Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study

Abstract Background Immunological bronchoalveolar lavage (iBAL) is a frequently employed diagnostic tool in interstitial lung disease (ILD). The association between iBAL cellular composition and disease progression remains elusive. We evaluated whether the alveolar cellular composition at initial di...

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Main Authors: Iris A. Simons, Bart G. Boerrigter, Maud C.M. Hovestadt, Kirsten A. Mooij-Kalverda, Shiqi Zhang, Leonoor S. Boers, Anke H. Maitland– van der Zee, Esther J. Nossent, Jan Willem Duitman
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Language:English
Published: BMC 2025-04-01
Series:Respiratory Research
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Online Access:https://doi.org/10.1186/s12931-025-03236-3
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author Iris A. Simons
Bart G. Boerrigter
Maud C.M. Hovestadt
Kirsten A. Mooij-Kalverda
Shiqi Zhang
Leonoor S. Boers
Anke H. Maitland– van der Zee
Esther J. Nossent
Jan Willem Duitman
author_facet Iris A. Simons
Bart G. Boerrigter
Maud C.M. Hovestadt
Kirsten A. Mooij-Kalverda
Shiqi Zhang
Leonoor S. Boers
Anke H. Maitland– van der Zee
Esther J. Nossent
Jan Willem Duitman
author_sort Iris A. Simons
collection DOAJ
description Abstract Background Immunological bronchoalveolar lavage (iBAL) is a frequently employed diagnostic tool in interstitial lung disease (ILD). The association between iBAL cellular composition and disease progression remains elusive. We evaluated whether the alveolar cellular composition at initial diagnosis is predictive of the development of progressive pulmonary fibrosis (PPF) in patients with ILD. Methods A retrospective analysis of 111 patients diagnosed with ILD who underwent iBAL for diagnostic purposes between January 2018 and January 2023 was conducted. The identification of PPF was based on the criteria outlined in the ATS/ERS/JRS/ALAT clinical practice guidelines. Clinical data, pulmonary function tests, radiological imaging, and BAL cellular composition were collected. Groups were compared using the non-parametric Wilcoxon rank sum test. Linear mixed-effect modelling was used to assess the association between baseline cell composition and longitudinal lung function decline. Results A total of 33.3% of patients exhibited a PPF phenotype. A significant association between baseline iBAL CD4 + and CD8 + T cell percentages and forced vital capacity (FVC) decline within the first year was observed. Other cell types were not associated with ILD progression within one-year of follow-up. Conclusions CD4 + and CD8 + T cell percentages significantly correlated with FVC changes in patients with fibrotic ILD. No further associations were found between the baseline iBAL cellular profiles and disease progression. These findings suggest that baseline iBAL cellular profiles may hold some promise in predicting fibrotic ILD disease progression. Further (prospective) studies using larger cohorts may be needed to elucidate the association between the cellular composition of iBAL fluid and pulmonary fibrosis progression.
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spelling doaj-art-1ea478b7e0ee43b8ad9b3b7ff126a3672025-08-20T01:47:29ZengBMCRespiratory Research1465-993X2025-04-0126111110.1186/s12931-025-03236-3Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort studyIris A. Simons0Bart G. Boerrigter1Maud C.M. Hovestadt2Kirsten A. Mooij-Kalverda3Shiqi Zhang4Leonoor S. Boers5Anke H. Maitland– van der Zee6Esther J. Nossent7Jan Willem Duitman8Pulmonary Medicine, Amsterdam UMC location University of AmsterdamPulmonary Medicine, Amsterdam UMC location Free UniversityPulmonary Medicine, Amsterdam UMC location University of AmsterdamPulmonary Medicine, Amsterdam UMC location University of AmsterdamInfection & Immunity, Inflammatory Diseases, Amsterdam UMC location University of AmsterdamInfection & Immunity, Inflammatory Diseases, Amsterdam UMC location University of AmsterdamPulmonary Medicine, Amsterdam UMC location University of AmsterdamPulmonary Medicine, Amsterdam UMC location Free UniversityPulmonary Medicine, Amsterdam UMC location University of AmsterdamAbstract Background Immunological bronchoalveolar lavage (iBAL) is a frequently employed diagnostic tool in interstitial lung disease (ILD). The association between iBAL cellular composition and disease progression remains elusive. We evaluated whether the alveolar cellular composition at initial diagnosis is predictive of the development of progressive pulmonary fibrosis (PPF) in patients with ILD. Methods A retrospective analysis of 111 patients diagnosed with ILD who underwent iBAL for diagnostic purposes between January 2018 and January 2023 was conducted. The identification of PPF was based on the criteria outlined in the ATS/ERS/JRS/ALAT clinical practice guidelines. Clinical data, pulmonary function tests, radiological imaging, and BAL cellular composition were collected. Groups were compared using the non-parametric Wilcoxon rank sum test. Linear mixed-effect modelling was used to assess the association between baseline cell composition and longitudinal lung function decline. Results A total of 33.3% of patients exhibited a PPF phenotype. A significant association between baseline iBAL CD4 + and CD8 + T cell percentages and forced vital capacity (FVC) decline within the first year was observed. Other cell types were not associated with ILD progression within one-year of follow-up. Conclusions CD4 + and CD8 + T cell percentages significantly correlated with FVC changes in patients with fibrotic ILD. No further associations were found between the baseline iBAL cellular profiles and disease progression. These findings suggest that baseline iBAL cellular profiles may hold some promise in predicting fibrotic ILD disease progression. Further (prospective) studies using larger cohorts may be needed to elucidate the association between the cellular composition of iBAL fluid and pulmonary fibrosis progression.https://doi.org/10.1186/s12931-025-03236-3Bronchoalveolar lavage (BAL)Interstitial lung disease (ILD)PrognosisProgressive pulmonary fibrosis (PPF)Pulmonary fibrosis (PF)
spellingShingle Iris A. Simons
Bart G. Boerrigter
Maud C.M. Hovestadt
Kirsten A. Mooij-Kalverda
Shiqi Zhang
Leonoor S. Boers
Anke H. Maitland– van der Zee
Esther J. Nossent
Jan Willem Duitman
Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study
Respiratory Research
Bronchoalveolar lavage (BAL)
Interstitial lung disease (ILD)
Prognosis
Progressive pulmonary fibrosis (PPF)
Pulmonary fibrosis (PF)
title Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study
title_full Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study
title_fullStr Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study
title_full_unstemmed Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study
title_short Alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype: a retrospective cohort study
title_sort alveolar cell composition in interstitial lung disease and the development of a pulmonary progressive fibrosing phenotype a retrospective cohort study
topic Bronchoalveolar lavage (BAL)
Interstitial lung disease (ILD)
Prognosis
Progressive pulmonary fibrosis (PPF)
Pulmonary fibrosis (PF)
url https://doi.org/10.1186/s12931-025-03236-3
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