorded and evaluated over time to assess the effect of different interventions on their severity and impact on QoL.…”

“…Many comorbidities have been stated to be more common in IPF than in the general population, including cardiovascular and thromboembolic diseases, depression, reflux disease, emphysema, lung cancer, sleep disorders and diabetes [85]. Furthermore, a significant association between the frequency of comorbidities and impaired health-related QoL has been described recently [86]. One may thus hypothesise that active screening for and treatment of comorbid conditions may ameliorate the burden of disease, increase QoL and perhaps improve the prognosis in IPF.…”