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Background <p>Bronchiectasis is a common feature in idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis-associated interstitial lung disease (RA-ILD). While these so-called traction bronchiectasis are often considered a secondary phenomenon in fibrosing ILD, their prognostic significance and relationship to respiratory pathogen detection and outcomes remain unclear.</p> Methods <p>We conducted a retrospective, single-center cohort study in IPF or fibrosing RA-ILD patients with available high-resolution computed tomography (HRCT) and lower-respiratory tract microbial samples between 2014 and 2024. Bronchiectasis was assessed using the bronchiectasis subscore of the Brody score; fibrosis was quantified by deep-learning–based automated HRCT analysis. Primary outcome was 5-year transplant-free survival; secondary outcomes included isolation of pathogens per CDC criteria, PFT trajectories, bronchiectasis-associated symptoms, and hospitalization. Statistical methods included Cox regression, linear mixed-effects modeling and correlation analysis.</p> Results <p>267 IPF and 56 RA-ILD patients were included. Median modified Brody score was 11.5 (IQR 7–16; max possible range 0–72). Higher Brody scores strongly correlated with fibrotic extent (<i>R</i> = 0.6, <i>P</i> &lt; 0.001). Higher scores had significantly lower baseline FVC and DLCO (<i>P</i> &lt; 0.001), but no differences in PFT trajectories over time. In multivariable Cox regression, higher bronchiectasis scores were independently associated with mortality (HR 1.03 per point [95%CI 1.01–1.06], <i>P</i> = 0.003); fibrosis extent showed similar results (HR 1.02, CI 1.00–1.03, <i>P</i> = 0.017). Pathogens were found at a median of 3 months after baseline in 50.9% (IPF) and 46.4% (RA-ILD), without association with survival, symptoms or Brody scores. <i>Staphylococcus aureus</i> was most common (28.9%); <i>Pseudomonas aeruginosa</i> was rare (1.9%).</p> Conclusion <p>In both IPF and RA-ILD, higher bronchiectasis scores were associated with fibrosis extent and mortality, but not classical clinical bronchiectasis features. This supports traction bronchiectasis as a marker of fibrotic remodeling rather than a distinct syndrome.</p> Trial registration <p>Not applicable.</p>

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Clinical implications of traction bronchiectasis in IPF and fibrotic RA-ILD – a retrospective single-center cohort study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月13日

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  • Jakob Raith,
  • Jannik Ruwisch,
  • Jonas C Schupp,
  • Theresa Graalmann,
  • Nora Drick,
  • Marius M Hoeper,
  • Antje Prasse,
  • Jan Fuge,
  • Felix C Ringshausen,
  • Leonard Knegendorf,
  • Jessica Rademacher,
  • Sabine Dettmer,
  • Benjamin Seeliger

摘要

Background

Bronchiectasis is a common feature in idiopathic pulmonary fibrosis (IPF) and rheumatoid arthritis-associated interstitial lung disease (RA-ILD). While these so-called traction bronchiectasis are often considered a secondary phenomenon in fibrosing ILD, their prognostic significance and relationship to respiratory pathogen detection and outcomes remain unclear.

Methods

We conducted a retrospective, single-center cohort study in IPF or fibrosing RA-ILD patients with available high-resolution computed tomography (HRCT) and lower-respiratory tract microbial samples between 2014 and 2024. Bronchiectasis was assessed using the bronchiectasis subscore of the Brody score; fibrosis was quantified by deep-learning–based automated HRCT analysis. Primary outcome was 5-year transplant-free survival; secondary outcomes included isolation of pathogens per CDC criteria, PFT trajectories, bronchiectasis-associated symptoms, and hospitalization. Statistical methods included Cox regression, linear mixed-effects modeling and correlation analysis.

Results

267 IPF and 56 RA-ILD patients were included. Median modified Brody score was 11.5 (IQR 7–16; max possible range 0–72). Higher Brody scores strongly correlated with fibrotic extent (R = 0.6, P < 0.001). Higher scores had significantly lower baseline FVC and DLCO (P < 0.001), but no differences in PFT trajectories over time. In multivariable Cox regression, higher bronchiectasis scores were independently associated with mortality (HR 1.03 per point [95%CI 1.01–1.06], P = 0.003); fibrosis extent showed similar results (HR 1.02, CI 1.00–1.03, P = 0.017). Pathogens were found at a median of 3 months after baseline in 50.9% (IPF) and 46.4% (RA-ILD), without association with survival, symptoms or Brody scores. Staphylococcus aureus was most common (28.9%); Pseudomonas aeruginosa was rare (1.9%).

Conclusion

In both IPF and RA-ILD, higher bronchiectasis scores were associated with fibrosis extent and mortality, but not classical clinical bronchiectasis features. This supports traction bronchiectasis as a marker of fibrotic remodeling rather than a distinct syndrome.

Trial registration

Not applicable.