European Respiratory Society
Bronchiectasis

Bronchiectasis is a hot topic in respiratory medicine, attracting an increasing amount of interest from clinicians, scientists, physiotherapists and the pharmaceutical industry. However, there is a lack of knowledge about the disease in terms of the research performed, clinical management, classification and patient treatment. The disease is also very complex because it can be caused by multiple underlying disorders, meaning its clinical presentation is highly diverse. This Monograph will tackle these issues by providing a series of chapters from recognised world experts covering: clinical management, service delivery, pathophysiology, microbiology and underlying disorders. The book also addresses the challenges faced in clinical trials and the need for drug development, and presents a number of clinical cases designed to aid learning. The Bronchiectasis Monograph substantially integrates the 2017 ERS guidelines on management of these patients. It is an essential reference for anyone caring for bronchiectasis patients or engaged in bronchiectasis research.

  • ERS Monograph
  1. Page ix
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  5. Page 1
    Abstract
    Marta Almagro Manero, C/Puigcerda 265, 08020 Barcelona, Spain. E-mail: marta.almagro@gmail.com

    Great advances continue to be made in our knowledge and awareness of bronchiectasis. Once considered an orphan and neglected disease, in recent years the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) network was established, the first European Respiratory Society guidelines for the management of adult bronchiectasis were published in September 2017, and the European Bronchiectasis Patients Advisory Group was set up by the European Lung Foundation (ELF). These are exciting developments. However, there is still a lot to do and the intention of this chapter is to give some focus to where this work should be directed as seen from a patient's perspective, because after all it is the patients who are the recipients of all these efforts.

    Cite as: Almagro Manero M, Boyd J. A patient's perspective. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 1–7 [https://doi.org/10.1183/2312508X.10015117].

  6. Page 8
    Abstract
    James D. Chalmers, Scottish Centre for Respiratory Research, University of Dundee, Ninewells Hospital and Medical School, Dundee, DD1 9SY, UK. E-mail: jchalmers@dundee.ac.uk

    Bronchiectasis pathophysiology is heterogeneous, complex and poorly understood. Cole's vicious cycle hypothesis has been central to our understanding of the development and progression of bronchiectasis since the 1980s. This concept allowed us to focus on four different components: inflammation, airway structural damage, impaired mucociliary clearance and infection. Infection is the dominant stimulus for neutrophil recruitment to the airway and is considered to be a major driver of disease progression in bronchiectasis. Although bacterial pathogens are the most clinically important, mycobacteria, viruses and fungi are also identified in stable bronchiectasis, and contribute to disease phenotype and progression. Exacerbations in bronchiectasis are likely to be heterogeneous events with multiple potential causative factors. Dysfunction of cilia and an increase of mucus leaves the respiratory epithelium vulnerable to infection. Ciliary dysfunction can be primary or acquired through inflammation and infection. New technologies including whole-genome sequencing, metagenomics and big data are increasing our ability to cut through the heterogeneity of disease. Significant progress has been made in our understanding of the pathophysiology of bronchiectasis; however, there are many unanswered questions necessitating a renewed effort by the scientific community.

    Cite as: Boaventura R, Shoemark A, Chalmers JD. Pathophysiology. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 8–28 [https://doi.org/10.1183/2312508X.10021218].

  7. Page 29
    Abstract
    Carlo Castellani, Cystic Fibrosis Centre, Gaslini Institute, Via Gerolamo Gaslini, 5, 16100 Genoa, Italy. E-mail: carlocastellani@yahoo.com

    Bronchiectasis is a very common manifestation of CF that usually starts in the upper lobes and tends over time to progress to other pulmonary areas, with progression related to age and severity. CF was once almost exclusively a paediatric disease, but the continuous advances in care have paralleled an impressive improvement in survival, and in several countries most patients are now adults and occasionally elderly. Although most cases are diagnosed in newborns and children, CF can be identified, and is possibly underdiagnosed, in adults. The CF diagnostic option should always be considered in adults with bronchiectasis when other more common causes have been excluded. The sweat test is the gold standard for diagnosis, but genetic analysis, specific electrophysiological tests, and an accurate investigation of pulmonary and extrapulmonary clinical manifestations need to be considered. Uncertain diagnoses should be referred to specialised CF centres.

    Cite as: Castellani C, Simmonds NJ. Identifying undiagnosed cystic fibrosis in adults with bronchiectasis. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 29–44 [https://doi.org/10.1183/2312508X.10015317].

  8. Page 45
    Abstract
    Melissa J. McDonnell, Dept of Respiratory Medicine, Galway University Hospitals, Newcastle Road, Galway, Ireland. E-mail: melissajanefriel@gmail.com

    Comorbidities are common and significant in bronchiectasis, often contributing to symptoms, exacerbations, hospital admissions and mortality. In this chapter, we provide a state-of-the-art summary of key comorbidities observed in bronchiectasis patients in terms of their prevalence, impact, pathophysiology and prognosis. We discuss clinical tools used to quantify comorbidity and predict prognosis in bronchiectasis and outline the implications of multimorbidity in the clinical management of bronchiectasis patients to maximise outcome and reduce the illness burden associated with the disease.

    Cite as: McDonnell MJ, Ward C, Rutherford RM. Comorbidities and their impact. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 45–61 [https://doi.org/10.1183/2312508X.10015417].

  9. Page 62
    Abstract
    Pieter C. Goeminne, Dept of Respiratory Disease, AZ Nikolaas, Moerlandstraat 1, 9100 Sint-Niklaas, Belgium. E-mail: pieter.goeminne@aznikolaas.be

    Severity assessment has proven useful in many respiratory diseases and is expected to become increasingly important in the management of bronchiectasis patients. Clinical severity scores allow clinicians and researchers to identify low- and high-risk patients, make evidence-based treatment decisions and select the right population for clinical trials. In this chapter, we discuss the importance of severity assessment. A wide variety of patient-related factors are known to have an impact on disease severity in bronchiectasis, and an overview of these factors is provided. Currently, different clinical prediction tools are available for classification of disease severity in bronchiectasis: the BSI, the FACED score (and its derivative, the E-FACED score including exacerbations) and the BACI. Finally, we analyse the differences among the scoring systems, and highlight their advantages and disadvantages.

    Cite as: Altenburg J, Goeminne PC. Defining severe bronchiectasis. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 62–81 [https://doi.org/10.1183/2312508X.10015517].

  10. Page 82
    Abstract
    Geraint B. Rogers, South Australian Health and Medical Research Institute, North Terrace, Adelaide, 5001, South Australia, Australia. E-mail: geraint.rogers@sahmri.com

    Non-CF bronchiectasis is characterised by dysregulated immunity and impaired airway clearance. Mucus accumulation results in an increased susceptibility to persistent lung infections by pathogens such as Haemophilus influenzae, Pseudomonas aeruginosa and Aspergillus fumigatus. The presence of these microbes within the lower airways contributes to a “vicious circle” of impaired mucociliary function, bronchial inflammation and progressive lung injury. The importance of pathogen detection as a guide to antimicrobial therapy has led to the use of narrowly focused diagnostic practices. However, extended culture techniques and DNA sequencing technologies have revealed a more diverse airway microbiota, including an abundance of species that are refractory to common diagnostic protocols. Interactions that occur between these microbial species can profoundly affect the expression of pathogenicity and virulence by important airway pathogens, such as P. aeruginosa. This chapter sets out current hypotheses regarding the contribution of the respiratory microbiome to non-CF bronchiectasis, and discusses how the insight gained using emerging analytical technologies can inform clinical care.

    Cite as: Rogers GB. The bronchiectasis microbiome. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 82–98 [https://doi.org/10.1183/2312508X.10015617].

  11. Page 99
    Abstract
    William R. Good, Dept of Medicine, Faculty of Medical and Health Sciences, The University of Auckland, Private Bag 92019, Auckland, 1142, New Zealand. E-mail: William.Good@middlemore.co.nz

    Clinical end-points are a crucial part of any clinical trial. In bronchiectasis, debate has arisen over the optimal end-points to use because of variable results from recent intervention trials. We discuss the challenges faced in choosing end-points and review the end-points that have been used in bronchiectasis clinical trials to date. These clinical end-points and potential biomarkers include exacerbations, health-related quality of life and symptom-burden assessment, exercise capacity and lung function, radiological changes, sputum and systemic inflammatory markers, and microbiological assessment (including microbiome analysis). Limited evidence is available about when an end-point is preferable, and each end-point provides a unique insight into bronchiectasis morbidity. It is hoped that as we begin to understand bronchiectasis in greater detail, these clinical end-points can be tailored to specific groups and interventions in order to provide meaningful outcomes for patients, and ultimately more evidence-based treatment options.

    Cite as: Good WR, Jayaram L, Vandal AC, et al. How do we know what works? Clinical trial end-points and quality of life assessment. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 99–132 [https://doi.org/10.1183/2312508X.10015717].

  12. Page 133
    Abstract
    James D. Chalmers, Scottish Centre for Respiratory Research, University of Dundee, Ninewells Hospital and Medical School, Dundee, DD1 9SY. E-mail: jchalmers@dundee.ac.uk

    Phenotypes refer to observable characteristics in patients that link to meaningful clinical outcomes while endotypes refer to distinct biological mechanisms that may link to clinical phenotype, clinical outcomes or treatment response. Phenotyping and endotyping could be extremely useful in cutting through the heterogeneity of bronchiectasis and leading to personalised treatment. These approaches are at an early stage but this chapter reviews the progress made to date and the potential to implement stratified approaches into clinical practice in future.

    Cite as: Chalmers JD. Phenotypes and endotypes. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 133–152 [https://doi.org/10.1183/2312508X.10017118].

  13. Page 153
    Abstract
    John R Hurst, UCL Respiratory, Royal Free Campus, University College London, London, UK. E-mail: j.hurst@ucl.ac.uk

    Primary immunodeficiency (PID) syndromes, especially antibody deficiencies, are associated with an increased risk of bronchiectasis. It is important to detect PID-associated bronchiectasis because Ig replacement therapy is a specific intervention that reduces the risk of subsequent infections and may therefore reduce the development and progression of bronchiectasis. Ig replacement is primarily dosed to clinical response. Otherwise, the management of bronchiectasis in PID is similar to bronchiectasis of other aetiology. The main exceptions to this are the recommendation to repeat CT scans even in asymptomatic patients, given the apparent risk of bronchiectasis despite adequate Ig replacement, and that care is best shared by respiratory and immunology specialists working as part of a multiprofessional team.

    Cite as: Lowe DM, Hurst JR. Primary immunodeficiency. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 153–166 [https://doi.org/10.1183/2312508X.10015917].

  14. Page 167
    Abstract
    Pierluigi Paggiaro, Dipartimento Cardio-Toraco-Vascolare, Ospedale Universitario di Cisanello, Via Paradisa 2, 56124 Pisa, Italy. E-mail: pierluigi.paggiaro@unipi.it

    The association of COPD and asthma with bronchiectasis is well known but the prevalence of these combinations varies.

    COPD and bronchiectasis share common risk factors, clinical features and functional abnormalities. In clinical practice, it is common to identify patients with both COPD and bronchiectasis. In these patients, it can be unclear whether recurrent bronchiectasis exacerbations have led to the obstructive pattern, or whether COPD with frequent exacerbations has led to the development of bronchiectasis. The COPD/bronchiectasis overlap should be identified as a specific phenotype as it requires a different therapeutic approach.

    The association between asthma and bronchiectasis is more complex. Bronchiectasis has been detected in severe asthma in ≤40% of patients who experience frequent exacerbations; it usually has a non-eosinophilic inflammatory pattern that is poorly responsive to standard asthma treatment. These patients require a different kind of pharmacological treatment (antibiotic therapy and/or physiotherapy). In patients with bronchiectasis without a history of asthma, some “asthma-like features” may be observed.

    The definition of these “bronchietasis phenotypes” has a strong impact on prompt diagnosis and personalised therapy.

    Cite as: Dente FL, Roggi MA, Del Cesta R, Polverino E, Paggiaro P. COPD and asthma overlap with bronchiectasis. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 167–185 [https://doi.org/10.1183/2312508X.10016017].

  15. Page 186
    Abstract
    Andrew M. Jones, Manchester Adult Cystic Fibrosis Centre, Manchester University Hospitals NHS Foundation Trust, Wythenshawe Hospital, Southmoor Road, Manchester M23 9LT, UK. E-mail: andrew.jones@mft.nhs.uk

    Aspergillus fumigatus is commonly encountered in patients with bronchiectasis, and is capable of causing a range of clinical problems as a consequence of both its action as a pathogen and a host hypersensitivity response. In susceptible individuals, an allergic response to Aspergillus allergens within the airway leads to a T-helper cell type 2-dominated inflammation, ABPA, which can be a causal factor in the development of bronchiectasis in some patients and a complication of pre-existing bronchiectasis in others. Aspergillus, an infective pathogen, can manifest as chronic pulmonary aspergillosis that may complicate the clinical course of bronchiectasis. Whilst Aspergillus-related disease is encountered relatively frequently, the prevalence and role of other fungal species in non-CF bronchiectasis is, at present, unclear. Evolving knowledge of the microbiota in bronchiectasis is likely to help our understanding of the role of all fungi in the pathogenesis of bronchiectasis, as treatment options against Aspergillus and other fungi, although still relatively limited, are beginning to expand.

    Cite as: Green H, Newton P, Jones AM. Fungal lung disease. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 186–203 [https://doi.org/10.1183/2312508X.10016117].

  16. Page 204
    Abstract
    Rachel Thomson, Gallipoli Medical Research Institute, University of Queensland, Brisbane, 4120, Australia. E-mail: R.Thomson@uq.edu.au

    NTM are ubiquitous environmental organisms that have been postulated as a cause of progressive bronchiectasis in their own right and also commonly colonise and infect patients with pre-existing bronchiectasis from other causes. The interplay can be complex pathophysiologically and it is not always clear which came first in the individual patient. Clinicians need to be astutely aware of the primary disease process, how to assess the significance of NTM isolation in sputum of bronchiectasis patients, particularly when co-infection with other organisms is present, understand when and how NTM should be treated, and at the same time, deliver optimal management of the underlying bronchiectasis.

    Cite as: Baird TM, Thomson R. Diagnosis, classification and epidemiology of pulmonary nontuberculous mycobacterial disease. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 204–221 [https://doi.org/10.1183/2312508X.10014818].

  17. Page 222
    Abstract
    Michael Loebinger, Host Defence Unit, Royal Brompton Hospital, Sydney St, London, SW3 6NP, UK. E-mail: m.loebinger@rbht.nhs.uk

    The treatment of disease caused by NTM is challenging, employing multidrug regimens for at least 1 year. Adverse events are not uncommon, and as some subjects may remain stable without treatment the decision on which patients to treat and at what point to begin treatment is of great importance. The choice of treatment regimen is determined primarily by the NTM species and disease severity. Drug sensitivity testing also plays an important role, but the interpretation is not straightforward. For slow-growing mycobacteria rifamycins, macrolides and ethambutol form the mainstay of treatment. Rapidly growing mycobacteria frequently display extensive drug resistance and may require a combination of parental and oral agents. Surgery should also be considered in selected cases. Outcomes vary widely between species, with an almost universal response to treatment seen in Mycobacterium kansasii whereas in Mycobacterium abscessus a cure may often not be attainable.

    Cite as: Cowman S, Loebinger MR. Management of pulmonary nontuberculous mycobacteria disease. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 222–237 [https://doi.org/10.1183/2312508X.10016317].

  18. Page 238
    Abstract
    Kevin Winthrop, 3181 SW Sam Jackson Park Road, MC: GH104, Portland, OR 97239, USA. E-mail: winthrop@ohsu.edu

    Bacterial infection is a common cause of bronchiectasis and persistent microbial colonisation is thought to be a central component of the ongoing pathophysiology of the disease. Many bronchiectasis patients also have underlying immune-mediated inflammatory diseases that require treatment with immunosuppressive medications. Examples of these medications include nonbiologic disease-modifying antirheumatic drugs, such as methotrexate or leflunomide, and biologic disease-modifying antirheumatic drugs, such as etanercept or infliximab. These medications are known to place all patients at greater risk of infection, and theoretically can contribute to persistent bacterial colonisation and recurrent pulmonary infections in bronchiectasis patients. Ultimately, this can lead to bronchiectasis progression. With the increasing use of these medications, the safety of their use in patients with bronchiectasis remains a question. In this chapter, we discuss the infection risk of immunosuppressive medications in patients with bronchiectasis, the management of these infections (paying special focus to pulmonary NTM infections) and how to balance the need for continued immunosuppression with further infection risk.

    Cite as: Ranches GP, Winthrop KL. Nontuberculous mycobacteria infections in patients receiving immunosuppressive agents. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 238–253 [https://doi.org/10.1183/2312508X.10016417].

  19. Page 254
    Abstract
    Anthony De Soyza. E-mail: Anthony.de-soyza@ncl.ac.uk

    Bronchiectasis arises in many systemic disorders. This has important consequences for the management of the primary condition and also for the management and outcomes of the bronchiectasis. The increased risk of infections with bronchiectasis can be a barrier to aggressive treatment of systemic inflammatory diseases. This chapter discusses the associations between bronchiectasis and systemic diseases, and highlights the challenges in managing these.

    Cite as: De Soyza A. Systemic and connective tissue diseases. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 254–266 [https://doi.org/10.1183/2312508X.10016517].

  20. Page 267
    Abstract
    Amelia Shoemark, Respiratory Research Centre, University of Dundee, Dundee, UK. E-mail: ashoemark@dundee.ac.uk

    PCD is a genetically and clinically heterogeneous condition. It is characterised by inherited abnormality of motile ciliary function. Symptoms include recurrent upper and lower respiratory tract infections from an early age, usually resulting in bronchiectasis as an adult. Approximately 50% of people with PCD have situs inversus. Diagnosis of PCD is complex and requires a multi-test approach since no test has complete sensitivity or specificity. This chapter describes the central aspects of PCD diagnosis, based on evidence from the 2017 European Respiratory Society guidelines. Diagnostic investigations include measurement of nasal nitric oxide, genetic testing and sampling the nasal epithelium to conduct microscopic analysis of cilia. Laboratory microscopy investigations include ciliary beat frequency and pattern by high-speed video microscopy, ciliary protein expression by immunofluorescence, and ciliary ultrastructure by transmission electron microscopy. A diagnosis is confirmed by the presence of a hallmark defect of ciliary ultrastructure observed by electron microscopy or bi-allelic pathogenic mutations in a known PCD gene.

    Cite as: Shoemark A, Lucas JS. Diagnosis of primary ciliary dyskinesia: current practice and future perspectives. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 267–281 [https://doi.org/10.1183/2312508X.10016617].

  21. Page 282
    Abstract
    Claudia E. Kuehni, Institute of Social and Preventive Medicine, University of Bern, Mittelstrasse 43, 3012, Bern, Switzerland. E-mail: claudia.kuehni@ispm.unibe.ch

    PCD is a genetically and clinically heterogeneous inherited disease, characterised by abnormal motile ciliary function. Although early symptoms predominantly affect the airways and most patients develop bronchiectasis, PCD is a multisystem disease and management must be multidisciplinary. Particularly relevant are chronic upper and lower respiratory tract symptoms, laterality disorders, cardiac manifestations and fertility. There is no strong evidence for the effectiveness of different therapies for PCD. This chapter highlights central aspects of PCD management, describes current practice and gives an overview on ongoing efforts to improve the evidence base.

    Cite as: Kuehni CE, Goutaki M, Rubbo B, et al. Management of primary ciliary dyskinesia: current practice and future perspectives. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 282–299 [https://doi.org/10.1183/2312508X.10016717].

  22. Page 300
    Abstract
    Francesco Blasi, Dept of Pathophysiology and Transplantation, Università degli Studi di Milano, Via F. Sforza 35, 20122 Milan, Italy. E-mail: francesco.blasi@unimi.it

    Pulmonary exacerbations are key events in the natural history of bronchiectasis, and determinants of mortality and healthcare costs. Recent literature shows that “frequent exacerbator” is a valid clinical phenotype, with more frequent hospitalisations, impaired quality of life and increased 5-year mortality. As a consequence, exacerbation rate and time to first exacerbation are fundamental end-points in clinical research and targets for therapy. Society recommendations have, to some degree, addressed the clinical management of frequently exacerbating patients. After improvement of any treatable underlying causes and optimisation of airway clearance, long-term inhaled antibiotics are suggested for patients with chronic Pseudomonas aeruginosa respiratory infection. Depending on whether lack of efficacy or intolerance occurs, second-line treatment is represented by an add-on or switch to long-term macrolide therapy. On the contrary, macrolides represent first-line treatment in frequent exacerbators without chronic P. aeruginosa infection. However, most of these approaches lack strong evidence from RCTs in a large target population and multiple factors in patients with recurrent exacerbations should be considered in clinical management.

    Cite as: Gramegna A, Di Pasquale M, Blasi F. Management of frequently exacerbating patients. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 300–311 [https://doi.org/10.1183/2312508X.10015218].

  23. Page 312
    Abstract
    Respiratory Disease Dept, Vall d'Hebron Research Institute, University Hospital Vall d'Hebron, Barcelona, Spain. E-mail: eva.polverino@vhir.org

    Antibiotics are considered a pillar of treatment of bronchiectasis due to the high frequency of respiratory infections in this condition. Acute infections are usually considered bronchiectasis exacerbations requiring antibiotic treatment and pneumonia cases. Up to 60% of bronchiectasis patients suffer some kind of chronic bronchial infection, with Haemophilus influenzae and Pseudomonas aeruginosa the most commonly identified pathogens. Optimal management of infections is therefore crucial in bronchiectasis, and the risk of antimicrobial resistance has to be considered due to the potential implications for bronchiectasis patients and the general population. This chapter will review the current evidence on the use of short- and long-term antibiotic therapy and on the relevance of antimicrobial resistance risk in adult patients with bronchiectasis.

    Cite as: Polverino E, Perez-Miranda J. Antibiotic management and resistance. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 312–330 [https://doi.org/10.1183/2312508X.10016917].

  24. Page 331
    Abstract
    Beatriz Herrero-Cortina, Universidad San Jorge, Campus Universitario, Autovía A23 Km 299, 50830 Villanueva de Gállego, Zaragoza, Spain. E-mail: beafisiorespi@gmail.com

    People with bronchiectasis are characterised by a combination of an impaired mucociliary clearance system with functional limitation and lower physical activity levels; hence, physiotherapy interventions should be a priority strategy for the management of this population. ACTs used regularly reduce the respiratory symptoms related to cough and, therefore, improve health-related quality of life (HRQoL). Short-term clinical benefits in people with bronchiectasis are observed for functional exercise capacity, symptoms and HRQoL after completing a pulmonary rehabilitation programme, but these improvements are not maintained long term. Consequently, interventions to increase physical activity and reduce sedentary time may be incorporated in pulmonary rehabilitation programmes and in the overall management of people with bronchiectasis. Furthermore, strategies to promote behavioural change and adherence are needed to ensure successful implementation of these interventions in clinical practice. Further research is needed to explore the effects of physiotherapy interventions during an acute exacerbation and continued beyond discharge, and their impact on disease severity.

    Cite as: Herrero-Cortina B, Lee AL, O'Neill B, et al. Airway clearance techniques, pulmonary rehabilitation and physical activity. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 331–352 [https://doi.org/10.1183/2312508X.10017017].

  25. Page 353
    Abstract
    Stefano Aliberti, Dept of Pathophysiology and Transplantation, University of Milan, Internal Medicine Dept, Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, Italy. E-mail: stefano.aliberti@unimi.it

    Bronchiectasis is a chronic respiratory disease characterised by heterogeneity in terms of clinical and radiological presentation, aetiological diagnosis, microbiological features and disease severity. Identifying bronchiectasis patients who require a specialist/tertiary care follow-up is a major challenge in bronchiectasis. In different care settings, a multidisciplinary approach is essential in the management of bronchiectasis to optimise pharmacological and non-pharmacological treatment and to stimulate clinical research. This chapter will focus on the role of each specialist involved in the multidisciplinary team and will provide a description of patients who require a specialist/tertiary care follow-up.

    Cite as: Amati F, Gramegna A, Contarini M, et al. Site of care and multidisciplinary approach. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 353–370 [https://doi.org/10.1183/2312508X.10017117].

  26. Page 371
    Abstract
    Sanjay H. Chotirmall, Lee Kong Chian School of Medicine, Nanyang Technological University, Clinical Sciences Building, 11 Mandalay Road, Singapore 308232. E-mail: schotirmall@ntu.edu.sg

    A palpable recognition of the growing global importance of bronchiectasis is evident. This is paralleled by increases in the quantity and quality of research in the field. To ensure we make progress, continue on an upward trajectory and draft a realistic roadmap for bronchiectasis research over the next decade, it is imperative to learn from past works and fully understand the current research state. Most research performed to date is clinical and a need exists to go back to basics and establish strong platforms for basic science and translational studies focused on understanding pathophysiological mechanisms that drive disease onset and progression. Such approaches will allow attempts at resolution of the unresolved heterogeneity that persists in disease which has negatively influenced many clinical trial outcomes. Elucidating specific disease endo-phenotypes, clarifying ethno-geographic variation and building upon the strong clinical foundations already set for this devastating disease should all be key priorities over the coming decade of bronchiectasis research.

    Cite as: Chotirmall SH. Future directions: the next 10 years in research. In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). Sheffield, European Respiratory Society, 2018; pp. 371–387 [https://doi.org/10.1183/2312508X.10017217].

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