EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis

Doubková, Martina; Švancara, Jan; Svoboda, Michal; Šterclová, Martina; Bartoš, Vladimı́r; Plačková, Martina; Lacina, Ladislav; Žůrková, Monika; Binková, Ilona; Bittenglová, Radka; Lošťáková, Vladimíra; Merta, Zdeněk; Šišková, Lenka; Tyl, Richard; Lisá, Pavlína; Šuldová, Hana; Petřík, František; Pšíkalová, Jana; Řihák, Vladimír; Snížek, Tomáš; Reiterer, Pavel; Homolka, J; Musilova, Pavlina; Lněnička, Jaroslav; Paluch, Peter; Hrdina, R.; Králová, Renata; Hortvíková, Hana; Strenková, Jana; Vašáková, Martina

doi:10.1111/crj.12700

Cited by 69 publications

(75 citation statements)

References 41 publications

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“…A wealth of data has demonstrated that low FVC and/or low DLco are risk factors for acute exacerbations in patients with IPF [11,32,33], possibly because patients with more advanced disease are more vulnerable to insults such as infection that may lead to an acute exacerbation [11]. Similarly, the risk of mortality was higher in patients with more severe impairment in DLco in the INSTAGE trial than in patients in the INPULSIS trials, consistent with previous studies identifying low DLco as a risk factor for mortality in patients with IPF [4,33,34].…”

Section: Discussionsupporting

confidence: 84%

“…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]. A better evidence base for the treatment of severe IPF remains an unmet need [5].…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionsupporting

confidence: 84%

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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“…Studies in patients with progressive fibrosing ILDs have identified several factors that predict mortality, but these need to be interpreted carefully given the variation in the methodology used and the retrospective nature of most of the studies. Lower FVC is an established predictor of mortality in patients with progressive fibrosing ILDs, as evidenced by numerous studies spanning IPF [55][56][57], RA-ILD [4,49], SSc-ILD [58][59][60], chronic HP [61,62] and fibrotic iNSIP [53]. The same is true of DLco [55,56,60,[63][64][65], although this is harder to assess in multi-center studies due to a lack of standardization in its measurement.…”

Section: Predictors Of Disease Progression In Patients With Fibrosingmentioning

confidence: 99%

“…Lower FVC is an established predictor of mortality in patients with progressive fibrosing ILDs, as evidenced by numerous studies spanning IPF [55][56][57], RA-ILD [4,49], SSc-ILD [58][59][60], chronic HP [61,62] and fibrotic iNSIP [53]. The same is true of DLco [55,56,60,[63][64][65], although this is harder to assess in multi-center studies due to a lack of standardization in its measurement. A decline in FVC > 10% predicted is another well-established predictor of mortality [49,56,62,65], but smaller declines in FVC have also been shown to be associated with a worse prognosis, at least in patients with IPF [66][67][68].…”

Section: Predictors Of Disease Progression In Patients With Fibrosingmentioning

confidence: 99%

The natural history of progressive fibrosing interstitial lung diseases

2019

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A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.

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“…However, only a small percentage of patients included in these registries were treated with novel agents – all as part of clinical trials or specialized access programs –, therefore further recording of patients is needed in order to assess the effect of novel agents in current clinical practice. Such registries are already ongoing [4, 26-28]. The last issue regarding the heterogeneity of a disease with relative low incidence and prevalence is the acquisition of data for the treatment of special subpopulations.…”

Section: Introductionmentioning

confidence: 99%

Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF)

et al. 2018

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Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial pneumonia of unknown origin. Despite the fact that the guidelines on the diagnosis and management of the disease were updated in 2015, incorporating novel agents recently introduced in the therapeutic approach of IPF, there is a lack of data on the epidemiology, disease status, and treatment in clinical practice. Contemporary data provided by national registries in IPF provide valuable information to guide clinical management of the disease in the real-world setting, adjusted to the local needs. Objective: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF) is a Greek observational registry aiming at gaining further knowledge on the characteristics, management, progression, and outcomes of patients with IPF treated under real-world, clinical practice conditions in Greece. Methods: Approximately 300 patients will be enrolled consecutively in seven reference centers, constituting the largest IPF registry ever established in Greece. Conclusion: This registry is expected to provide data on the characteristics of IPF patients in Greece and the entire clinical management during the course of the disease.

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EMPIRE Registry, Czech Part: Impact of demographics, pulmonary function and HRCT on survival and clinical course in idiopathic pulmonary fibrosis

Abstract: DL changes over time were shown as a better predictor of mortality compared with FVC changes in our study. In our opinion it is necessary to implement the DL analysis into clinical trials and routine practice.

Cited by 69 publications

References 41 publications

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

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

The natural history of progressive fibrosing interstitial lung diseases

Design, Rationale, Methodology, and Aims of a Greek Prospective Idiopathic Pulmonary Fibrosis Registry: Investigating Idiopathic Pulmonary Fibrosis in Greece (INDULGE IPF)

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