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Observational Study
. 2023 Aug 24;24(1):209.
doi: 10.1186/s12931-023-02503-5.

Lung function trajectories in patients with idiopathic pulmonary fibrosis

Megan L Neely  1   2 , Anne S Hellkamp  3   4 , Shaun Bender  5 , Jamie L Todd  3   4 , Timothy Liesching  6 , Tracy R Luckhardt  7 , Justin M Oldham  8 , Rishi Raj  9 , Eric S White  5 , Scott M Palmer  3   4
Affiliations
Observational Study

Lung function trajectories in patients with idiopathic pulmonary fibrosis

Megan L Neely et al. Respir Res. .

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterised by decline in lung function. We evaluated trajectories of forced vital capacity (FVC) and diffusing capacity (DLco) in a cohort of patients with IPF.

Methods: Patients with IPF that was diagnosed or confirmed at the enrolling centre in the previous 6 months were enrolled into the IPF-PRO Registry between June 2014 and October 2018. Patients were followed prospectively, with lung function data collected as part of routine clinical care. Mean trajectories of FVC and DLco % predicted in all patients and in subgroups by characteristics assessed at enrolment were estimated using a joint model that accounted for factors such as disease severity and visit patterns.

Results: Of 1002 patients in the registry, 941 had ≥ 1 FVC and/or DLco measurement after enrolment. The median (Q1, Q3) follow-up period was 35.1 (18.9, 47.2) months. Overall, mean estimated declines in FVC and DLco % predicted were 2.8% and 2.9% per year, respectively. There was no evidence that the mean trajectories of FVC or DLco had a non-linear relationship with time at the population level. Patients who were male, white, had a family history of ILD, were using oxygen, or had prior/current use of antifibrotic therapy at enrolment had greater rates of decline in FVC % predicted. Patients who were male or white had greater rates of decline in DLco % predicted.

Conclusions: Data from the IPF-PRO Registry suggest a constant rate of decline in lung function over a prolonged period, supporting the inexorably progressive nature of IPF. A graphical abstract summarising the data in this manuscript is available at: https://www.usscicomms.com/respiratory/IPF-PRORegistry_LungFunctionTrajectories .

Trial registration: NCT01915511.

Keywords: Forced vital capacity; Interstitial lung disease; Lung function testing.

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Conflict of interest statement

Megan L Neely, Anne S Hellkamp, Jamie L Todd and Scott M Palmer are employees of the Duke Clinical Research Institute (DCRI), which receives funding support from Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI) to coordinate the IPF-PRO/ILD-PRO Registry. Jamie L Todd reports grants paid to her institution from AstraZeneca and CareDx and has participated on advisory boards for Altavant Sciences, Natera, Sanofi, Theravance. Scott M Palmer reports research funding paid to DCRI from Bristol-Myers Squibb and Genentech and has participated on advisory boards for Altavant and Bristol-Myers Squibb. Shaun Bender was an employee of BIPI at the time that this study was conducted. Timothy Liesching, Justin M Oldham, Tracy R Luckhardt and Rishi Raj are principal investigators at enrolling centres for the IPF-PRO Registry. Tracy R Luckhardt serves on the IPF-PRO/ILD-PRO Registry Biomarker Committee. Justin M Oldham serves on the IPF-PRO/ILD-PRO Registry Publication Committee. Rishi Raj reports an investigator-initiated research grant from and has served on an advisory board for BI. Eric S White is an employee of BIPI.

Figures

Fig. 1
Fig. 1
Modelling of (a) estimated mean FVC % predicted values over time and (b) estimated mean DLco % predicted values over time
Fig. 2
Fig. 2
Modelling of estimated mean FVC % predicted values in subgroups with significant interactions with time
Fig. 3
Fig. 3
Modelling of estimated mean FVC % predicted values in subgroups with significant associations with FVC % predicted at baseline but without significant interactions with time
Fig. 4
Fig. 4
Modelling of estimated mean DLco % predicted values in subgroups with significant interactions with time
Fig. 5
Fig. 5
Modelling of estimated mean DLco % predicted values in subgroups with significant associations with DLco % predicted at baseline but without significant interactions with time. Definite, probable and possible IPF according to 2011 ATS/ERS/JRS/ALAT diagnostic guidelines [19]
See this image and copyright information in PMC

References

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