Lung function trajectories in patients with idiopathic pulmonary fibrosis
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..
Medienart: |
E-Artikel |
---|
Erscheinungsjahr: |
2023 |
---|---|
Erschienen: |
2023 |
Enthalten in: |
Zur Gesamtaufnahme - volume:24 |
---|---|
Enthalten in: |
Respiratory research - 24(2023), 1 vom: 24. Aug. |
Sprache: |
Englisch |
---|
Beteiligte Personen: |
Neely, Megan L [VerfasserIn] |
---|
Links: |
Volltext [kostenfrei] |
---|
Themen: |
Forced vital capacity |
---|
Anmerkungen: |
© The Author(s) 2023 |
---|
doi: |
10.1186/s12931-023-02503-5 |
---|
funding: |
|
---|---|
Förderinstitution / Projekttitel: |
|
PPN (Katalog-ID): |
SPR052847950 |
---|
LEADER | 01000caa a22002652 4500 | ||
---|---|---|---|
001 | SPR052847950 | ||
003 | DE-627 | ||
005 | 20231121064842.0 | ||
007 | cr uuu---uuuuu | ||
008 | 230824s2023 xx |||||o 00| ||eng c | ||
024 | 7 | |a 10.1186/s12931-023-02503-5 |2 doi | |
035 | |a (DE-627)SPR052847950 | ||
035 | |a (SPR)s12931-023-02503-5-e | ||
040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
041 | |a eng | ||
100 | 1 | |a Neely, Megan L |e verfasserin |4 aut | |
245 | 1 | 0 | |a Lung function trajectories in patients with idiopathic pulmonary fibrosis |
264 | 1 | |c 2023 | |
336 | |a Text |b txt |2 rdacontent | ||
337 | |a Computermedien |b c |2 rdamedia | ||
338 | |a Online-Ressource |b cr |2 rdacarrier | ||
500 | |a © The Author(s) 2023 | ||
520 | |a 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. | ||
650 | 4 | |a Interstitial lung disease |7 (dpeaa)DE-He213 | |
650 | 4 | |a Lung function testing |7 (dpeaa)DE-He213 | |
650 | 4 | |a Forced vital capacity |7 (dpeaa)DE-He213 | |
700 | 1 | |a Hellkamp, Anne S |4 aut | |
700 | 1 | |a Bender, Shaun |4 aut | |
700 | 1 | |a Todd, Jamie L |4 aut | |
700 | 1 | |a Liesching, Timothy |4 aut | |
700 | 1 | |a Luckhardt, Tracy R |4 aut | |
700 | 1 | |a Oldham, Justin M |4 aut | |
700 | 1 | |a Raj, Rishi |4 aut | |
700 | 1 | |a White, Eric S |4 aut | |
700 | 1 | |a Palmer, Scott M |4 aut | |
773 | 0 | 8 | |i Enthalten in |t Respiratory research |d London : BioMed Central, 2001 |g 24(2023), 1 vom: 24. Aug. |w (DE-627)SPR028499905 |w (DE-600)2041675-1 |x 1465-993X |7 nnns |
773 | 1 | 8 | |g volume:24 |g year:2023 |g number:1 |g day:24 |g month:08 |
856 | 4 | 0 | |u https://dx.doi.org/10.1186/s12931-023-02503-5 |z kostenfrei |3 Volltext |
912 | |a GBV_USEFLAG_A | ||
912 | |a GBV_SPRINGER | ||
951 | |a AR | ||
952 | |d 24 |j 2023 |e 1 |b 24 |c 08 |