European Respiratory Society
Bronchiectasis (out of print)

This book has been superseded by a newer edition. This monograph offers a comprehensive and cutting edge review of non-Cystic fibrosis-associated bronchiectasis in adults from leading international experts. The chapters cover the epidemiology, aetiology and pathophysiology of this condition, define modern investigation algorithms and describe recent developments in treatment strategies. This book provides a definitive guide to the management of patients with bronchiectasis.

  • European Respiratory Society Monographs
  1. Page v
  2. Page vi
  3. Page vii
  4. Page 1
    Correspondence: D. Bilton, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK, Email

    Bronchiectasis remains a significant cause of morbidity and mortality in the developed world. The true prevalence of the condition remains elusive, in part, because of the innate difficulty in determining causation, when more than one respiratory condition exists in the same patient, but also due to the increasing rate of diagnosis by radiological means where no clinical symptoms are present. The wide ranging aetiology of bronchiectasis will be discussed in this chapter; however, some aspects will be discussed in greater detail throughout this Monograph.

    The diagnosis of bronchiectasis should be the beginning of a targeted search for causation, which may lead to directed treatment, thereby limiting the disease progression. Over the next 5 years a reduction in the number of cases labelled as idiopathic bronchiectasis should be expected, as the continual expanding knowledge of immunology and immunogenetics, with respect to large studies of patients with bronchiectasis, can be applied.

  5. Page 11
    Correspondence: B.N. Lambrecht, Dept of Pulmonary Medicine, Laboratory of Immunoregulation and Mucosal Immunology, Ghent University, De Pintelaan 185, B-9000 Ghent, Belgium, Email

    Over recent years there has been a tremendous increase in the understanding of pulmonary immunity, mostly driven by large research efforts in understanding the basis of asthma and chronic obstructive pulmonary disease. Bronchiectasis is well understood. In this article, an overview of pulmonary defence mechanisms as well as inflammatory mechanisms is given as a basis to understand the pathogenesis of bronchiectasis.

  6. Page 22
    Correspondence: M. Goddard, Dept of Pathology, Papworh Hospital NHS Foundation Trust, Papworth Everard, Cambridge, CB23 3RE, UK, Email

    The clinical presentation of bronchiectasis occurs after initial irreversible damage to the airway has occurred. The clinician then has to control symptoms and limit the progression of the disease. A clearer understanding of the pathogenesis of this disease will enable the development of better treatment strategies.

    Bronchiectasis is a multi-factorial disease process in which there are a number of key steps, although they are not always clinically identifiable. There is often an initiator or damaging event such as a viral infection which, in an individual with a predisposing risk such as a degree of immune dysfunction or an impaired mucociliary clearance system, leads to persistent and damaging bacterial infections. These infections go on to provoke an inappropriate and self-damaging inflammatory response in which neutrophil activity leads to progressive tissue damage and a relentless cycle of infection, inflammation and bronchial wall injury. Persistent infection and chronic inflammatory cell infiltration further amplify the local inflammatory milieu and may lead to systemic complications.

  7. Page 32
    Correspondence: J.S. Elborn, Centre for Infection and Immunity, School of Medicine, Dentistry and Biomedical Sciences, Queen’s University Belfast, 97 Lisburn Road, Belfast, BT9 7BL, UK, Email

    The diagnosis of bronchiectasis is made on the basis of high-resolution computed tomography (HRCT) scan findings. A diagnosis of bronchiectasis should be considered in all patients with persistent cough productive of sputum, where another clear diagnosis has not been made. This includes patients with an initial diagnosis of chronic obstructive pulmonary disease or severe asthma. Once bronchiectasis has been confirmed by HRCT scanning, patients should undergo a range of investigations to determine whether or not there is an underlying cause. This can usually be determined in approximately 50% of patients with bronchiectasis. The common conditions which should be sought are cystic fibrosis, immunodeficiency syndromes, primary ciliary dyskinesia, and autoimmune diseases, such as rheumatoid arthritis and ulcerative colitis. For many of these conditions, there is specific treatment to improve symptoms and reduce lung injury but, without an accurate diagnosis, appropriate therapy may not be instituted.

  8. Page 44
    Correspondence: N.J. Screaton, Diagnostic Imaging Dept, Papworth Hospital, Papworth Everard, CB23 3RE, UK, Email

    Imaging plays a crucial role in the diagnosis and monitoring of bronchiectasis and the management of complications. Chest radiography is useful as an initial screening tool and during acute exacerbations, but has limited sensitivity and specificity. High-resolution computed tomography (HRCT) is the reference standard for diagnosis and quantification of bronchiectasis, providing detailed morphological information. Computed tomography (CT) is also valuable in diagnosing and managing complications. Routine surveillance using HRCT has been mooted, particularly in cystic fibrosis (CF), where advances in treatment have increased life expectancy considerably, but cumulative radiation dose remains a concern.

    Pulmonary magnetic resonance imaging is an evolving technique that provides both structural and functional information. Its advantage is the lack of ionising radiation. Limitations include cost, availability and its inferior spatial resolution compared to CT. The technique requires further evaluation, but has potential benefits where serial follow-up imaging is being considered, such as in CF. Evaluation of mucociliary clearance using radionuclide scintigraphy may be of value, particularly in drug development.

  9. Page 68
    Correspondence: J.E. Foweraker, Dept of Microbiology, Papworth Hospital, Papworth Everard, Cambridge, CB23 3RE, UK, Email

    Non-cystic fibrosis (CF) bronchiectasis is a complex disorder characterised by recurrent chest infections and poorly regulated respiratory innate and adaptive immunity. These lead to a “vicious cycle” of impaired mucociliary clearance, chronic infection, bronchial inflammation and progressive lung injury. The most prevalent pathogenic bacteria are Haemophilus influenzae, Pseudomonas aeruginosa, Streptococcus pneumoniae, Staphylococcus aureus and Moraxella catarrhalis although variations in sampling techniques and detection methods have influenced their isolation rates. These organisms can inhibit mucociliary clearance, destroy respiratory epithelium and produce biofilms that promote persistent infection by blocking innate immune defences and increasing antibiotic resistance. While numerous studies have examined the role of different bacteria in CF and chronic obstructive pulmonary disease, little is known about how they contribute to the pathogenesis of non-CF bronchiectasis. There is also a paucity of data regarding the role of respiratory viruses in this condition. This chapter describes the microbiology of non-CF bronchiectasis, defines the bacterial mechanisms that may contribute to persistent infection and airway damage and discusses the potential role for respiratory viruses in this condition. Understanding the pathogenic properties of these microorganisms may allow the development of novel therapies for the management of respiratory exacerbations.

  10. Page 97
    Correspondence: C.K. van der Ent, Dept of Paediatric Pulmonology, University Medical Center Utrecht, Lundlaan 6, 3584 EA Utrecht, the Netherlands, Email

    Fungal spores are ubiquitously present in the air. Inhalation of these spores by humans causes disease in susceptible patients; most prevalent are invasive aspergillosis and allergic bronchopulmonary aspergillosis (ABPA). This chapter provides an overview of the pathogenecity, clinical appearance, diagnosis and treatment of ABPA.

    ABPA is a hypersensitivity lung disease limited to patients with asthma or cystic fibrosis (CF) with a prevalence of 1–2% and 2–15%, respectively within these groups. It is triggered by the exposure to Aspergillus fumigatus. Although it is not clear what initiates this hypersensitivity response, polymorphisms in genes that drive innate and adaptive immune mechanisms as well as loss-of-function mutations in the CF transmembrane conductance regulator (CFTR) are associated with ABPA development. The chronic inflammatory conditions in ABPA eventually result in airway remodelling and functional impairment.

    The diagnosis of ABPA is based both on clinical symptoms, laboratory testing and diagnostic imaging. Treatment consists of a two tiered approach, glucocorticoids to control immunological activity and antifungal agents to suppress fungal load.

  11. Page 115
    Correspondence: C.L. Daley, Division of Mycobacterial and Respiratory Infections, National Jewish Health, 1400 Jackson Street, Denver, CO 80206, USA, Email

    Nontuberculous mycobacteria (NTM) represent a large group of bacteria that have been isolated from environmental sources. When NTM are inhaled by a susceptible individual, infection can occur and lead to progressive lung disease. Epidemiological studies have described increases in the prevalence of NTM disease in multiple areas worldwide. Risk factors for disease include chronic lung diseases, such as bronchiectasis and chronic obstructive pulmonary disease, as well as various forms of immune deficiency. Patients typically present with either fibrocavitary or nodular bronchiectatic disease. Isolation of NTM from respiratory specimens does not always indicate disease so clinicians must evaluate clinical, radiographic and microbiologic information in order to diagnosis NTM-related lung disease. The American Thoracic Society has developed diagnostic criteria that can aid clinicians but the criteria cannot account for all clinical scenarios or for all NTM species given the large spectrum of pathogenicity encountered. Treatment usually consists of at least two antibiotics but the exact regimen will vary depending on the species and there is some variation in recommendations.

  12. Page 130
    Correspondence: P.G. Noone, CB 7020, Pulmonary Division, University of North Carolina School of Medicine, Chapel Hill NC 27599-7020, USA, Email:

    Primary ciliary dyskinesia (PCD) is a genetic disorder of cilia structure and function, chronic infections of the respiratory tract, fertility problems and disorders of organ laterality. Establishing a definitive diagnosis can be challenging, requiring a compatible phenotype and detection of ciliary functional and ultra-structural defects, along with newer screening tools such as nasal nitric oxide and genetics testing. 10 known PCD-causing mutations within two genes are now available in a clinical panel, and in the future, comprehensive genetic testing may serve to identify young infants with PCD to improve the long-term outlook for patients with the disease. Therapy includes regular pulmonary function testing and monitoring of sputum flora to allow a targeted approach to treatment. Referral to an academic centre with expertise in bronchiectasis and/or PCD is prudent to ensure access to the most recent diagnostic testing and therapies. With increased understanding of the disease it is likely that we will expand the definitions of classic and non-classic PCD, as well as its relationship to less common ciliopathies.

  13. Page 150
    Correspondence: I. Sermet-Gaudelus, Service de PneumoPédiatrie, Université Paris Descartes, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75015, Paris, France, Email

    Channelopathies are diseases caused by dysfunction of ion channel subunits. They result in impaired mucociliary clearance and may therefore lead to bronchiectasis.

    The main channelopathy associated with bronchiectasis is cystic fibrosis (CF), an autosomal recessive disease caused by mutations in the CFTR gene, which encodes the chloride CFTR channel.

    Bronchiectasis can be associated to channelopathies in following cases: 1) patients with already known typical CF; 2) patients with bronchiectasis who, on investigation, are found to have a single-organ manifestation of CF; 3) patients with only one or none mutation of CFTR with abnormal sweat test or nasal potential difference (PD) where CFTR mutations play the role of a modifier deleterious gene; and 4) patients with only one or no mutation of CFTR with normal sweat test or nasal PD, who may still have an undefined channelopathy. In these last two cases, it may be that, CFTR mutation combined with another ion transport abnormality, in a situation of transheterozygosity, creates the conditions for abnormal airway surface liquid (ASL) hydration regulation and defective mucociliary clearance.

  14. Page 163
    Correspondence: Ph. Camus, Dept of Pulmonary Disease and Intensive Care, University Medical Center Le Bocage and Medical School, Université de Bourgogne, POB 77908- F-21079, Dijon, France, Email

    The two major inflammatory bowel diseases (IBD), ulcerative colitis and Crohn’s disease (CD), can involve the respiratory system in several ways. The most typical pattern of involvement is in the form of airway inflammation and narrowing, which may involve specific areas of the tracheobronchial tree from the trachea to the bronchioles or which can be diffuse. Marked inflammation, which can be granulomatous in CD, causes, at times, marked airway obstruction. This pattern of involvement is amenable to different forms of inhaled and oral corticosteroid therapy. Drugs used to treat IBD are though to have no responsibility in causing the syndrome. This is in contrast to parenchymal lung disease in IBD. Colectomy may trigger the onset of airway involvement and will not improve or cure established airway inflammation in IBD.

  15. Page 178
    Correspondence: J.S. Brown, Centre for Respiratory Research, Dept of Medicine, Rayne Institute, Royal Free and University College Medical School, 5 University Street, London WC1E 6JF, UK, Email

    Bacterial infection of the lung is a cause of bronchiectasis and also the main clinical problem in patients with bronchiectasis. As a consequence, inherited or acquired immunodeficiencies that allow repetitive lung infection with respiratory pathogens (such as Streptococcus pneumoniae and Haemophilus influenzae) can drive the development and progression of bronchiectasis. The immune defects most strongly associated with bronchiectasis are those resulting in hypogammaglobulinaemia. These include the primary immunodeficiencies, common variable immunodeficiency and X-linked agammaglobulinaemia and the secondary immunodeficiences caused by lymphoproliferative malignancy, allogeneic bone marrow transplantation and chemo/immunotherapy. Identifying hypogammaglobulinaemia is important and allows patients to be given immunoglobulin replacement, reducing exacerbation frequency and probably progression of bronchiectasis. Conditions resulting in T-cell dysfunction (such as chronic HIV infection or immunosuppression), reduced bacterial opsonisation (such as complement deficiencies), failure of phagocyte migration (leukocyte adhesion deficiency) and impaired intracellular killing of bacteria (chronic granulomatous disease) may also predispose to bronchiectasis. In this chapter we describe the main immunodeficiencies associated with bronchiectasis and suggest a staged approach to immunological investigations.

  16. Page 192
    Correspondence: R. Wilson, Royal Brompton Hospital, Fulham Road, London, SW3 6NP, UK, Email

    The association between bronchiectasis and autoimmune disease is well recognised, and best described with rheumatoid arthritis. The prevalence of bronchiectasis in rheumatoid arthritis varies considerably in studies, with obliterative bronchiolitis a common feature. The prognosis of rheumatoid arthritis with bronchiectasis seems to be worse than either condition alone. The advent of high-resolution computed tomography has increased the sensitivity of detecting bronchiectasis, but this should be assessed for clinical significance. Traction bronchiectasis results from interstitial fibrosis pulling the airway wider, rather than damage weakening the bronchial wall, and is less likely to lead to bronchial suppuration. Bronchial wall damage in bronchiectasis is caused by inflammation, but it is difficult to differentiate damage caused by severe or recurrent infections, predisposed to by immunosuppression related to the autoimmune disease itself or its treatment, from damage caused by the autoimmune process. Increased use of new immunomodulatory or immunosuppressive agents has proved successful in modifying autoimmune disease processes, but has also led to emergence of infective complications that can cause bronchiectasis or exacerbate pre-existing disease.

  17. Page 211
    Correspondence: C.S. Haworth, Cambridge Centre for Lung Infection, Papworth Hospital, Cambridge, CB23 3RE, UK, Email

    Antibiotics play a crucial role in the management of patients with bronchiectasis by disrupting the vicious circle of infection, inflammation and airway damage central to the pathophysiology of the condition. Antibiotic use in patients with bronchiectasis can be divided into exacerbation treatment, chronic suppressive treatment and eradication treatment.

    Antibiotics administered during exacerbations are known to reduce serum C-reactive protein concentrations, sputum volume and bacterial density, as well as ameliorate symptoms. Clinical experience suggests that better outcomes are seen with higher dose/longer duration regimens.

    The prescription of long-term oral antibiotics should be considered in patients requiring exacerbation treatment at least three times per year. As patients chronically infected with Pseudomonas aeruginosa tend to have a faster rate of lung function decline, more admissions to hospital and a worse quality of life compared with bronchiectasis patients with other microorganisms, nebulised antipseudomonal antibiotics are commonly prescribed.

    Eradication antibiotics should be considered following identification of new growths of P. aeruginosa due to the increased morbidity associated with chronic infection.

  18. Page 223
    Correspondence: S.C. Bell, Dept of Thoracic Medicine, The Prince Charles Hospital, Rode Road, Chermside, Brisbane, QLD, 4032, Australia, Email

    Although the use of anti-inflammatory therapies in bronchiectasis remains an attractive proposition, there is currently insufficient evidence to support the use of inhaled and oral corticosteroids, non-steroidal anti-inflammatory drugs and macrolides. Individual patient trials may be warranted for inhaled corticosteroids and macrolides. It is hoped that recently completed and ongoing randomised control trials of macrolides will better define the use and safety in bronchiectasis. There remains an urgent need to perform adequately powered multicentre trials of other potentially useful therapies.

    It is anticipated that specialised bronchiectasis clinics will provide greater opportunities to study disease epidemiology and pathogenesis and allow better definition of study population for inclusion within future trials. There is a need for a more defined study population and a widely accepted definition of a pulmonary exacerbation in bronchiectasis which may be applied uniformly across studies to allow direct comparison of study outcomes. Finally, care should be taken to ensure adequate follow-up to detect potential adverse effects of new therapies, particularly on microbial resistance patterns.

  19. Page 239
    Correspondence: P.T. Bye, Dept of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Missenden Road, Camperdown, NSW 2050, Australia, Email

    Impaired mucociliary clearance and mucus retention contribute to the chronic cycle of airway inflammation, infection and damage in bronchiectasis. There is a strong rationale for the use of pharmacological strategies to aid airway clearance, often in combination with chest physiotherapy. Despite the availability of many candidate mucoactive agents, the evidence base for recommending these agents is currently limited. Recent research and trials have focused particularly on osmotic agents (hypertonic saline and mannitol), which increase airway hydration, and early studies appear promising for both of these agents. Dornase alfa is not effective in non-cystic fibrosis (CF) bronchiectasis, which underscores the importance of conducting high quality and adequately powered trials that specifically address the therapeutic options for non-CF bronchiectasis.

  20. Page 248
    Correspondence: J.D. Mitchell, Section of Thoracic Surgery, Division of Cardiothoracic Surgery, C-310, University of Colorado, Denver School of Medicine, 12631 E. 17th Avenue, C310, Aurora, CO 80045, USA, Email

    Surgical resection for bronchiectasis should be reserved for patients with localised disease who have failed medical management and have persistent symptoms that negatively affect their quality of life. Patients with unilateral segmental disease have the best outcomes. The key to successful surgical intervention includes: 1) complete resection of all affected areas; 2) relatively early intervention to prevent development of resistant organisms and spread to adjacent lung segments; 3) pre-operative targeted antimicrobial therapy based on in vitro sensitivities; 4) continuation of antimicrobial therapy post-operatively; 5) pre-operative nutritional supplementation when indicated; and 6) anticipation of potential complications that may alter the surgical approach. Surgical resection can be accomplished with minimal morbidity and mortality and it can usually be completed with a video-assisted thoracoscopic approach. The only surgical option for diffuse bronchiectasis is bilateral lung transplantation and is mainly employed when treating patients with cystic fibrosis.

  21. Page 258