Lung function trajectories in patients with idiopathic pulmonary fibrosis

Respiratory Research

Research

Shaun Bender¹  Eric S White¹  Tracy R Luckhardt²  Justin M Oldham³  Jamie L Todd⁴  Megan L Neely⁴  Anne S Hellkamp⁴  Scott M Palmer⁴  Timothy Liesching⁵  Rishi Raj⁶ 

[1] Boehringer Ingelheim Pharmaceuticals, Inc, Ridgefield, CT, USA;Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA;Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, USA;Duke Clinical Research Institute, Durham, NC, USA;Duke University Medical Center, Durham, NC, USA;Lahey Hospital & Medical Center, Burlington, MA, USA;Stanford University School of Medicine, Stanford, CA, USA;

关键词: Interstitial lung disease;    Lung function testing;    Forced vital capacity;   

DOI  :  10.1186/s12931-023-02503-5

 received in 2023-02-06, accepted in 2023-08-01,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundIdiopathic 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.MethodsPatients 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.ResultsOf 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.ConclusionsData 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 registrationNCT01915511.

【 授权许可】

CC BY   
© BioMed Central Ltd., part of Springer Nature 2023

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