Decline in Forced Vital Capacity as a Surrogate for Mortality in Patients with Fibrosing Interstitial Lung Diseases

Maher, T.M.; Bendstrup, Elisabeth; Kreuter, Michael; Martinez, F.J.; Sime, Patricia J.; Stowasser, Susanne; Voß, Florian; Stock, Christian

doi:10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a1851

Cited by 3 publications

(5 citation statements)

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“…Moreover, studies that assessed the link between FVC and survival found that mortality rate was significantly associated with a relative decline in FVC % predicted (2,21). These results are consistent with a post-hoc analysis of 2,553 patients who received nintedanib or placebo in clinical trials in patients with non-IPF PF-ILD (INBUILD), IPF (TOMORROW, INPULSIS-1 and−2, and NCT01979952), and SSc-ILD (SENSCIS), and also demonstrated a strong association between decline in FVC % predicted and death (42).…”

Section: Discussionsupporting

confidence: 85%

“…In IPF, there is evidence to suggest that patients who initiate nintedanib soon after IPF diagnosis may have a reduced hospitalization risk and lower medical costs (54). As QoL and survival rate are strongly associated with lung function (2,11,21,42), coupled with HCRU and medical costs increasing over time for non-IPF PF-ILD patients (34), early nintedanib treatment may theoretically reduce the substantial humanistic, economic and survival burden associated with PF-ILD.…”

Section: Discussionmentioning

confidence: 99%

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The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Cottin

Teague²,

Nicholson³

et al. 2022

Front. Med.

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Despite conventional treatment, a proportion of interstitial lung disease (ILD) patients develop a progressive phenotype known as “fibrosing ILD with a progressive phenotype” (PF-ILD), characterized by worsening respiratory symptoms, decline in lung function, and early mortality. This review describes the epidemiology, and the humanistic and economic burden of PF-ILDs other than idiopathic pulmonary fibrosis (non-IPF PF-ILD). A structured review of the literature was conducted, using predefined search strategies in Ovid MEDLINE and EMBASE, and supplemented with gray literature searches. The search identified 3,002 unique articles and an additional 3 sources were included from the gray literature; 21 publications were included. The estimated prevalence of non-IPF PF-ILD ranges from 6.9 to 70.3/100,000 persons and the estimated incidence from 2.1 to 32.6/100,000 person-years. Limited evidence demonstrates that PF-ILD has a significant impact on patients' quality of life, affecting their daily lives, psychological well-being, careers, and relationships. PF-ILD is also associated with significant economic burden, demonstrating higher healthcare resource use and direct costs compared with the non-progressive phenotype, and indirect costs, which include job losses. This review indicates that PF-ILD places a considerable humanistic burden on both patients and caregivers, and a substantial economic burden on healthcare systems, patients, and society.

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Section: Discussionsupporting

confidence: 85%

Section: Discussionmentioning

confidence: 99%

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Cottin

Teague²,

Nicholson³

et al. 2022

Front. Med.

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“…However, the rate reported in the study by Reichmann et al was for IPF-related inpatient hospitalizations and not for all-cause inpatient hospitalization as reported in our study. In addition to evaluating the relationship between inpatient hospitalization and FVC decline, studies within IPF and chronic fibrosing ILD have shown the relationship between FVC decline and other outcomes, including mortality and acute exacerbations [ 14 , 25 – 30 ]. Although these outcomes were not evaluated in our study, these studies provide additional evidence of the importance of maintaining FVC on health outcomes in patients with chronic fibrosing ILDs.…”

Section: Discussionmentioning

confidence: 99%

Impact of chronic fibrosing interstitial lung disease on healthcare use: association between fvc decline and inpatient hospitalization

Singer,

Chastek,

Sargent

et al. 2023

BMC Pulm Med

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Background Many types of interstitial lung diseases (ILDs) may transition to progressive chronic-fibrosing ILDs with rapid lung function decline and a negative survival prognosis. In real-world clinical settings, forced vital capacity (FVC) measures demonstrating progressive decline may be linked to negative outcomes, including increased risks of costly healthcare resource utilization (HRU). Thus, we assessed the relationship between rate of decline in lung function and an increase in HRU, specifically inpatient hospitalization, among patients with chronic fibrosing ILD. Methods This study utilized electronic health records from 01-Oct-2015 to 31-Oct-2019. Eligible patients (≥ 18 years old) had ≥ 2 fibrosing ILD diagnosis codes, clinical activity for ≥ 15 months, and ≥ 2 FVC tests occurring 6 months apart. Patients with missing demographic data, IPF, or use of nintedanib or pirfenidone were excluded. Two groups were defined by relative change in percent of predicted FVC (FVC% pred) from baseline to 6 months: significant decline (≥ 10%) vs. marginal decline/stable FVC (decrease < 10% or increase). The primary outcome was defined as the occurrence of an inpatient hospitalization 6 months after the first FVC value. Descriptive and multivariable analysis was conducted to examine the impact of FVC decline on occurrence of inpatient hospitalization. Results The sample included 566 patients: 13% (n = 75) with significant decline and 87% (n = 491) with marginal decline/stable FVC; their mean age (SD) was 65 (13.7) years and 56% were female. Autoimmune diagnoses were observed among 40% of patients with significant decline, and 27% with marginal decline/stable FVC. The significant decline group had better lung function at baseline than the marginal/stable group. For patients with FVC% <80% at baseline, reduction of FVC% ≥10% was associated with significantly increased odds of an inpatient hospitalization (odds ratio [OR] 2.85; confidence interval [CI] 1.17, 6.94 [p = 0.021]). Conclusion Decline in FVC% ≥10% was associated with increased odds of inpatient hospitalization among patients with reduced lung function at baseline. These findings support the importance of preserving lung function among patients with fibrosing ILD.

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“…In adults with fibrosing ILDs, decline in forced vital capacity (FVC) is reflective of disease progression and is associated with mortality 10–13 . Placebo‐controlled trials have demonstrated that nintedanib, an intracellular inhibitor of tyrosine kinases, has a consistent effect on slowing decline in FVC in adults with fibrosing ILDs of diverse etiology 14 .…”

Section: Introductionmentioning

confidence: 99%

“…[7][8][9] In adults with fibrosing ILDs, decline in forced vital capacity (FVC) is reflective of disease progression and is associated with mortality. [10][11][12][13] Placebo-controlled trials have demonstrated that nintedanib, an intracellular inhibitor of tyrosine kinases, has a consistent effect on slowing decline in FVC in adults with fibrosing ILDs of diverse etiology. 14 Based on these data, and the similarities in the biological pathways that lead to pulmonary fibrosis in adults and children, 2,6,7 the effects of nintedanib in 39 children and adolescents with fibrosing ILD were investigated in the placebo-controlled InPedILD trial.…”

Section: Introductionmentioning

confidence: 99%

Estimating the effect of nintedanib on forced vital capacity in children and adolescents with fibrosing interstitial lung disease using a Bayesian dynamic borrowing approach

Maher,

Brown,

Cunningham

et al. 2024

Pediatric Pulmonology

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BackgroundThe rarity of childhood interstitial lung disease (chILD) makes it challenging to conduct powered trials. In the InPedILD trial, among 39 children and adolescents with fibrosing ILD, there was a numerical benefit of nintedanib versus placebo on change in forced vital capacity (FVC) over 24 weeks (difference in mean change in FVC % predicted of 1.21 [95% confidence interval: −3.40, 5.81]). Nintedanib has shown a consistent effect on FVC across populations of adults with different diagnoses of fibrosing ILD.MethodsIn a Bayesian dynamic borrowing analysis, prespecified before data unblinding, we incorporated data on the effect of nintedanib in adults and the data from the InPedILD trial to estimate the effect of nintedanib on FVC in children and adolescents with fibrosing ILD. The data from adults were represented as a meta‐analytic predictive (MAP) prior distribution with mean 1.69 (95% credible interval: 0.49, 3.08). The adult data were weighted according to expert judgment on their relevance to the efficacy of nintedanib in chILD, obtained in a formal elicitation exercise.ResultsCombined data from the MAP prior and InPedILD trial analyzed within the Bayesian framework resulted in a median difference between nintedanib and placebo in change in FVC % predicted at Week 24 of 1.63 (95% credible interval: −0.69, 3.40). The posterior probability for superiority of nintedanib versus placebo was 95.5%, reaching the predefined success criterion of at least 90%.ConclusionThese findings, together with the safety data from the InPedILD trial, support the use of nintedanib in children and adolescents with fibrosing ILDs.

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Decline in Forced Vital Capacity as a Surrogate for Mortality in Patients with Fibrosing Interstitial Lung Diseases

Cited by 3 publications

References 0 publications

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Impact of chronic fibrosing interstitial lung disease on healthcare use: association between fvc decline and inpatient hospitalization

Estimating the effect of nintedanib on forced vital capacity in children and adolescents with fibrosing interstitial lung disease using a Bayesian dynamic borrowing approach

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