Article (Scientific journals)
Acute exacerbations in patients with progressive pulmonary fibrosis.
Kreuter, Michael; Belloli, Elizabeth A; Bendstrup, Elisabeth et al.
2024 • In ERJ Open Research, 10 (6), p. 00403-2024
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Keywords :
Pulmonary and Respiratory Medicine
Abstract :
[en] [en] BACKGROUND: Acute exacerbations of fibrosing interstitial lung diseases (ILDs) are associated with high mortality. We used prospective data from the INBUILD trial to investigate risk factors for acute exacerbations and the impact of these events in patients with progressive pulmonary fibrosis. METHODS: Patients with progressive fibrosing ILDs other than idiopathic pulmonary fibrosis (IPF) were randomised to receive nintedanib or placebo. Associations between baseline characteristics and time to first acute exacerbation were assessed using pooled data from both treatment groups using Cox proportional hazard models, firstly univariable models and then a multivariable model using forward stepwise selection. The risk of death was estimated based on the Kaplan-Meier method. RESULTS: Over a median follow-up of approximately 19 months, acute exacerbations were reported in 58 (8.7%) of 663 patients. In the risk factor analysis, the final model included diffusing capacity of the lung for carbon monoxide (D LCO) % predicted, treatment and age. Lower D LCO % predicted was associated with an increased risk of acute exacerbation with a hazard ratio (HR) of 1.56 (95% CI 1.21-2.02) per 10 units lower (p<0.001). Age ≥65 years was associated with a numerically increased risk (HR 1.55, 95% CI 0.87-2.77; p=0.14). Treatment with nintedanib conferred a numerically reduced risk versus placebo (HR 0.60, 95% CI 0.35-1.02; p=0.06). The estimated risks of death ≤30 days and ≤90 days after an acute exacerbation were 19.0% (95% CI 8.9-29.2) and 32.0% (95% CI 19.7-44.2). CONCLUSIONS: Acute exacerbations of progressive pulmonary fibrosis may have similar risk factors and prognostic impact as acute exacerbations of IPF.
Disciplines :
Cardiovascular & respiratory systems
Author, co-author :
Kreuter, Michael;  Center for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center and Pulmonary, Critical Care & Sleep Medicine, Marienhaus Clinic Mainz, Mainz, Germany
Belloli, Elizabeth A;  Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, USA
Bendstrup, Elisabeth ;  Department of Respiratory Diseases and Allergy, Centre for Rare Lung Diseases, Aarhus University Hospital, Aarhus, Denmark ; Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
Cerri, Stefania;  Center for Rare Lung Disease - Azienda Ospedaliero-Universitaria Policlinico di Modena, Modena, Italy
Flaherty, Kevin R;  Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI, USA
Shapera, Shane;  Division of Respirology, University Health Network, University of Toronto, Toronto, ON, Canada
Song, Jin Woo;  Department of Pulmonary and Critical Care Medicine, Asan Medical Center, Seoul, South Korea
Mueller, Heiko;  Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim am Rhein, Germany
Rohr, Klaus B;  Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany
Kondoh, Yasuhiro;  Department of Respiratory Medicine and Allergy, Tosei General Hospital, Japan
INBUILD trial investigators
Other collaborator :
Guiot, Julien  ;  Université de Liège - ULiège > Département des sciences cliniques > Pneumologie - Allergologie ; Université de Liège - ULiège > GIGA ; Université de Liège - ULiège > Département des sciences biomédicales et précliniques ; Centre Hospitalier Universitaire de Liège - CHU > > Service de pneumologie - allergologie
Language :
English
Title :
Acute exacerbations in patients with progressive pulmonary fibrosis.
Publication date :
November 2024
Journal title :
ERJ Open Research
eISSN :
2312-0541
Publisher :
European Respiratory Society
Volume :
10
Issue :
6
Pages :
00403-2024
Peer reviewed :
Peer Reviewed verified by ORBi
Funders :
Boehringer Ingelheim
Funding text :
The INBUILD trial was supported by Boehringer Ingelheim. Funding information for this article has been deposited with the Crossref Funder Registry.
Available on ORBi :
since 16 September 2026

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