scholarly journals The efficacy of pulmonary rehabilitation in improving the clinical status in idiopathic pulmonary fibrosis

2020 ◽  
pp. 35-44
Author(s):  
Nicoleta Stefania MOTOC ◽  
Anca Diana MAIEREAN ◽  
Milena Adina MAN ◽  
Teodora Gabriela ALEXESCU ◽  
Lorena CIUMARNEAN ◽  
...  

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fatal lung disease with a median survival rate of 2-4 years after diagnosis, occurring primarily in older adults. The diagnosis is suggested by histology or radiologic evidence of a usual interstitial pneumonia (UIP) pattern and exclusion of known cases of pulmonary fibrosis. There are some comorbidities associated with IPF such as pulmonary hypertension, emphysema, chronic obstructive pulmonary disease (COPD), asthma, lung cancer, cardiovascular disorders, gastroesophageal reflux disease (GERD), sleep disorders and psychiatric disturbances. The management of IPF focuses on the amelioration of symptoms, preserving lung function, improving health status, minimizing the adverse effects of therapy and improving survival. Pulmonary rehabilitation is suggested for IPF patients when adequate medical therapy controls poorly the disease progression and mental, physical or social consequences of the illness persist during daily life. Currently, there are only two approved available antifibrotic therapies, pirfenidone and nintedanib, capable to reduce disease progression and physical deterioration, but cure is elusive and improvements are hardly observed. In addition, there is a continuous need of non-drug therapy components which should be included in IPF patient management: education, psychosocial support, exercise training, nutrition, symptom management and palliative care, non-invasive ventilation and pulmonary transplant. These complementary therapies have been proven to improve dyspnea, exercise capacity, fatigue and quality of life. Key words: idiopathic pulmonary fibrosis, rehabilitation, quality of life, palliative care,

2018 ◽  
Vol 38 (5) ◽  
pp. 273-278 ◽  
Author(s):  
Mansueto Gomes-Neto ◽  
Cassio Magalhães Silva ◽  
Diego Ezequiel ◽  
Cristiano Sena Conceição ◽  
Micheli Saquetto ◽  
...  

2017 ◽  
Vol 3 (4) ◽  
pp. 00084-2017 ◽  
Author(s):  
Kaisa Rajala ◽  
Juho T. Lehto ◽  
Eva Sutinen ◽  
Hannu Kautiainen ◽  
Marjukka Myllärniemi ◽  
...  

This study was undertaken to investigate idiopathic pulmonary fibrosis (IPF) patients' health-related quality of life (HRQoL) and symptoms in a real-life cross-sectional study. Our secondary aim was to create a simple identification method for patients with increased need for palliative care by studying the relationship between modified Medical Research Council (mMRC) dyspnoea scale, HRQoL and symptoms.We sent a self-rating HRQoL questionnaire (RAND-36) and modified Edmonton Symptom Assessment Scale (ESAS) to 300 IPF patients; 84% of the patients responded to these questionnaires.The most prevalent (>80%) symptoms were tiredness, breathlessness, cough and pain in movement. An increasing mMRC score showed a linear relationship (p<0.001) to impaired HRQoL in all dimensions of RAND-36 and the severity of all symptoms in ESAS. Dimensions of RAND-36 fell below general population reference values in patients with mMRC score ≥2. The intensity of pain in movement (p<0.001) and at rest (p=0.041), and the prevalence of chest pain (p<0.001) had a positive linear relationship to increased mMRC score.An increasing mMRC score reflects impaired HRQoL and a high symptom burden. In clinical practice, the mMRC scale could be used for screening and identification of IPF patients with increased need for palliative care.


2016 ◽  
Vol 48 (1) ◽  
pp. 187-195 ◽  
Author(s):  
Robert B. Hopkins ◽  
Natasha Burke ◽  
Charlene Fell ◽  
Genevieve Dion ◽  
Martin Kolb

Idiopathic pulmonary fibrosis (IPF) is a rare disease, with estimates of prevalence varying considerably across countries due to paucity in data collection. The aim of this study was to investigate the prevalence and incidence of IPF in Canada using administrative data requiring minimal extrapolation.We used mandatory national administrative data from 2007–2011 to identify IPF cases of all ages with an International Classification of Diseases (Version 10, Canadian) diagnosis code of J84.1. We used a broad definition that excluded cases with subsequent diagnosis of other interstitial lung diseases, and a narrow definition that required further diagnostic testing prior to IPF diagnosis. We explored survival and quality of life.For all ages, the broad prevalence of IPF was 41.8 per 100 000 (14 259 cases) and was higher for men. The incidence rate was 18.7 per 100 000 (6390 cases) and was higher for men. The narrow prevalence was 20.0 per 100 000 (6822 cases) and incidence was 9.0 per 100 000 (3057 cases). The 4-year risk of death was 41.0% and the quality of life with IPF after 2 years was lower than for Global Initiative for Chronic Obstructive Lung Disease stage IV chronic obstructive pulmonary disease.Using comprehensive national data, the prevalence of IPF in Canada was higher than other national estimates, suggesting that either IPF may be more common in Canada or that data capture may have been previously limited.


2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Revati Amin ◽  
K. Vaishali ◽  
G. Arun Maiya ◽  
Aswini Kumar Mohapatra ◽  
Uday Narayan Yadav ◽  
...  

Abstract Background Idiopathic pulmonary fibrosis (IPF) is one of the common types of interstitial lung disease having high prevalence and mortality worldwide. As a result of patient-centred hindering factors of adherence to centre-based pulmonary rehabilitation (PR), home-based PR is an alternate mode of rehabilitating individuals with IPF. This systematic review will evaluate the effectiveness of unsupervised home-based PR on functional capacity and health-related quality of life (HRQoL) in individuals with IPF. Methods Clinically stable, high resolution computed tomography and physician diagnosed IPF participants having modified Medical Research Council score below 5 will be considered for the systematic review. Studies involving home-based PR as an intervention to treat individuals with IPF will be considered. Randomised controlled trials and quasi-randomised studies (with two groups followed up) are eligible to be included. Outcomes of our interest are functional capacity (6-min walk distance, shuttle walk test and incremental shuttle walk test) and secondary outcome measure would include assessment of quality of life and adverse effects of intervention. Electronic databases such as SCOPUS, Medline (PubMed and Web of Science), PEDRo and CINAHL will be searched using database specific terms. Additionally, forward and backward citations of included studies will be searched to identify potential records. Two review authors, independently, will conduct the screening, data extraction using a customised standard tool, and critical appraisal using Cochrane Risk of Bias 2 tool of included studies. If data permits, meta-analysis will be conducted. In case of substantial heterogeneity, we will do a narrative synthesis. Subgroup analysis will be undertaken based on various contextual and interventional factors. Discussion This review will provide comprehensive evidence on the effectiveness of unsupervised home-based PR to physiotherapists, policy makers and researchers who are interested in IPF management. Findings from this review may guide the development and evaluation of more robust evidence based home-based PR that aimed to improve functional capacity among people with IPF. Systematic review registration PROSPERO CRD42020213883.


2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Katherine Janssen ◽  
Drew Rosielle ◽  
Qi Wang ◽  
Hyun Joo Kim

Abstract Background Idiopathic pulmonary fibrosis (IPF) is a fatal disease that results in poor quality of life due to progressive respiratory symptoms, anxiety, and depression. Palliative care improves quality of life and survival in other progressive diseases. No randomized controlled trials have investigated the impact of palliative care on quality of life, anxiety, or depression in IPF. Methods We conducted a randomized, controlled, pilot study to assess the feasibility of measuring the effect of a palliative care clinic referral on quality of life, anxiety, and depression in IPF. Patients were randomized to usual care (UC) or usual care + palliative care (UC + PC) with routine pulmonary follow up at 3 and 6 months. The UC + PC group received a minimum of one PC clinic visit. Primary outcome was change from baseline in quality of life, anxiety, and depression as measured by the St. George’s Respiratory Questionnaire (SGRQ), the Hospital Anxiety and Depression Index (HADS), and the Patient Health Questionnaire (PHQ-9) at 6 months. Results Twenty-two patients were randomized between September 2017 through July 2018; 11 to UC and 11 to UC + PC. There was no difference in the change in SGRQ score at 3 months or 6 months, however, the symptom score trended towards a significant worsening for UC + PC at both 3 and 6 months (mean change at 3 months for UC and UC + PC was − 7.8 and + 10.7, respectively, p = 0.066; mean change at 6 months for UC and UC + PC was − 6.0 and + 4.6, respectively, p = 0.055). There was no difference in the change in HADS anxiety or depression scores. There was a significant transient worsening in PHQ-9 scores for UC + PC at 3 months (UC: -1.6, UC + PC: + 0.9, p = 0.008); this effect did not persist at 6 months. Conclusion This pilot study demonstrated that a randomized controlled trial of palliative care in idiopathic pulmonary fibrosis patients is feasible. Receiving palliative care did not lead to improved quality of life, anxiety, or depression compared to usual care after 6 months. Patients in the UC + PC group trended towards worsening symptoms and a small but statistically significant transient worsening in depression. These findings should be interpreted with caution, and need to be evaluated in adequately powered clinical trials. NCT03981406, June 10, 2019, retrospectively registered.


2019 ◽  
Vol 6 (1) ◽  
pp. e000422 ◽  
Author(s):  
Toby M Maher ◽  
Michael Kreuter ◽  
David J Lederer ◽  
Kevin K Brown ◽  
Wim Wuyts ◽  
...  

IntroductionWhile current standard of care (SOC) for idiopathic pulmonary fibrosis (IPF) slows disease progression, prognosis remains poor. Therefore, an unmet need exists for novel, well-tolerated agents that reduce lung function decline and improve quality of life. Here we report the design of two phase III studies of the novel IPF therapy, GLPG1690.Methods and analysisTwo identically designed, phase III, international, randomised, double-blind, placebo-controlled, parallel-group, multicentre studies (ISABELA 1 and 2) were initiated in November 2018. It is planned that, in each study, 750 subjects with IPF will be randomised 1:1:1 to receive oral GLPG1690 600 mg, GLPG1690 200 mg or placebo, once daily, on top of local SOC, for at least 52 weeks. The primary endpoint is rate of decline of forced vital capacity (FVC) over 52 weeks. Key secondary endpoints are week 52 composite endpoint of disease progression or all-cause mortality (defined as composite endpoint of first occurrence of ≥10% absolute decline in per cent predicted FVC or all-cause mortality at week 52); time to first respiratory-related hospitalisation until end of study; and week 52 change from baseline in the St George’s Respiratory Questionnaire total score (a quality-of-life measure).Ethics and disseminationStudies will be conducted in accordance with Good Clinical Practice guidelines, Declaration of Helsinki principles, and local ethical and legal requirements. Results will be reported in a peer-reviewed publication.Trial registration numbersNCT03711162; NCT03733444.


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