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Bronchiectasis

Pathophysiology description (concise knowledge-base narrative)

2025-12-15
Falcon MONDO:0004822 Model: Edison Scientific Literature 35 citations

Pathophysiology description (concise knowledge-base narrative) Non‑CF bronchiectasis arises from a self-sustaining “vicious vortex” of mucociliary dysfunction, persistent infection with Proteobacteria-dominant dysbiosis (notably Pseudomonas aeruginosa), and chronic neutrophil-driven inflammation that releases proteases and NETs, injuring the epithelium and altering mucus rheology, thereby worsening clearance and perpetuating infection. Inflammasome activation with airway IL‑1β links to higher severity, mucus hyperconcentration, epithelial barrier dysfunction, and Proteobacteria enrichment. Excess NSP activity (NE, PR3, CatG) over antiproteases is a causative axis, validated by clinical benefit of DPP‑1 inhibition that reduces airway protease activity and exacerbations. Clinically, frequent exacerbators and those with chronic P. aeruginosa have worse outcomes; airway clearance use remains suboptimal despite guideline recommendations (keir2021pathophysiologyofbronchiectasis pages 2-2, perea2024pathophysiologyandgenomics pages 2-3, aogain2024infectionandthe pages 1-2, johnson2024biomarkersinbronchiectasis pages 1-2, johnson2024biomarkersinbronchiectasis pages 3-4, aogain2024infectionandthe pages 6-7, chen2024theclinicalimpacts pages 1-2).

References

  1. (keir2021pathophysiologyofbronchiectasis pages 2-2): Holly R. Keir and James D. Chalmers. Pathophysiology of bronchiectasis. Seminars in Respiratory and Critical Care Medicine, 42:499-512, Jul 2021. URL: https://doi.org/10.1055/s-0041-1730891, doi:10.1055/s-0041-1730891. This article has 81 citations and is from a peer-reviewed journal.

  2. (keir2021pathophysiologyofbronchiectasis pages 1-2): Holly R. Keir and James D. Chalmers. Pathophysiology of bronchiectasis. Seminars in Respiratory and Critical Care Medicine, 42:499-512, Jul 2021. URL: https://doi.org/10.1055/s-0041-1730891, doi:10.1055/s-0041-1730891. This article has 81 citations and is from a peer-reviewed journal.

  3. (chalmers2025neutrophilicinflammationin pages 1-2): James D. Chalmers, Mark Metersky, Stefano Aliberti, Lucy Morgan, Sebastian Fucile, Melanie Lauterio, and Patrick P. McDonald. Neutrophilic inflammation in bronchiectasis. European Respiratory Review, 34:240179, Apr 2025. URL: https://doi.org/10.1183/16000617.0179-2024, doi:10.1183/16000617.0179-2024. This article has 20 citations and is from a peer-reviewed journal.

  4. (perea2024pathophysiologyandgenomics pages 2-3): Lidia Perea, Rosa Faner, James D. Chalmers, and Oriol Sibila. Pathophysiology and genomics of bronchiectasis. European Respiratory Review, 33:240055, Jul 2024. URL: https://doi.org/10.1183/16000617.0055-2024, doi:10.1183/16000617.0055-2024. This article has 29 citations and is from a peer-reviewed journal.

  5. (keir2021pathophysiologyofbronchiectasis pages 3-4): Holly R. Keir and James D. Chalmers. Pathophysiology of bronchiectasis. Seminars in Respiratory and Critical Care Medicine, 42:499-512, Jul 2021. URL: https://doi.org/10.1055/s-0041-1730891, doi:10.1055/s-0041-1730891. This article has 81 citations and is from a peer-reviewed journal.

  6. (keir2021pathophysiologyofbronchiectasis pages 2-3): Holly R. Keir and James D. Chalmers. Pathophysiology of bronchiectasis. Seminars in Respiratory and Critical Care Medicine, 42:499-512, Jul 2021. URL: https://doi.org/10.1055/s-0041-1730891, doi:10.1055/s-0041-1730891. This article has 81 citations and is from a peer-reviewed journal.

  7. (aogain2024infectionandthe pages 1-2): Micheál Mac Aogáin, Alison J. Dicker, Pontus Mertsch, and Sanjay H. Chotirmall. Infection and the microbiome in bronchiectasis. European Respiratory Review, 33:240038, Jul 2024. URL: https://doi.org/10.1183/16000617.0038-2024, doi:10.1183/16000617.0038-2024. This article has 24 citations and is from a peer-reviewed journal.

  8. (aogain2024infectionandthe pages 4-5): Micheál Mac Aogáin, Alison J. Dicker, Pontus Mertsch, and Sanjay H. Chotirmall. Infection and the microbiome in bronchiectasis. European Respiratory Review, 33:240038, Jul 2024. URL: https://doi.org/10.1183/16000617.0038-2024, doi:10.1183/16000617.0038-2024. This article has 24 citations and is from a peer-reviewed journal.

  9. (aogain2024infectionandthe pages 6-7): Micheál Mac Aogáin, Alison J. Dicker, Pontus Mertsch, and Sanjay H. Chotirmall. Infection and the microbiome in bronchiectasis. European Respiratory Review, 33:240038, Jul 2024. URL: https://doi.org/10.1183/16000617.0038-2024, doi:10.1183/16000617.0038-2024. This article has 24 citations and is from a peer-reviewed journal.

  10. (chen2024theclinicalimpacts pages 1-2): Yen-Fu Chen, Hsin-Han Hou, Ning Chien, Kai-Zen Lu, Chieh-Hua Lin, Yu-Chieh Liao, Kuo-Lung Lor, Jung-Yien Chien, Chung-Ming Chen, Chung-Yu Chen, Shih-Lung Cheng, Hao-Chien Wang, Po-Ren Hsueh, and Chong-Jen Yu. The clinical impacts of lung microbiome in bronchiectasis with fixed airflow obstruction: a prospective cohort study. Respiratory Research, Aug 2024. URL: https://doi.org/10.1186/s12931-024-02931-x, doi:10.1186/s12931-024-02931-x. This article has 6 citations and is from a domain leading peer-reviewed journal.

  11. (johnson2024biomarkersinbronchiectasis pages 3-4): Emma D Johnson, Merete B. Long, and James D. Chalmers. Biomarkers in bronchiectasis. European Respiratory Review, 33:230234, Jul 2024. URL: https://doi.org/10.1183/16000617.0234-2023, doi:10.1183/16000617.0234-2023. This article has 17 citations and is from a peer-reviewed journal.

  12. (chalmers2025neutrophilicinflammationin pages 9-10): James D. Chalmers, Mark Metersky, Stefano Aliberti, Lucy Morgan, Sebastian Fucile, Melanie Lauterio, and Patrick P. McDonald. Neutrophilic inflammation in bronchiectasis. European Respiratory Review, 34:240179, Apr 2025. URL: https://doi.org/10.1183/16000617.0179-2024, doi:10.1183/16000617.0179-2024. This article has 20 citations and is from a peer-reviewed journal.

  13. (johnson2024biomarkersinbronchiectasis pages 1-2): Emma D Johnson, Merete B. Long, and James D. Chalmers. Biomarkers in bronchiectasis. European Respiratory Review, 33:230234, Jul 2024. URL: https://doi.org/10.1183/16000617.0234-2023, doi:10.1183/16000617.0234-2023. This article has 17 citations and is from a peer-reviewed journal.