Beyond asthma and COPD: a closer look into the diagnosis and management of bronchiectasis

In the past two decades, bronchiectasis has been considered an orphan disease with little understanding of the underlying pathophysiology and no single dedicated treatment.^[1] A coordinated effort by bronchiectasis experts around the globe in the last 10-15 years has seen understanding of the condition deepen and several specific treatments now in clinical trials.^[2],[3]

Each year, some 20,000 people are diagnosed with bronchiectasis, and from 2008 to 2012 prevalence increased by 20%, with the number of people recorded to be living with bronchiectasis going up by 40,000.^[4] The current UK prevalence equates to 1 patient with bronchiectasis for every 20 with chronic obstructive pulmonary disease (COPD) in primary care.^[5]

In my experience, primary care and general respiratory physicians are seeing more of the condition. This article will outline the important points in the diagnosis and management of bronchiectasis.

Diagnosing bronchiectasis

Diagnostic delay, or the time between onset of symptoms and clinical diagnosis, is common in many chronic diseases and particularly in respiratory conditions.^[6] One study on diagnostic delay in bronchiectasis concluded that the delay could be as long as 17 years.^[6]

The clinical presentation is typically either of recurrent chest infections or chronic cough (> 8 weeks) productive of mucopurulent sputum.^[7]  The condition is diagnosed by high resolution computed tomography scan (HRCT) identifying radiographic features including bronchial dilatation.^[8]

There are four key factors influencing the development of bronchiectasis:^[9]

• impaired mucociliary function
• chronic infection
• neutrophilic inflammation
• structural damage to the airways.

This cycle of pathophysiology illustrates the complexity of the disease.  Patients can enter this cycle at any stage and, as a result, there are many different aetiologies underlying bronchiectasis.^[9] Despite improved understanding of the condition, around 40% of cases remain idiopathic, with no underlying aetiology identified.^[10]

Asthma and COPD can both lead to bronchiectasis and overlap in symptoms between these three conditions can also lead to misdiagnosis.^[11],[12] Severely asthmatic patients and frequently exacerbating COPD patients, particularly those isolating pseudomonas in sputum, may have bronchiectasis and should be referred for an HRCT.^[8]

Patients with other inflammatory conditions, such as rheumatoid arthritis and inflammatory bowel disease, are at an increased risk of developing bronchiectasis, so a higher index of suspicion is required.^[8]

Patients suspected of having bronchiectasis require a baseline chest X-ray and then an HRCT scan to confirm diagnosis.^[8] Chest X-ray is not sensitive or specific for diagnosis of bronchiectasis.^[13] Where bronchiectasis is confirmed, a referral to secondary care should be made to define the underlying aetiology and initiate appropriate treatment.^[8]

Managing bronchiectasis

Chest physiotherapy is central to the management of both stable and exacerbating bronchiectasis.^[14] The British Thoracic Society Guideline for bronchiectasis in adults recommends that all patients are seen by a respiratory physiotherapist to be given education about their condition and taught airway clearance techniques.^[8]

It is also recommended that exacerbating patients are seen daily by a respiratory physiotherapist until their airway clearance is optimised.^[8] However, access to respiratory physiotherapy is variable across the UK,^[15] and many countries across Europe do not have capacity to support this recommendation.^[16]

Bronchiectasis exacerbations

Many clinicians will see bronchiectasis patients at the point of exacerbation. The current definition of an exacerbation is:^[17]

“…a person with bronchiectasis with a deterioration of three or more key symptoms for at least 48 hours, in addition to a clinician’s decision that a change in bronchiectasis treatment is required.”

Key symptoms include cough, sputum volume and/or consistency, sputum purulence, breathlessness and/or exercise intolerance, fatigue and/or malaise and haemoptysis.^[17]

At exacerbation, empirical antibiotic treatment should be given, guided by previous culture results if these are available.^[8] It is recommended that sputum be sent for culture prior to commencement of treatment and change to antibiotics made if clinical response to initial treatment is poor and sputum microbiology suggests resistant organisms.^[8] Antibiotic treatment should be given for two weeks,^[18] rather than the standard of five days given in other respiratory conditions.

Frequently exacerbating patients (three or more exacerbations in 12 months) and those with new isolation of pseudomonas in sputum are those who require referral back to secondary care, if not already under their care.^[8] Both these groups have poorer disease outcomes and may require additional treatments such as oral or nebulised antibiotic prophylaxis.^{[19],[20],[8]}

Summary

Bronchiectasis is an increasingly prevalent condition presenting with chronic productive cough or recurrent chest infections.^[5] A high index of suspicion is required in patients with severe or poorly controlled asthma, COPD patients with frequent exacerbations and patients with co-morbid inflammatory conditions such as rheumatoid arthritis, if they have symptoms of chronic productive cough or recurrent chest infections.^[8]

Where a suspicion of bronchiectasis exists, patients should be referred for HRCT scan of the chest and confirmation of diagnosis should prompt referral to a respiratory physician for further investigations towards aetiology and initiation of relevant treatment.

Generalist physicians will come in to contact with exacerbating patients and these patients require sputum culture to be sent and empirical antibiotic treatment for two weeks, with good attention to airway clearance remaining the cornerstone of management.^[8]

Any advice given and opinions expressed in this article are those of the author and do not reflect the view of Chiesi Limited (Chiesi).  All content in this article is for informational and educational purposes only.  Although Chiesi strives to always provide accurate information, it is not responsible for and does not verify for accuracy any of the information contained within.

[1] Chalmers JD, Aliberti S, Blasi F. Management of bronchiectasis in adults. Eur Respir J. 2015; 45(5): 1446-1462

[2] Chalmers JD, Haworth CS, Metersky ML, et al. Phase 2 Trial of the DPP-1 Inhibitor Brensocatib in Bronchiectasis. The New England Journal of Medicine. 2020; 383(22): 2127-2137

[3] University of Dundee. Gremubamab Eradication Trial – 2. Available at: https://sites.dundee.ac.uk/great-2/

[4] Asthma + Lung UK. The battle for breath – the impact of lung disease in the UK. Available at: https://www.asthmaandlung.org.uk/battle-breath-report

[5] Chalmers JD, Sethi S. Raising awareness of bronchiectasis in primary care: overview of diagnosis and management strategies in adults. NPJ Prim Care Respir Med. 2017; 27(1): 18

[6] Girón RM, de Gracia Roldán J, Olveira C, et al. Sex bias in diagnostic delay in bronchiectasis: An analysis of the Spanish Historical Registry of Bronchiectasis. Chron Respir Dis. 2017; 14(4): 360-369

[7] National Institute for Health and Care Excellence. Bronchiectasis. Available at: https://cks.nice.org.uk/topics/bronchiectasis/

[8] Hill AT, Sullivan AL, Chalmers JD, et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2019; 74(Suppl 1): 1-69

[9] Flume PA, Chalmers JD, Olivier KN. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity. Lancet. 2018; 392(10150): 880-890

[10] Chalmers JD, Polverino E, Crichton ML, et al. Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC). Lancet Respir Med. 2023; 11(7): 637-649

[11] Polverino E, Dimakou K, Hurst J, et al. The overlap between bronchiectasis and chronic airway diseases: state of the art and future directions. Eur Respir J. 2018; 52(3): 1800328

[12] Hurst JR, Elborn JS, De Soyza A; BRONCH-UK Consortium. COPD-bronchiectasis overlap syndrome. Eur Respir J. 2015; 45(2): 310-313

[13] Ledda RE, Balbi M, Milone F, et al. Imaging in non-cystic fibrosis bronchiectasis and current limitations. BJR Open. 2021; 3(1): 20210026

[14] Phillips J, Lee A, Pope R, Hing W. Physiotherapists’ use of airway clearance techniques during an acute exacerbation of bronchiectasis: a survey study. Arch Physiother. 2021; 11(1): 3

[15] Taskforce for Lung Health. Increasing access to pulmonary rehabilitation for people with lung conditions could save NHS England £69m every year. Available at: https://www.taskforceforlunghealth.org.uk/taskforce/press-release/increasing-access-to-pulmonary-rehabilitation-for-people-with-lung-conditions

[16] Spinou A, Chalmers JD. Respiratory physiotherapy in the bronchiectasis guidelines: is there a loud voice we are yet to hear? Eur Respir J. 2019; 54(3): 1901610

[17] Choi H, Chalmers JD. Bronchiectasis exacerbation: a narrative review of causes, risk factors, management and prevention. Ann Transl Med. 2023; 11(1): 25

[18] Polverino E, Goeminne PC, McDonnell MJ, et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017; 50(3): 1700629

[19] Chalmers JD, Aliberti S, Filonenko A, et al. Characterization of the “Frequent Exacerbator Phenotype” in Bronchiectasis. Am J Respir Crit Care Med. 2018; 197(11): 1410-1420

[20] Finch S, McDonnell MJ, Abo-Leyah H, Aliberti S, Chalmers JD. A Comprehensive Analysis of the Impact of Pseudomonas aeruginosa Colonization on Prognosis in Adult Bronchiectasis. Ann Am Thorac Soc. 2015; 12(11): 1602-1611