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Peer-reviewedCC BY-NCERJ open research

Progressive pulmonary fibrosis in fibrotic interstitial lung disease: a Danish cohort study

  • Line Kølner-Augustson1,2
  • Malene Knarborg3
  • Nanna Makholm4
  • Thomas Prior5,6
  • Jesper Rømhild Davidsen7,8
  • Saher Burhan Shaker9
  • Elisabeth Bendstrup4,10
  • Anders Løkke1,2
  • Ole Hilberg1,2
10 affiliations
  1. 1Department of Medicine, Lillebaelt Hospital, Vejle, Denmark.
  2. 2Department of Regional Health Research, University of Southern Denmark, Odense, Denmark.
  3. 3Medical Department, Gødstrup Hospital, Herning, Denmark.
  4. 4Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark.
  5. 5Department of Respiratory Diseases, Aalborg University Hospital, Aalborg, Denmark.
  6. 6Department of Clinical Medicine, Aalborg University, Aalborg, Denmark.
  7. 7South Danish Centre for Interstitial Lung Diseases (SCILS), Department of Respiratory Medicine, Odense University Hospital, Odense, Denmark.
  8. 8Odense Respiratory Research Unit (ODIN), Department of Clinical Research, University of Southern Denmark, Odense, Denmark.
  9. 9Department of Respiratory Medicine, University of Copenhagen, Herlev-Gentofte Hospital, Hellerup, Denmark.
  10. 10Department of Clinical Medicine, Aarhus University, Aarhus, Denmark.
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Abstract

Background

Progressive pulmonary fibrosis (PPF) represents a common disease behaviour across fibrotic interstitial lung diseases (F-ILDs). PPF was defined in 2022, but data on extent and prognosis remain limited. The aim of this study was to determine the proportion of those with F-ILD who progress to PPF, investigate predictors of PPF and compare survival between PPF and non-PPF patients.Methods

This prospective longitudinal cohort study included all incident cases of F-ILD diagnosed at Danish tertiary interstitial lung disease (ILD) centres between 1 June 2016 and 31 May 2017. Patients with idiopathic pulmonary fibrosis or fewer than two pulmonary function tests were excluded. Data were obtained from the Danish ILD database (DANILDA) and national registries. PPF was defined according to the 2022 criteria. Survival was analysed using Kaplan-Meier estimates and Cox regression with PPF modelled as a time-dependent covariate and delayed entry handled by left truncation.Results

Of the 197 patients included, 57% developed PPF during 4 years of follow-up (median 13 months). No baseline variables predicted PPF development. The PPF group experienced a greater annual decline in forced vital capacity (FVC) (adjusted mean difference -140 mL·year⁻¹; p=0.001) and diffusing capacity of the lung for carbon monoxide (-2.97 percentage points per year; p=0.009) than the non-PPF group. The 3-, 5- and 7-year survival rates were 73%, 58% and 48%, respectively, among those with PPF, and did not differ significantly between people with PPF and those with non-PPF. A higher baseline ILD-gender-age-physiology index (ILD-GAP) stage was independently associated with decreased survival (hazard ratio 2.64; p<0.001).Conclusion

In this nationwide real-world cohort, almost six out of 10 patients with F-ILD developed PPF within 4 years. Although patients with PPF exhibited a greater annual decline in FVC, survival rates did not differ between people with PPF and those with non-PPF.

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Cite

BibTeXRIS
BibTeX
@article{kolneraugustson2026progressive,
  title = {{Progressive pulmonary fibrosis in fibrotic interstitial lung disease: a Danish cohort study}},
  author = {Line Kølner-Augustson and Malene Knarborg and Nanna Makholm and Thomas Prior and Jesper Rømhild Davidsen and Saher Burhan Shaker and Elisabeth Bendstrup and Anders Løkke and Ole Hilberg},
  journal = {ERJ open research},
  year = {2026},
  month = nov,
  doi = {10.1183/23120541.00476-2026},
  url = {https://europepmc.org/article/PMC/PMC13725832},
  note = {CC BY-NC},
}
RIS
TY  - JOUR
TI  - Progressive pulmonary fibrosis in fibrotic interstitial lung disease: a Danish cohort study
AU  - Line Kølner-Augustson
AU  - Malene Knarborg
AU  - Nanna Makholm
AU  - Thomas Prior
AU  - Jesper Rømhild Davidsen
AU  - Saher Burhan Shaker
AU  - Elisabeth Bendstrup
AU  - Anders Løkke
AU  - Ole Hilberg
PY  - 2026
DA  - 2026/11/01
JO  - ERJ open research
DO  - 10.1183/23120541.00476-2026
UR  - https://europepmc.org/article/PMC/PMC13725832
AB  - Background  Progressive pulmonary fibrosis (PPF) represents a common disease behaviour across fibrotic interstitial lung diseases (F-ILDs). PPF was defined in 2022, but data on extent and prognosis remain limited. The aim of this study was to determine the proportion of those with F-ILD who progress to PPF, investigate predictors of PPF and compare survival between PPF and non-PPF patients.Methods  This prospective longitudinal cohort study included all incident cases of F-ILD diagnosed at Danish tertiary interstitial lung disease (ILD) centres between 1 June 2016 and 31 May 2017. Patients with idiopathic pulmonary fibrosis or fewer than two pulmonary function tests were excluded. Data were obtained from the Danish ILD database (DANILDA) and national registries. PPF was defined according to the 2022 criteria. Survival was analysed using Kaplan-Meier estimates and Cox regression with PPF modelled as a time-dependent covariate and delayed entry handled by left truncation.Results  Of the 197 patients included, 57% developed PPF during 4 years of follow-up (median 13 months). No baseline variables predicted PPF development. The PPF group experienced a greater annual decline in forced vital capacity (FVC) (adjusted mean difference -140 mL·year⁻¹; p=0.001) and diffusing capacity of the lung for carbon monoxide (-2.97 percentage points per year; p=0.009) than the non-PPF group. The 3-, 5- and 7-year survival rates were 73%, 58% and 48%, respectively, among those with PPF, and did not differ significantly between people with PPF and those with non-PPF. A higher baseline ILD-gender-age-physiology index (ILD-GAP) stage was independently associated with decreased survival (hazard ratio 2.64; p<0.001).Conclusion  In this nationwide real-world cohort, almost six out of 10 patients with F-ILD developed PPF within 4 years. Although patients with PPF exhibited a greater annual decline in FVC, survival rates did not differ between people with PPF and those with non-PPF.
SN  - 2312-0541
ER  -