Bronchiectasis

Abstract

Bronchiectasis refers to abnormal dilatation of the bronchi. Airway dilatation can lead to failure of mucus clearance and increased risk of infection. Pathophysiological mechanisms of bronchiectasis include persistent bacterial infections, dysregulated immune responses, impaired mucociliary clearance and airway obstruction. These mechanisms can interact and self-perpetuate, leading over time to impaired lung function. Patients commonly present with productive cough and recurrent chest infections, and the diagnosis of bronchiectasis is based on clinical symptoms and radiological findings. Bronchiectasis can be the result of several different underlying disorders, and identifying the aetiology is crucial to guide management. Treatment is directed at reducing the frequency of exacerbations, improving quality of life and preventing disease progression. Although no therapy is licensed for bronchiectasis by regulatory agencies, evidence supports the effectiveness of airway clearance techniques, antibiotics and mucolytic agents, such as inhaled isotonic or hypertonic saline, in some patients. Bronchiectasis is a disabling disease with an increasing prevalence and can affect individuals of any age. A major challenge is the application of emerging phenotyping and endotyping techniques to identify the patient populations who would most benefit from a specific treatment, with the goal of better targeting existing and emerging treatments and achieving better outcomes.

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Fig. 1: Morphology of healthy and dilatated bronchi.
Fig. 2: Aetiologies of bronchiectasis grouped by regions and populations.
Fig. 3: Microbial infection and dysfunctional immunity contribute to the pathophysiology of bronchiectasis.
Fig. 4: Airway obstruction, impaired mucociliary clearance and other mechanisms promoting bronchiectasis.
Fig. 5: Radiological features of bronchiectasis.
Fig. 6: Resolution of bronchiectasis in a child.

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Acknowledgements

J.D.C. is supported by the GSK/British Lung Foundation Chair of Respiratory Research. A.B.C. is supported by an Australian National Health and Medical Research Council Practitioner Fellowship (grant 105821). S.H.C. is supported by the Singapore Ministry of Health’s National Medical Research Council under its Transition Award (NMRC/TA/0048/2016), the Lee Kong Chian School of Medicine, Nanyang Technological University Start-Up Grant and would like to acknowledge the Academic Respiratory Initiative for Pulmonary Health (TARIPH).

Reviewer information

Nature Reviews Disease Primers thank L.-A. Daniel, K. Olivier, E. Polverino, H. Tiddens and the other anonymous reviewer(s) for their contribution to the peer review of this work.

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Introduction (J.D.C.); Epidemiology (P.J.M.); Mechanisms/pathophysiology (S.H.C.); Diagnosis, screening and prevention (A.B.C.); Management (R.D.); Quality of life (P.J.M.); Outlook (J.D.C.); Overview of Primer (J.D.C.).

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Correspondence to James D. Chalmers.

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Competing interests

J.D.C. has been an investigator, advisory board member or trial steering committee member for several bronchiectasis clinical trials, including for Aradigm, Bayer, Grifols, Novartis and Zambon. He is chair of the European Bronchiectasis Registry. A.B.C. is a member of the data safety monitoring board for an unlicensed vaccine study (for GlaxoSmithKline) and an adviser for study design of an unlicensed product for cough (for Merck). She declares no financial conflicts of interest regarding the content of this manuscript. P.J.M. has served on the advisory committee to the FDA for Bayer and is an advisory board member for Aradigm, Bayer, Grifols, Hill Rom and Insmed. She declares no financial conflicts of interest regarding the content of this manuscript. S.H.C. and R.D. declare no competing interests.

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European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC): www.bronchiectasis.eu

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Chalmers, J.D., Chang, A.B., Chotirmall, S.H. et al. Bronchiectasis. Nat Rev Dis Primers 4, 45 (2018). https://doi.org/10.1038/s41572-018-0042-3

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