Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry

Snyder, Laurie D.; Neely, Megan L.; Hellkamp, Anne S.; O’Brien, Emily C.; Andrade, Joao de; Conoscenti, Craig; Leonard, Thomas B.; Bender, Shaun; Gulati, Mridu; Culver, Daniel A.; Kaner, Robert J.; Palmer, Scott M.; Kim, Hyun Joo; Asi, Wael; Baker, Albert; Beegle, Scott; Belperio, John A.; Condos, Rany; Cordova, Francis; Dilling, Daniel F.; Flaherty, Kevin R.; Glassberg, Marilyn K.; Guntupalli, Kalpalatha K.; Gupta, Nishant; Case, Amy Hajari; Hotchkin, David; Huie, Tristan J.; Kreider, Maryl; Lancaster, Lisa; Lasky, Joseph A.; Lederer, David J.; Lee, Doug; Liesching, Timothy; Lipchik, Randolph J.; Lobo, Jason; Mageto, Yolanda; Menon, P. Suresh; Morrison, Lake; Namen, Andrew M.; Oldham, Justin M.; Raj, Rishi; Ramaswamy, Murali; Russell, Tonya; Sachs, Paul; Safdar, Zeenat; Sigal, Barry; Silhan, Leann; Strek, Mary E.; Suliman, Sally; Tabak, Jeremy; Walia, Rajat; Whelan, Timothy; Fleming, Julie W; Morris, Wendy L.

doi:10.1186/s12931-019-1043-9

Cited by 52 publications

(60 citation statements)

References 31 publications

(35 reference statements)

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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: 83%

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: 83%

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%

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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“…Previous qualitative research with patients and caregivers suggests that although supplemental oxygen use may improve activity and physical function, it may also negatively affect QOL by prohibiting patients from living "normal lives." 32 Given that supplemental oxygen use was associated with worse survival in the IPF-PRO registry, 33 oxygen use may also be a marker for underlying disease severity that negatively affects health-related QOL. Finally, given that the AmbOx trial was conducted in a single tertiary center in the United Kingdom, the findings may not be generalizable to real-world, multicenter registry populations such as included in the IPF-PRO registry.…”

Section: Discussionmentioning

confidence: 99%

Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis

investigators

2020

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BACKGROUND: Limited data are available on the association between clinically measured disease severity markers and quality of life (QOL) in idiopathic pulmonary fibrosis (IPF). The study examined the associations between objective disease severity metrics and QOL in a contemporary IPF population. METHODS: This study evaluated baseline data from patients enrolled in the multicenter, USbased Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry between June 2014 and July 2018. Disease severity metrics included FVC % predicted, diffusing capacity for carbon monoxide (DLCO) % predicted, supplemental oxygen use with activity, supplemental oxygen use at rest, and two summary scores (the Gender-Age-Lung Physiology index, based on gender, age, and % predicted values for DLCO and FVC; and the Composite Physiologic Index, based on % predicted values for DLCO, FVC, and FEV 1). Multivariable adjusted regression models were used to examine cross-sectional associations between each severity measure and St. George's Respiratory Questionnaire (SGRQ) total score. RESULTS: Among 829 patients with complete SGRQ data, the median (interquartile range) SGRQ score at enrollment was 40 (26-53), with higher scores indicating worse QOL. Modest SGRQ impairments were observed with increasing Gender-Age-Lung Physiology score (2.9 [1.8-4.0] per 1-point increase] and with increasing Composite Physiologic Index scores (3.0 [2.4-3.6] per 5-point increase). Substantial SGRQ impairments were observed for oxygen use with activity (15.6 [12.9-18.2]), oxygen use at rest (16.2 [13.0-19.4]), and decreasing DLCO (5.0 [4.0-6.1] per 10% decrease in % predicted). CONCLUSIONS: Objective measures of disease severity, including severity scores, physiologic parameters, and supplemental oxygen use, are associated with worse QOL in patients with IPF.

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Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry

Cited by 52 publications

References 31 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

Disease Severity and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis

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