Severe idiopathic pulmonary fibrosis: what can be done?

Caminati, Antonella; Cassandro, Roberto; Torre, Olga

doi:10.1183/16000617.0047-2017

Cited by 37 publications

(15 citation statements)

References 84 publications

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“…Although the disease trajectory of IPF is variable, for many patients with IPF, survival is worse than many common malignancies. This necessitates early integration of palliative care to improve patients’ quality of life (QOL) and to relieve symptoms in addition to disease-specific pharmacological treatment and lung transplant assessment [ 5 – 9 ].…”

Section: Introductionmentioning

confidence: 99%

Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

et al. 2018

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BackgroundIdiopathic pulmonary fibrosis (IPF) is a chronic disease with a high symptom burden and poor survival that influences patients’ health-related quality of life (HRQOL). We aimed to evaluate IPF patients’ symptoms and HRQOL in a well-documented clinical cohort during their last two years of life.MethodsIn April 2015, we sent the Modified Medical Research Council Dyspnea Scale (MMRC), the modified Edmonton Symptom Assessment Scale (ESAS) and a self-rating HRQOL questionnaire (RAND-36) to 300 IPF patients, of which 247 (82%) responded. Thereafter, follow-up questionnaires were sent every six months for two years.ResultsNinety-two patients died by August 2017. Among these patients, HRQOL was found to be considerably low already two years before death. The most prominent declines in HRQOL occurred in physical function, vitality, emotional role and social functioning (p < 0.001). The proportion of patients with MMRC scores ≥3 increased near death. Breathlessness and fatigue were the most severe symptoms. Symptom severity for the following symptoms increased significantly and reached the highest mean scores during the last six months of life (numeric rating scale/standard deviation): breathlessness (7.1/2.8), tiredness (7.0/2.3), dry mouth (6.0/3.0), cough (5.8/2.9), and pain with movement (5.0/3.5).ConclusionsTo our knowledge this is the first study demonstrating, that IPF patients experience remarkably low HRQOL already two years before death, especially regarding physical role. In addition, they suffer from severe breathlessness and fatigue. Furthermore, physical, social and emotional wellbeing deteriorate, and symptom burden increases near death. Regular symptom and HRQOL measurements are essential to assess palliative care needs in patients with IPF.Electronic supplementary materialThe online version of this article (10.1186/s12890-018-0738-x) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

et al. 2018

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“…Both nintedanib and pirfenidone are approved by regulatory agencies worldwide for treatment of IPF and received conditional recommendations in the IPF guidelines [4,11]. Notably, nintedanib and pirfenidone have been shown to have similar effects on rate of decline in FVC over time, even among patients with normal FVC and among patients with more advanced disease (FVC <50% predicted) [28][29][30][31][32][33][34]. Nintedanib has been shown to have similar effects on FVC among patients with definite UIP on HRCT or surgical biopsy in comparison to those with possible UIP and traction bronchiectasis, suggesting that efficacy is independent of IPF phenotype [35].…”

Section: Antifibrotic Medicationsmentioning

confidence: 99%

Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis

2019

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Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifesting other “non-IPF progressive fibrotic interstitial lung diseases” (non-IPF-PF) may be similar to those in patients with IPF. Thus, it is biologically plausible that pharmacological agents with antifibrotic properties may be efficacious in non-IPF-PF. Indeed, studies are underway or planned to assess the safety and efficacy of nintedanib or pirfenidone among patients with several non-IPF fibrotic lung diseases. In this review, we briefly summarise the use of pirfenidone and nintedanib in IPF as well as the rationale and potential for use of these medications in non-IPF-PF that are being investigated in ongoing and upcoming clinical trials.

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“…While IPF progresses in all patients, the pattern of disease progression is variable and remains a challenge to predict [3,4]. A better evidence base for the treatment of severe IPF remains an unmet need [5].…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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Background: The two 52-week INPULSIS trials investigated nintedanib versus placebo in patients with IPF, FVC ≥50% predicted and DLco 30-79% predicted. The 24-week INSTAGE trial investigated nintedanib plus sildenafil versus nintedanib alone in patients with IPF and DLco ≤35% predicted. We used data from INPULSIS and INSTAGE to compare the effects of nintedanib in patients with IPF with less versus more severe impairment in gas exchange at baseline. Methods: Analyses were conducted in patients treated with nintedanib alone in the INPULSIS and INSTAGE trials and in patients treated with placebo in the INPULSIS trials. Outcomes included the rate of decline in FVC over 24 weeks, the proportions of patients who had a confirmed or suspected idiopathic acute exacerbation over 24 weeks, deaths over 24 weeks, and adverse events. Analyses were descriptive. Results: In total, 638 and 136 patients received nintedanib alone in the INPULSIS and INSTAGE trials, respectively, and 423 patients received placebo in the INPULSIS trials. Rates of FVC decline were − 52.3 and − 66.7 mL/24 weeks in patients treated with nintedanib alone in INPULSIS and INSTAGE, respectively, and − 102.8 mL/24 weeks in patients treated with placebo in INPULSIS. Confirmed or suspected idiopathic acute exacerbations were reported in 0.6 and 3.7% of patients treated with nintedanib alone in INPULSIS and INSTAGE, respectively, and 2.1% of patients treated with placebo in INPULSIS. Deaths occurred in 2.0, 11.0 and 1.9% of patients in these groups, respectively. Diarrhoea adverse events were reported in 52.5 and 48.5% of patients treated with nintedanib alone in INPULSIS and INSTAGE, respectively, and 16.1% of patients treated with placebo in INPULSIS.Conclusions: Based on data from the INSTAGE and INPULSIS trials, nintedanib had a similar effect on FVC decline over 24 weeks, and a similar safety and tolerability profile, in patients with IPF and more versus less severe impairment in gas exchange. These data support the use of nintedanib in patients with IPF who have advanced disease.Trial registration: INPULSIS (NCT01335464 and NCT01335477); INSTAGE (NCT02802345).

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Severe idiopathic pulmonary fibrosis: what can be done?

Cited by 37 publications

References 84 publications

Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

Marked deterioration in the quality of life of patients with idiopathic pulmonary fibrosis during the last two years of life

Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis

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

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