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Respiratory tract diseases

Bronchiectasis: diagnosis, treatment and management

Bronchiectasis is a common chronic disease where the airways of the lungs become abnormally widened. Patients present with recurrent cough, sputum production and are at risk of respiratory tract infections. Pharmacists and healthcare professionals need to be aware of the latest management strategies.

Bronchiectasis, light photomicrograph showing dilated and distorted bronchus containing pus

Source: Shutterstock.com

Light photomicrograph showing dilatated and distorted bronchus containing pus. Bronchiectasis is associated with recurrent cough, sputum production, and recurrent chest infections

Bronchiectasis is the term given to abnormal irreversibly dilated and thickened bronchial walls. It is associated with recurrent cough and sputum production, and recurrent chest infections. Bronchiectasis is caused by repeated insults to the lower respiratory tract, altering muco-ciliary clearance that leads to stasis of secretions, infection, inflammation and, eventually, destruction and dilatation of the peribronchial and bronchial tree. The anatomical distribution of bronchiectasis may suggest an underlying aetiology. Upper lobe bronchiectasis may suggest cystic fibrosis (CF)-related bronchiectasis, allergic bronchopulmonary aspergillosis (ABPA), sarcoidosis or tuberculosis. A localised distribution of bronchiectasis suggests external compression, retained foreign body or even past pneumonia (see Table 1)[1]. CF-related bronchiectasis has a different pathophysiology, therefore, its management is different and not covered in this article.

Table 1: Main aetiologies of bronchiectasis
ConditionExample
Genetic disorders Cystic fibrosis, primary ciliary dyskinesia, alpha-1-antitrypsin deficiency, Young’s syndrome
CongenitalBronchomalacia
Post-infectious Mycobacteria (post-tuberculosis), pneumonia (bacterial, viral, fungal)
Iatrogenic  Post-chemotherapy/radiotherapy
Immune deficiency HIV, common variable immune deficiency, antibody deficiency
Exaggerated immune responseAllergic bronchopulmonary aspergillosis
Mechanical Foreign body, extrinsic compression, endobronchial lesions
Gastrointestinal disordersGastroesophageal reflux disease, inflammatory bowel disease
Connective tissue disorders Rheumatoid arthritis, systemic lupus erythematosus, Sjogren’s syndrome, sarcoidosis, ankylosing spondylitis  
Airways obstructionAsthma, chronic obstructive pulmonary disease (COPD)
IdiopathicNo specific cause identified

Epidemiology

Reported cases of bronchiectasis vary worldwide. In the UK, the point prevalence in 2013 in women was 566.1 per 100,000 individuals and 485.5 per 100,000 in men, with an increasing prevalence with age (more than 60 years old). There is around a two-fold increased mortality compared with the general population[2]. In practice, life expectancy will be dependent on the severity of bronchiectasis and likely to be unaffected in patients with mild bronchiectasis, but reduced in those with severe bronchiectasis.

Diagnosis

Clinical presentation or severity of bronchiectasis in a patient can be variable. Patients with bronchiectasis usually present with chronic sputum production and recurrent chest infections. Some may also have haemoptysis, pleuritic chest pain, breathlessness, lethargy and weight loss. Examination findings include any or all of: pulmonary crackles, finger clubbing, cachexia, respiratory failure and cor-pulmonale. Radiological confirmation is required with a computed tomography (CT) of the chest. This also helps assess the severity of bronchiectasis (cylindrical, varicose and cystic), as well as the possible aetiology. Overall, this is defined as clinically significant bronchiectasis. In practice, mild radiological bronchiectasis can be identified on CT scan of the chest but if there are no symptoms suggestive of bronchiectasis, no action is needed unless clinically significant bronchiectasis ensues.

Microbiology

Patients with more advanced bronchiectasis are frequently infected with potential pathogenic microorganisms. Haemophilus influenzae is the commonest pathogen isolated in bronchiectasis. Pseudomonas aeruginosa, Streptococcus pneumoniae, Staphylococcus aureus and Moraxella catarrhalis are also commonly isolated. In a study of 385 stable patients with bronchiectasis, pathogenic microorganisms were isolated in the baseline sputum cultures from 75.3% of patients. Of those with positive cultures, predominant organisms isolated were H. influenzae (38.6%); P. aeruginosa (21%); S. aureus (12.4%); M. catarrhalis (11.4%); S. pneumoniae (9.7%); and others (primarily enteric Gram-negative organisms, 9.3%)[3]. The presence of S. aureus may be indicative of bronchiectasis due to CF, post-tuberculosis and post-ABPA. More than one organism may be isolated over time. There is increased interest in the potential for cross-infection, particularly with patients infected with P. aeruginosa. A small study by Mitchelmore et al. found evidence of cross-infection between three of 46 patients within an unsegregated bronchiectasis cohort. Longitudinal surveillance is warranted[4].

Non-tuberculous mycobacterium (NTM) is being isolated more often (around 9%) from patients with bronchiectasis, therefore, cultures for these organisms should be performed, especially in the case of unexplained deterioration. As these organisms are mainly environmental, to attribute the organism for the clinical deterioration, there should be at least two positive mycobacterial cultures or a single culture from invasive sampling (e.g. broncho-alveolar lavage)[5][6]. NTM is known to cause both deteriorating nodular bronchiectasis and fibro-cavitatory disease. There are a variety of mycobacteria that have been implicated in bronchiectasis, with Mycobacterium avium complex being the most frequently identified mycobacterium to date.

The isolation of Candida species from sputum cultures usually indicates prior antibiotic treatment and does not require anti-fungal treatment. The isolation of Aspergillus fumigatus is seen in patients with ABPA and those taking long-term antibiotics.

Investigations

Initial investigations recommended in patients suspected of having bronchiectasis are aimed at establishing the underlying cause and assessing disease severity. Sputum is routinely tested for microbial culture and sensitivity, as well as mycobacterial culture. Relevant initial blood tests include full blood count, C-reactive protein and erythrocyte sedimentation rate (ESR) to assess the degree of systemic inflammation and whether there is peripheral blood eosinophilia. Depending on the suspected cause, other investigations may be necessary. Immunoglobulins (e.g. IgA, IgM and IgG, including IgG subclasses 1–4) are checked if immunoglobulin deficiency is suspected. Antibody levels to tetanus toxoid, S. pneumoniae and H. influenzae type B are assessed if antibody deficiency is suspected. Owing to the association with connective tissue disorders, anti-CCP, ANA, ANCA and anti-DS DNA can be tested. Finally, if allergic bronchopulmonary aspergillosis is suspected, the following investigations including serum IgE, skin prick testing or serum IgE testing to A. fumigatus and Aspergillus precipitins need to be checked. If CF is suspected, a sweat test for chloride must be carried out, along with molecular blood tests for CFTR mutation analysis. If PCD is suspected, a nasal nitric oxide test should be carried out in the first instance.

Pulmonary function

Variable lung function tests can be seen in patients with bronchiectasis, even in people with more severe disease (some may have normal spirometry, a restrictive defect or airway obstruction). Patients with a forced expiratory volume in 1 second (FEV1) <30% predicted are at increased risk of hospitalisation and have a higher mortality[7].

At least annual FEV1 and forced vital capacity (FVC) and the FEV1/FVC ratio monitoring in patients is useful. In addition, monitoring spirometry post treatment with intravenous antibiotics is recommended as one of the markers of treatment response.

Radiology

Despite being quite non-specific, chest x-rays are often the initial mode of imaging used for investigating bronchiectasis. However, changes are only notable in severe disease, therefore, a normal chest x-ray does not exclude bronchiectasis. A high-resolution CT scan of the chest is considered the radiological investigation of choice. The signet sign seen on CT represents bronchial wall dilatation when the internal lumen’s diameter is greater than the adjacent pulmonary artery. Spiral and volumetric CTs have higher resolution compared with standard CT, but carry a higher radiation dose[8]. CT scans can also assist in determining the cause of bronchiectasis such as ABPA (proximal changes), CF (upper lobe predominance), NTM (nodular bronchiectasis) and tracheobronchomegaly[9]. The severity of bronchiectasis on CT also correlates with the level of airway obstruction on pulmonary function tests[10]. Tubular bronchiectasis represents mild bronchiectasis, whereas varicose and cystic bronchiectasis represents more severe bronchiectasis. Having cystic bronchiectasis, or three or more lobes affected with bronchiectasis is used in the scoring system to assess risk of hospitalisation and mortality[7].

Bronchoscopy

If CT imaging shows localised disease, bronchoscopy is recommended to exclude an endobronchial lesion. Bronchoscopy with broncho-alveolar lavage is also useful in patients with suspected NTM infection who cannot produce regular sputum. In addition, bronchoscopy can help localise the site of bleeding in patients with haemoptysis and guide where to perform bronchial artery embolisation.

Treatment and goals

Symptom control, reduction in the number of exacerbations, preserving lung function and improving quality of life are the main aims of treatment in bronchiectasis. The British Thoracic Society (BTS) guidelines define an exacerbation as needing antibiotics and an acute deterioration with worsening symptoms (cough, increased sputum volume or change of viscosity, increased sputum purulence with or without increasing wheeze, breathlessness, haemoptysis) and/or systemic upset[6]. Patient education on the disease goes a long way in helping with understanding of the disease, recognising exacerbations and compliance with treatment. Patients should all be provided a self-management plan; an example of which can be found on the BTS website[6].

The BTS guidelines recommend that certain patients with bronchiectasis should have regular follow up in secondary care in view of their poor prognosis[6]. These include:

  • Patients with recurrent exacerbations — three or more per year;
  • Patients with deteriorating bronchiectasis;
  • Patients with bronchiectasis associated with rheumatoid arthritis, immune deficiency, inflammatory bowel disease and primary ciliary dyskinesia;
  • Patients with repeated isolates of NTM;
  • Patients with methicillin-resistant S. aureus (MRSA) colonisation;
  • Patients with chronic P. aeruginosa infection with two or more isolates, while stable in the last 12 months;
  • Patients with ABPA;
  • Patients on prophylactic antibiotic therapy;
  • Patients with advanced disease and those requiring transplantation.

Physiotherapy

Physiotherapy is an essential part of treatment in bronchiectasis. It helps with expectoration of bronchopulmonary secretions and improves effective ventilation[11]. Various airway clearance techniques can be used. In the UK, the active cycle breathing technique is the commonest technique, sometimes used in combination with postural drainage and manual techniques (see Table 2)[12]. Patients are encouraged to be as independent as possible in doing their own physiotherapy. In case of an exacerbation when it may be difficult for the patient to carry out routine exercises, manual techniques can be considered for airway clearance. Adjuncts to airway clearance, such as 0.9% saline or hypertonic saline, can also be helpful before starting physiotherapy as they decrease sputum viscosity and increase the ease to expectorate[13]. Lung function (in particular FEV1) should be checked before and after taking hypertonic saline because patients can develop bronchospasm. The duration and frequency of physiotherapy depends on the severity of bronchiectasis. In more advanced bronchiectasis, it should be carried out at least twice daily and sessions normally limited to 30 minutes per session. Currently there is very little information about the efficacy of non-invasive ventilation (NIV) in non-CF-bronchiectasis. In routine practice, NIV can be used in acute exacerbations in patients with hypercapnic respiratory failure.

Table 2: Showing physiotherapy techniques used for airway clearance in bronchiectasis
MethodDescription
Postural drainageThis method uses gravity to move mucus from the deeper parts of the lung to the upper airway to facilitate expectoration
Autogenic drainageThis technique aims to increase expiratory airflow. Patients breathe at different lung volumes to loosen secretions and mobilise it towards larger airways 
Positive expiratory pressure devicesThis technique involves devices, such as flutter valve, acapella and RC cornet 
High frequency chest wall oscillationAn inflatable vest performs chest physiotherapy by vibrating at a high frequency, which allows the patient to cough up secretions that have travelled to the larger airways

Nutrition

A nutritional assessment should be made in patients with severe bronchiectasis because they are at risk of malnutrition. During each review, the patient’s body mass index (BMI) should be checked. A BMI of less than 18.5kg/m2 or weight loss of more than 5% in eight weeks or 10% in six months is evidence of malnutrition[14]. A low BMI of less than 18.5kg/m2 is associated with poorer outcome[15].

Pharmacotherapy

Antibiotics for exacerbations

Several studies have shown that improved bacterial clearance is seen in patients who receive targeted antibiotics. Therefore, before starting antimicrobial therapy, a sputum sample should be sent for culture and sensitivity. If no microbiology is available, BTS guidelines recommend patients should initially be started on amoxicillin 500mg three-times daily (TDS) oral or clarithromycin 500mg twice daily (BD) oral (if they are allergic to penicillin)[6]. If patients are not responding to the empirical antibiotics, the sputum culture results and in vitro sensitivity test results should be reviewed. Higher doses of prolonged antibiotics are needed in patients who have purulent sputum and severe bronchiectasis[16]. Intravenous antibiotics are reserved for patients who have not responded to oral antibiotics, require hospital admission or have resistant pathogens (e.g. P. aeruginosa) that necessitate intravenous antibiotics. However, there are no randomised control trials (RCTs) that have explored the optimal duration of antibiotics. The BTS guidelines recommend 14 days for exacerbations because it is well recognised, particularly in more severe bronchiectasis, that prolonged and high antibiotic doses are needed[7]. Common antibiotics used are shown in Table 3.

Table 3: Antibiotics recommended for use in exacerbations
OrganismRecommended first-line treatmentRecommended second-line treatment

Streptococcus pneumoniae

Amoxicillin 500mg three-times daily (TDS)  

Doxycycline 100mg twice daily (BD)

Haemophilus influenzae— beta lactamase negative

Amoxicillin 500mg TDS

Or

Amoxicillin 1g TDS

Or

Amoxicillin 3g BD

Doxycycline 100mg BD

Or

Ciprofloxacin 500mg or 750mg BD

Or

Ceftriaxone 2g once daily (OD) intravenous

Haemophilus influenzae -beta lactamase positive

Amoxicillin with clavulanic acid 625mg one tablet TDS 

Doxycycline 100mg BD

Or

Ciprofloxacin 500mg or 750mg BD

Or 

Ceftriaxone 2g OD intravenous

Moraxella catarrhalis

Amoxicillin with clavulanic acid 625mg one tablet TDS

Clarithromycin 500mg BD

Or

Doxycycline 100mg BD

Or

Ciprofloxacin 500mg or 750mg BD

Methicillin-susceptible Staphylococcus aureus (MSSA)

Flucloxacillin 500mg four times daily (QDS) 

Clarithromycin 500mg BD

Or

Doxycycline 100mg BD

Or 

Amoxicillin with clavulanic acid 625mg one tablet TDS

Methicillin-resistant Staphylococcus aureus (MRSA)

Oral preparations

Doxycycline 100mg BD

 

Rifampicin (<50kg) 450mg OD 

Rifampicin (>50kg) 600mg OD

 

Trimethoprim

Third-line:

Linezolid 600mg BD

MRSA

Intravenous preparations

Vancomycin 1gm BD*

(monitor serum levels and adjust dose accordingly)

Or

Teicoplanin 400mg OD

Linezolid 600mg BD

Coliforms (e.g. Klebsiella, enterobacter)

Oral ciprofloxacin 500mg or 750mg BD

Intravenous

Ceftriaxone 2g OD

Pseudomonas aeruginosa

Oral ciprofloxacin 500mg BD (750mg BD in more severe infections)

Monotherapy:

Intravenous ceftazidime 2g TDS

Or

Piperacillin with tazobactam 4.5g TDS

Or

Aztreonam 2g TDS

Or

Meropenem 2g TDS

 

Combination therapy:

The above can be combined with gentamicin or tobramycin or colistin 2 MU TDS (<60kg; 50,000–75,000 units/kg daily in three divided doses)

Caution with aminoglycosides in pregnancy, renal failure, the elderly or those on multiple other medicines.

*Elderly (>65 years), 500mg vancomycin every 12 hours or 1g OD

Source: BNF 72 

The use of adjunct nebulised tobramycin given in combination with oral ciprofloxacin during exacerbations was more effective at reducing the bacterial load compared with oral ciprofloxacin alone, but did not improve clinical outcomes, which likely reflects the increased local side effects with inhaled tobramycin[17].

Combination antibiotics may be required in some circumstances but are normally not required for H. influenzae, M. catarrhalis, S. pneumoniae or S. aureus (methicillin sensitive). If there are multiple organisms grown on sputum culture, choosing an antibiotic that covers all these organisms is preferred[7]. In drug-resistant pathogens, there is no evidence that combination antibiotics leads to improved clinical outcomes, however, it is thought to reduce the risk of further drug resistance developing[6].

Long-term antibiotics

According to BTS guidelines, these are considered in patients who have three or more exacerbations in a year requiring antibiotics[7]. If patients are not having multiple exacerbations, but recover slowly from exacerbations, they can also be considered for long-term antibiotic therapy.

Macrolides:  The BLESS trial was a double-blind, randomised, placebo-controlled trial that assessed the effect of long-term, low-dose erythromycin (erythromycin ethylsuccinate; 400mg, which is equivalent to 250mg erythromycin base) BD on exacerbations of bronchiectasis. The primary outcome was the reduction in annual mean rate of protocol defined pulmonary exacerbations per patient. In total, 117 patients were recruited and randomised to either the erythromycin group (n=59) or the placebo group (n=58). The study demonstrated a significant reduction in the number of exacerbations in the erythromycin group compared with the placebo group overall and a pre-specified subgroup with baseline P. aeruginosa infection. The erythromycin group also showed a reduction in sputum production over 24 hours and attenuation of lung function decline. However, there was an increased rate of macrolide resistance in the erythromycin group[18].

The BAT trial assessed the efficacy of daily oral azithromycin maintenance treatment on exacerbations of non-CF bronchiectasis during one year of treatment. Overall, 43 patients were randomised to receive 250mg of azithromycin daily, while 40 patients received placebo. The study concluded that patients on azithromycin 250mg once daily for a year compared with placebo had fewer exacerbations during treatment, showed improved FEV1 and FVC and quality of life. However, the sputum microbiology remained similar[19].

The EMBRACE trial assessed azithromycin 500mg three times a week for six months and showed a reduction in overall exacerbations at the end of treatment and six months post-treatment. Azithromycin is thought to have bacteriostatic and anti-inflammatory effects by decreasing the production of pro-inflammatory cytokines[20]. Patients on long-term macrolides may have experienced side effects such as tinnitus, hearing impairment, cholestatic hepatitis, gastrointestinal problems and possibly increased vascular events. The long-term impact of increased macrolide resistance with the oropharyngeal flora needs to be established.

Other long-term antibiotics:  A systematic review on inhaled/nebulised antibiotics from 28 days to one year showed that they may provide an effective bacterial suppressive therapy with an acceptable safety profile in adult patients with stable non-CF bronchiectasis and chronic bronchial infection[21].

The efficacy of nebulised gentamicin over 12 months in non-CF bronchiectasis was assessed in a RCT published in 2011. In total, 65 patients were randomised to either 80mg BD of nebulised gentamicin or placebo (nebulised saline 0.9%). The gentamicin group were found to have reduced bacterial density compared with the saline group, as well as 30.8% eradication in those infected with P. aeruginosa and 92.8% with other pathogens. The nebulised gentamicin group were also found to have less sputum purulence (p<0.0001), greater exercise capacity (p=0.03) and fewer exacerbations (p<0.0001) with increased time to first exacerbation. In terms of quality of life, the gentamicin group showed greater improvements in their St. George’s Respiratory questionnaire (p<0.004)[22].

Two randomised, double-blind, placebo-controlled phase III trials assessed the safety and efficacy of nebulised aztreonam. The trials (AIR-BX1 and AIR-BX2) included patients who had bronchiectasis with Gram-negative organisms from sputum or bronchoscopic samples. Patients were randomised to receive aztreonam for inhalation solution (AZLI) 75mg TDS or placebo TDS. In both studies, two 4-week courses of nebulised AZLI 75mg or placebo were each followed by a 4-week off-treatment period. In AIR-BX1, 134 patients received AZLI and 132 received placebo. In AIR-BX2, 136 patients received AZLI, while 138 patients received placebo. The primary end point was change from baseline of the quality of life-bronchiectasis respiratory symptoms score at 4 weeks. AZLI did not provide significant clinical benefit as a treatment for non-CF bronchiectasis because there was insignificant improvement in the primary end point and there were more adverse events, in particular increased dyspnoea, cough and sputum production[23].

Nebulised tobramycin has been investigated for use in patients with bronchiectasis and chronic infection with P. aeruginosa. A small crossover study, published in 2005, was carried out to assess the clinical effectiveness and safety of nebulised tobramycin therapy. Patients (n=30) were given 300mg nebulised tobramycin or placebo BD for six months, followed by a one month washout period before receiving the other therapy. With nebulised tobramycin, there was a reduction in P. aeruginosa density in sputum, a reduction in risk of hospitalisation and number of days of hospitalisation. Overall, 10% of patients stopped treatment due to bronchospasm[24].

The outcomes of the one-year studies with inhaled ciprofloxacin and nebulised ciprofloxacin are yet to be published.

Licensed inhaled/nebulised antibiotics for patients with bronchiectasis are urgently needed; in the UK nebulised gentamicin, colomycin and tobramycin are all used, but are currently unlicensed.

Antimicrobials for NTM

For patients who require treatment, it is usually for 18–24 months, and in the UK triple therapy is given (rifampicin plus ethambutol and clarithromycin/ciprofloxacin/moxifloxacin). In macrolide-resistant organisms, an alternative regimen could be used such as rifampicin, ethambutol and moxifloxacin. Inhaled amikacin may be a useful adjunct in poorly responding patients to enhance mycobacterial clearance.

Allergic bronchopulmonary aspergillosis (ABPA)

For active ABPA, treatment is with oral corticosteroids. An initial dose of prednisolone 0.5mg/kg/d, for 1–2 weeks, is recommended, before weaning by 5–10mg every 2 weeks until discontinuing according to clinical response and serum IgE levels. Itraconazole is used as a steroid-sparing agent for patients dependent on oral corticosteroids where difficulty in weaning is experienced. In addition, removal of environmental exposure of A. fumigatus spores is recommended (e.g. renovation, construction, mucking out stables).

Mucolytics

The only study in bronchiectasis where there is sufficient evidence for mucolytics is for nebulised DNase, which is essentially a mucus-degrading agent. The BTS guidelines on bronchiectasis recommend not using DNase because the previous RCT in 349 patients showed increased exacerbations and an accelerated fall in FEV1 for those taking nebulised DNAse for 24 weeks[6],[25].

Hyperosmolar agents

These agents are used to improve hydration of the airway surface and mucociliary clearance. The hyperosmolar agents that we have most evidence for are hypertonic saline and mannitol. The study by Nicolson and colleagues showed similar efficacy with 0.9% saline and hypertonic saline[26]. However, further studies are required to show whether saline (0.9% or hypertonic) is recommended in practice. Bilton et al. studied the effects of inhaled mannitol in a 12-month, double-blinded RCT. There was no statistically significant reduction in exacerbation rates. There was, however, an improvement in time to first exacerbation and quality of life[27].

Bronchodilators

Bronchodilators are often used to treat more advanced bronchiectasis, especially where patients also have co-existing asthma or COPD. Bronchodilators improve ciliary motility and facilitate clearance of secretions, and they are sometimes used before physiotherapy. Beta agonists and anticholinergics are often recommended for patients with breathlessness, however, no trials have assessed the effect of them in patients with bronchiectasis. If there is benefit with short-acting bronchodilators, long-acting bronchodilators are used.

Inhaled corticosteroids (ICS)

A double-blind, placebo-controlled trial showed that ICS, in the form of fluticasone, are associated with reduced neutrophil count and inflammatory markers such as interleukins-1,8 and Leukotriene B4[28]. Martinez-Garcia et al. in their prospective, randomised, double-blind study investigated whether ICS improve quality of life. Overall, 93 patients were randomised to receive either fluticasone 250µg BD, fluticasone 500µg BD or no treatment for 6 months. Patients who received 500µg BD of fluticasone had better symptom control from the first month of treatment according to their health-related questionnaire scores[29]. However, to date, RCTs have not shown a reduction of exacerbations. ICS are recommended by the BTS guidelines in patients with co-existing asthma and COPD, if they meet the criteria for ICS for asthma or COPD[6].

Combination inhaled therapy

A double-blind, randomised, parallel group clinical study of non-CF bronchiectasis patients (n=40) evaluated the efficacy and safety of medium-dose formeterol–budesonide (18/640µg daily) combination inhaler compared with a high-dose budesonide (1600µg daily) treatment over a one-year period. The formoterol–budesonide combination therapy was found to be more efficacious and safe in terms of symptom control, improvement in quality of life and fewer side effects than high-dose ICS alone[30]. There is also a theoretical increased risk of pneumonia and NTM infection in patients with chronic respiratory disease, such as bronchiectasis on ICS[31],[32].

Vaccination

There have been no specific RCTs on vaccines for patients with bronchiectasis. However, patients should still be advised to have the influenza vaccine on an annual basis and pneumococcal polysaccharide vaccine (PPV-23) if they have not had it previously.

Surgery

Patients who demonstrate unilateral localised disease and are not improving with maximal medical therapy should consider resection surgery[33]. Patients who have massive haemoptysis should also consider surgery. Currently, bronchial artery embolisation is the first-line treatment and surgery regarded as second line. Bilateral lung transplantation should be considered in patients aged 60 years and under with end-stage disease and predicted FEV1 of <30% if they continue to deteriorate despite medical therapy. Five-year survival is thought to be around 55–60%[34]. In most patients, double lung transplant is preferred to single lung transplant because the transplanted lung may become infected by pathogens that have colonised the diseased lung.

End-of-life care

Long-term oxygen therapy should be considered in bronchiectasis patients who develop respiratory failure. Along with active treatment, it is important to recognise when a patient with end-stage bronchiectasis is approaching end of life. Involvement of the palliative care team and planning for an advance directive are useful in symptom control and respecting the patient’s wishes.

Future perspective

Management of bronchiectasis will benefit from national and international networks and consensus guidelines, such as the recently published European Respiratory Society guidelines[35] on bronchiectasis, and the, soon to be published, updated BTS guidelines[6][35]. There have been BTS quality standards in bronchiectasis, which aim to improve care throughout the UK and, if adopted, internationally. Therefore, it will be interesting whether guidelines, quality standards and self-management plans lead to qualitative or quantitative improvement. Owing to the difficulties in treating bronchiectasis and the absence of large randomised trials, there is an urgent need for licensed therapies, in particular, long-term antibiotics, long-term anti-inflammatory treatment and muco-active therapies. 

Financial and conflicts of interest disclosure:

The authors have no relevant affiliations or financial involvement with any organisation or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. No writing assistance was used in the production of this manuscript.

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Citation: Clinical Pharmacist DOI: 10.1211/CP.2017.20203757

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Supplementary images

  • Bronchiectasis, light photomicrograph showing dilated and distorted bronchus containing pus

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