Health-related Quality of Life in Patients with Idiopathic Pulmonary Fibrosis -Cross-sectional and Longitudinal Study-

Tomioka, Haruaki; Imanaka, Kuniyasu; Hashimoto, Kimio; Iwasaki, Hiromichi

doi:10.2169/internalmedicine.46.6218

Cited by 59 publications

(68 citation statements)

References 25 publications

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“…20 Males tend to be more severely impacted by dyspnea than females in regard to quality of life. 21 Although pulmonary function and oxygenation were each correlated with the SGRQ score in 41 patients with IPF, dyspnea was the most prominent variable (based on regression analysis) contributing to impaired quality of life.…”

Section: Discussionmentioning

confidence: 99%

Physical Activity and Quality of Life Improvements of Patients With Idiopathic Pulmonary Fibrosis Completing a Pulmonary Rehabilitation Program

et al. 2014

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INTRODUCTION: Pulmonary rehabilitation is effective for patients with COPD, but its benefit is less clearly established in idiopathic pulmonary fibrosis (IPF), especially in regard to levels of physical activity and health-related quality of life. The objectives were to determine whether pulmonary rehabilitation increased physical activity as assessed by the International Physical Activity Questionnaire (IPAQ), and improved quality of life and symptoms as assessed by the St George respiratory questionnaire for IPF (SGRQ-I) and the Borg dyspnea index (BDI). METHODS: Subjects who met current criteria for IPF were randomized to a 3-month pulmonary rehabilitation program (n ‫؍‬ 11) or to a control group (n ‫؍‬ 10). The rehabilitation group participated in twiceweekly, 90-min exercise sessions (24 total sessions). The control group maintained its preceding, normal physical activity. All subjects underwent 6-min walk tests to assess the postexertion BDI. The SGRQ-I and a 5-point self-assessment of health were completed at baseline, after 3 months of intervention or observation, and after 3-month follow-up. All subjects completed the IPAQ weekly. RESULTS: Subjects in the rehabilitation group maintained significantly higher levels of physical activity throughout the 3-month rehabilitation program (rehabilitation: 51,364 ؎ 57,713 [mean ؎ SD] metabolic equivalent of task-minutes; control: 20,832 ؎ 37,155, P ‫؍‬ .027 by 2-tailed Mann-Whitney test). SGRQ-I symptom domain scores improved considerably by ؊9 ؎ 22 in the rehabilitation group, whereas in the control group they worsened (16 ؎ 12 rehabilitation compared with control, P ‫؍‬ .013 by 2-tailed Mann-Whitney test). During the 3-month follow-up, self-reported physical activity levels in the rehabilitation group were 14,428 ؎ 8,884 metabolic equivalent of task-minutes and in the control group 16,923 ؎ 32,620 (P ‫؍‬ .17 by 2-tailed Mann-Whitney test), demonstrating substantial reversal of activity in the rehabilitation group. BDI scores after 6-min walk tests did not change significantly. CONCLUSIONS: A 3-month rehabilitation program significantly improved symptoms (SGRQ-I) and physical activity levels (IPAQ) in subjects with IPF while they participated actively in the program. (ClinicalTrials.gov registration NCT01118221.) Key words: idiopathic pulmonary fibrosis; pulmonary rehabilitation; exercise; quality of life.

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Section: Discussionmentioning

confidence: 99%

Physical Activity and Quality of Life Improvements of Patients With Idiopathic Pulmonary Fibrosis Completing a Pulmonary Rehabilitation Program

et al. 2014

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“…Nine studies 61,[109][110][111][112][113][114][115][116] assessed HRQoL using the SF-36, seven used the SGRQ, 55,73,[117][118][119][120][121][122] five studies used both the SF-36 and SGRQ [123][124][125][126][127] and two studies used the SF-36, the SGRQ and the EQ-5D 68,72 (Table 65). Two studies used the EQ-5D.…”

Section: Systematic Review Of Health-related Quality Of Life Studiesmentioning

confidence: 99%

“…Two other multicentre studies were US-based only. 68,72 The remaining studies were single-centre studies from countries such as Japan (six studies 73,[112][113][114][115]122 ), Brazil (three studies 109,111,124 ), Poland (three studies 61,112,127 ), Turkey (one study 110 ), Canada (one study 123 ), the Netherlands (one study China (one study 118 ), Greece (two studies 116,120 ) and the USA (one study 55 ). Generalisability of these populations to those seen in current UK practice may, therefore, be limited.…”

Section: Systematic Review Of Health-related Quality Of Life Studiesmentioning

confidence: 99%

“…Six studies 55,68,72,73,125,126 were based on five RCTs of interventions where HRQoL was an outcome measure, one was a CCT, 61 and four studies were cohort 'before-and-after' studies that tested HRQoL at baseline and then again after an intervention; 110,113,114,116 one of these also had a control group of patients with COPD. 114 Five studies 111,119,[122][123][124] were single-cohort studies with no intervention, three 112,115,118 were single-cohort studies that collected HRQoL data at two different time points (one also had an additional control group 112 ), two studies 109,120 were cohort studies with matched controls, one was a cohort study with subgroup data 127 and one 117 was a combination of a focus group and survey. Seven of the 16 studies using the SF-36 did not report the overall physical or mental health summary scores, [109][110][111]113,114,124,125 one study reported only the overall summary scores (at baseline), 68 one study reported only an overall score encompassing both physical health and mental health component scores, 116 and one study did not present any useful data, reporting data as deviation values from national reference values.…”

Section: Systematic Review Of Health-related Quality Of Life Studiesmentioning

confidence: 99%

“…Seven of the 16 studies using the SF-36 did not report the overall physical or mental health summary scores, [109][110][111]113,114,124,125 one study reported only the overall summary scores (at baseline), 68 one study reported only an overall score encompassing both physical health and mental health component scores, 116 and one study did not present any useful data, reporting data as deviation values from national reference values. 115 The limitations of the methodological designs of these studies and potential for bias should be considered in the interpretation of these data.…”

Section: Systematic Review Of Health-related Quality Of Life Studiesmentioning

confidence: 99%

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The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

Loveman

Copley

Colquitt

et al. 2015

Health Technology Assessment

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BackgroundIdiopathic pulmonary fibrosis (IPF) is a life-limiting lung disease that generally affects people over 60 years old. The main symptoms are shortness of breath and cough, and as the disease progresses there is a considerable impact on day-to-day life. Few treatments are currently available.ObjectivesTo conduct a systematic review of clinical effectiveness and an analysis of cost-effectiveness of treatments for IPF based on an economic model informed by systematic reviews of cost-effectiveness and quality of life.Data sourcesEleven electronic bibliographic databases, including MEDLINE, EMBASE, Web of Science, and The Cochrane Library and the Centre for Reviews and Dissemination databases, were searched from database inception to July 2013. Reference lists of relevant publications were also checked and experts consulted.MethodsTwo reviewers independently screened references for the systematic reviews, extracted and checked data from the included studies and appraised their risk of bias. An advisory group was consulted about the choice of interventions until consensus was reached about eligibility. A narrative review with meta-analysis was undertaken, and a network meta-analysis (NMA) was performed. A decision-analytic Markov model was developed to estimate cost-effectiveness of pharmacological treatments for IPF. Parameter values were obtained from NMA and systematic reviews. Univariate and probabilistic sensitivity analyses were undertaken. The model perspective is NHS and Personal Social Services, and discount rate is 3.5% for costs and health benefits.ResultsFourteen studies were included in the review of clinical effectiveness, of which one evaluated azathioprine, threeN-acetylcysteine (NAC) (alone or in combination), four pirfenidone, one BIBF 1120, one sildenafil, one thalidomide, two pulmonary rehabilitation, and one a disease management programme. Study quality was generally good, with a low risk of bias. The current evidence suggests that some treatments appear to be clinically effective. The model base-case results show increased survival for five pharmacological treatments, compared with best supportive care, at increased cost. General recommendations cannot be made of their cost-effectiveness owing to limitations in the evidence base.LimitationsFew direct comparisons of treatments were identified. An indirect comparison through a NMA was performed; however, caution is recommended in the interpretation of these results. In relation to the economic model, there is an assumption that pharmacological treatments have a constant effect on the relative rate of per cent predicted forced vital capacity decline.ConclusionsFew interventions have any statistically significant effect on IPF and a lack of studies on palliative care approaches was identified. Research is required into the effects of symptom control interventions, in particular pulmonary rehabilitation and thalidomide. Other research priorities include a well-conducted randomised controlled trial on inhaled NAC therapy and an updated evidence synthesis once the results of ongoing studies are reported.Study registrationThis study is registered as PROSPERO CRD42012002116.FundingThe National Institute for Health Research Health Technology Assessment programme.

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Expectations, symptoms, and quality of life before and after 1 year of Pirfenidone treatment in patients with idiopathic pulmonary fibrosis: A single‐arm, open‐label nonrandomized study

Jastrzębski,

Kostorz‐Nosal,

Galle

et al. 2023

Health Science Reports

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Background and AimsAntifibrotic therapies reduce lung function decline in patients with idiopathic pulmonary fibrosis (IPF). This single‐arm, open‐label, nonrandomized study aimed to determine the influence of antifibrotic treatment on patients' reported symptoms and expectations of the therapy.MethodsFifty‐two patients with confirmed IPF at a mean age of 65 ± 8.63 years (73% male) completed the following surveys at baseline and after 12 months of Pirfenidone treatment: Short Form Healthy Survey (SF‐36), St. George's Respiratory Questionnaire (SGRQ), Baseline Dyspnea Index (BDI), Fatigue Assessment Scale (FAS), Leicester Cough Questionnaire (LCQ), and Patient's Needs and Expectations Authors' Survey.ResultsThe most important patients' needs were access to novel therapy, fast and easy access to health centers specializing in IPF treatment, and the improvement of the general condition or the maintenance of its level. These needs did not change with time, except for the significantly more important right of deciding on disease management after 12 months of treatment (p = 0.014). The quality of life per SF‐36, after 1 year of Pirfenidone treatment, significantly improved in the physical cumulative score (p = 0.004) and mental cumulative score (p = 0.003). Significant deteriorations were observed in bodily pain and vitality. For the remaining questionnaires (SGRQ, BDI, FAS, and LCQ), no significant changes in the course of the study were noticed. Around one in 10 patients subjected to Pirfenidone therapy had achieved general symptom improvement in all areas; that is, quality of life improvement as well as cough and dyspnea reduction.ConclusionsOne year of antifibrotic treatment resulted in a general improvement in the quality of life per the SF‐36 questionnaire. Patients' expectations of disease management did not change; also, access to novel therapies and easy access to health centers specializing in IPF management remained their top needs.

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Health-related Quality of Life in Patients with Idiopathic Pulmonary Fibrosis -Cross-sectional and Longitudinal Study-

Cited by 59 publications

References 25 publications

Physical Activity and Quality of Life Improvements of Patients With Idiopathic Pulmonary Fibrosis Completing a Pulmonary Rehabilitation Program

Physical Activity and Quality of Life Improvements of Patients With Idiopathic Pulmonary Fibrosis Completing a Pulmonary Rehabilitation Program

The clinical effectiveness and cost-effectiveness of treatments for idiopathic pulmonary fibrosis: a systematic review and economic evaluation

Expectations, symptoms, and quality of life before and after 1 year of Pirfenidone treatment in patients with idiopathic pulmonary fibrosis: A single‐arm, open‐label nonrandomized study

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