Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

initial presentation

1st line – exercise and improved nutrition

Back
INITIAL
|
initial presentation
1st line – 

exercise and improved nutrition

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72]

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation.

In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

Plus – airway clearance therapy

Back
INITIAL
|
initial presentation
Plus – 

airway clearance therapy

Treatment recommended for ALL patients in selected patient group

Airway clearance therapy includes maintenance of oral hydration; percussion, breathing, or coughing strategies (e.g., active cycle of breathing, directed coughing, and autogenic drainage); positioning and postural drainage; positive expiratory pressure devices; and oscillatory devices. These techniques may be used alone or in combination.[66]

Therapy is generally recommended for 15 to 30 minutes, 2 or 3 times daily. Many of these interventions require the assistance of a carer. The therapy is time-consuming, and patients often find the process unpleasant.[65] Patient preference should be strongly considered in which technique is chosen.

British Thoracic Society guidelines recommend that adults with bronchiectasis are offered active cycle of breathing techniques or oscillating positive expiratory pressure, with gravity-assisted positioning to enhance their effectiveness.[46] However, there is insufficient evidence that any airway clearance technique is better than any other.[65][67][68] Long-term benefit out to 1 year was demonstrated for the ELTGOL technique (slow expiration with glottis open on lateral side) compared with placebo in terms of fewer exacerbations, improved quality of life, and reduced cough impact.[69] 

In children and adolescents, regular airway clearance should be individualised according to the patients’ age and developmental stage and reviewed at least biannually by a respiratory physiotherapist with paediatric expertise.[10]

Plus – self-management plan

Back
INITIAL
|
initial presentation
Plus – 

self-management plan

Treatment recommended for ALL patients in selected patient group

Self-management plans aim to increase patients’ confidence in managing their own condition. The British Thoracic Society recommends they are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help. Select patients may be given antibiotics to keep in reserve at home in case of exacerbations. When possible, sputum should be collected for culture and sensitivity testing before starting antibiotics.[46] Although there is currently insufficient clinical evidence to show whether self-management plans benefit people with bronchiectasis, they have been shown to be effective in other conditions such as chronic obstructive pulmonary disease.[76]

Consider – inhaled bronchodilator

Back
INITIAL
|
initial presentation
Consider – 

inhaled bronchodilator

Additional treatment recommended for SOME patients in selected patient group

Nebulised bronchodilators (e.g., salbutamol) given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46]

In children and adolescents, guidelines recommend that patients with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10]

Primary options

salbutamol inhaled: children 4-11 years of age: 2.5 to 5 mg inhaled via nebuliser up to four times daily when required; adults: 100-200 micrograms (1-2 puffs) up to four times daily when required, or 2.5 to 5 mg inhaled via nebuliser up to four times daily when required

Consider – mucoactive agent

Back
INITIAL
|
initial presentation
Consider – 

mucoactive agent

Additional treatment recommended for SOME patients in selected patient group

Use of nebulised hyperosmolar agents, such as hypertonic saline, promote mucus clearance by inducing coughing. Nebulised hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[96][97] It may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[98] Bronchodilators should be used prior to administration of nebulised hyperosmolar agents.

Guidelines from the British Thoracic Society (BTS) recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[46]

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10] 

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105]

ACUTE

acute exacerbation: mild to moderate underlying disease

1st line – antibiotic eradication therapy

Back
ACUTE
|
acute exacerbation: mild to moderate underlying disease
|
first or new isolation of Pseudomonas aeruginosa
1st line – 

antibiotic eradication therapy

An acute exacerbation typically presents as worsening of cough, change in sputum colour, increase in sputum volume, fever, and/or malaise. Severity of underlying disease in adults can be scored using the Bronchiectasis Severity Index (BSI).[46][109][110][111]

In children and adolescents, an acute exacerbation can be defined as increased respiratory symptoms (predominantly cough with or without increased sputum volume and/or purulence) for 3 days or more. For children and adolescents with immunodeficiency a shorter time frame is used. Children with dyspnoea and/or hypoxia for any duration should be considered as having a severe exacerbation, and they require immediate treatment.[10]

The European Respiratory Society (ERS) recommends all adult patients should be offered eradication antibiotic therapy on a first or new detection of P aeruginosa although it notes that this recommendation is based on very low-quality evidence.[79]

The British Thoracic Society (BTS) also recommends eradication antibiotic treatment in adult patients with bronchiectasis associated with clinical deterioration and a new growth of P aeruginosa. If a new growth of P aeruginosa is detected in the context of stable bronchiectasis, then the BTS guideline recommends discussing the risks and benefits of eradication treatment with the patient, compared with clinical observation alone.[46] 

There is some evidence that including a nebulised antibiotic in eradication treatment for P aeruginosa is more efficacious than intravenous treatment alone.[79] 

For adult patients with a first or new isolation of P aeruginosa, the ERS outlines some commonly used treatment approaches, but notes that there is no clear evidence to support one regimen over another. The ERS outlines three suggested eradication regimens, which are all for a total duration of 3 months: (1) an oral fluoroquinolone (such as ciprofloxacin) for an initial 2-week period followed by intravenous antibiotics (e.g., a beta-lactam plus an aminoglycoside), followed by inhaled antibiotics (e.g., colistimethate, tobramycin, or gentamicin); (2) intravenous antibiotics (e.g., a beta-lactam plus an aminoglycoside) for an initial 2-week period, followed by inhaled antibiotics (e.g., colistimethate, tobramycin, or gentamicin); or (3) a 2-week initial phase of oral fluoroquinolone or intravenous antibiotics, plus inhaled antibiotics (e.g., ciprofloxacin plus inhaled colistimethate) followed by continued inhaled antibiotics alone.[79] After each phase, the ERS guideline recommends repeating sputum sampling and only moving to the next step if the culture is positive for P aeruginosa.[79] 

Cefepime may be used for adult patients with known P aeruginosa resistant to fluoroquinolones. Other intravenous options for adult patients with P aeruginosa include ceftazidime, piperacillin/tazobactam, aztreonam, and meropenem. Combination therapy may be needed in certain patients with known P aeruginosa, and advice should be sought from an infectious disease specialist regarding selection of a suitable regimen.

The BTS guideline on bronchiectasis in adults recommends oral ciprofloxacin for 2 weeks as first-line treatment. As second-line treatment, the guideline recommends an intravenous antipseudomonal beta-lactam antibiotic, with or without an intravenous aminoglycoside, for 2 weeks, followed by 3 months of nebulised colistimethate, gentamicin, or tobramycin.[46] 

For children and adolescents with a confirmed first or new isolation of P aeruginosa, the ERS recommends a stepwise treatment approach depending on whether the child is symptomatic. For asymptomatic children, oral ciprofloxacin and/or inhaled antibiotics for 2 weeks are recommended first, followed by inhaled antibiotics for 4-12 weeks (e.g., colistimethate, tobramycin). This should be followed by a repeat specimen from the child’s lower airway, if possible. If P aeruginosa is still present, or if the child becomes symptomatic, then the child should receive treatment as per symptomatic children.[10] 

For children with increased symptoms from baseline, intravenous antibiotics are recommended for 2 weeks (e.g., piperacillin/tazobactam or ceftazidime plus tobramycin) followed by inhaled antibiotics for 4-12 weeks (e.g., colistimethate, tobramycin).[10] Antibiotic choices will depend on patient factors, Pseudomonas susceptibility profile, and availability of antibiotics.[10] Inhaled antibiotics should be followed by a repeat specimen from the child’s lower airway, if possible. If P aeruginosa is still present, clinicians should consider repeating intravenous antibiotics, followed by inhaled antibiotics, at least once.[10]

Consult your local protocols for guidance on suitable eradication therapy regimens.

Plus – increased airway clearance

Back
ACUTE
|
acute exacerbation: mild to moderate underlying disease
|
first or new isolation of Pseudomonas aeruginosa
Plus – 

increased airway clearance

Treatment recommended for ALL patients in selected patient group

Airway clearance to clear mucus, with or without bronchodilators, is important and should be increased in frequency for patients of any disease severity during the treatment of exacerbations.

Plus – continued maintenance therapy

Back
ACUTE
|
acute exacerbation: mild to moderate underlying disease
|
first or new isolation of Pseudomonas aeruginosa
Plus – 

continued maintenance therapy

Treatment recommended for ALL patients in selected patient group

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72] 

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at least 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

The British Thoracic Society (BTS) recommends self-management plans are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help.[46] 

Nebulised bronchodilators given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46] Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10] 

Use of nebulised hyperosmolar agents, such as hypertonic saline, promotes mucus clearance by inducing coughing. Nebulised hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[96][97] It may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[98] Bronchodilators should be used prior to administration of nebulised hyperosmolar agents. 

Guidelines from the BTS recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[46] 

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10] 

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105] 

1st line – short-term oral antibiotic

Back
ACUTE
|
acute exacerbation: mild to moderate underlying disease
|
Pseudomonas aeruginosa not isolated, or chronically infected with Pseudomonas aeruginosa
1st line – 

short-term oral antibiotic

An acute exacerbation typically presents as worsening of cough, change in sputum colour, increase in sputum volume, fever, and/or malaise. Severity of underlying disease in adults can be scored using the Bronchiectasis Severity Index (BSI).[46][109][110][111]

In children and adolescents, an acute exacerbation can be defined as increased respiratory symptoms (predominantly cough with or without increased sputum volume and/or purulence) for 3 days or more. For children and adolescents with immunodeficiency a shorter time frame is used. Children with dyspnoea and/or hypoxia for any duration should be considered as having a severe exacerbation, and they require immediate treatment.[10]

Antibiotics are the mainstay of treatment and should be selected for their activity against likely pathogens.

For adults who have never had an exacerbation, and never had P aeruginosa in their sputum cultures, an appropriate initial choice would be an antibiotic with coverage against Haemophilus influenzae or Staphylococcus aureus, depending on the culture results.[46]

For adults with low likelihood of P aeruginosa, oral antibiotic treatment is appropriate. In patients with mild to moderate underlying disease and a known P aeruginosa infection and symptoms of a bronchiectasis exacerbation, antibiotics should be directed toward Pseudomonas species sensitivities. This would be in a patient known to be chronically infected with P aeruginosa; a first or new isolation of P aeruginosa would prompt eradication therapy. Sensitivity to fluoroquinolones must be confirmed when using oral therapy.

Examples of potentially suitable regimens for adults are listed above. The British Thoracic Society guideline on bronchiectasis lists the common organisms associated with acute exacerbations of bronchiectasis, along with suggested first-line and second-line antimicrobial agents. For Streptococcus pneumoniae, oral amoxicillin is recommended as a first-line option, while oral doxycycline can be used second-line. Oral amoxicillin is also recommended first-line for H influenzae that is beta-lactamase negative, with doxycycline and ciprofloxacin as second-line oral options. For H influenzae that is beta-lactamase positive, oral amoxicillin/clavulanate is the recommended first-line option, with oral doxycycline and ciprofloxacin as second-line options. Oral amoxicillin/clavulanate is also recommended as a first-line option for Moraxella catarrhalis, with oral clarithromycin, doxycycline, and ciprofloxacin as second-line options. For methicillin-sensitive S aureus (MSSA), options include oral clarithromycin, doxycycline, and amoxicillin/clavulanate. For methicillin-resistant S aureus (MRSA), first-line oral options include doxycycline, rifampicin, and trimethoprim/sulfamethoxazole, with linezolid as a second-line option. For coliforms, such as Klebsiella and Enterobacter, oral ciprofloxacin is the recommended first-line option. Oral ciprofloxacin is also the recommended first-line option for P aeruginosa.[46]

For children and adolescents, the empirical antibiotic of choice is oral amoxicillin/clavulanate but, as with adults, the antibiotic should be chosen according to airway cultures and previous hypersensitivity reactions.[10]

If a patient is already taking long-term antibiotics, but experiences a further exacerbation, then the author of this topic recommends continuing the maintenance antibiotic unless there are drug-drug interactions that preclude co-administration, or if there are substantial changes in antibiotic susceptibility.

It should be noted that fluoroquinolone antibiotics, such as ciprofloxacin, are associated with serious, disabling, and potentially irreversible adverse effects when taken systemically or inhaled. These adverse effects include tendonitis, tendon rupture, arthralgia, neuropathies, aortic dissection, significant hypoglycaemia, mental health adverse effects, and other musculoskeletal or nervous system effects.[90][91][92]

Treatment course: 14 days. The regimens shown below are examples. Where possible, antibiotic choice should be based on culture and sensitivity from sputum samples.[46]

Primary options

Adults

amoxicillin: 500-875 mg orally twice daily, or 250-500 mg orally three times daily

OR

Adults

amoxicillin/clavulanate: 500-875 mg orally twice daily, or 250-500 mg orally three times daily

More

OR

Adults

doxycycline: 100 mg orally twice daily

OR

Adults

rifampicin: 600 mg orally once daily

OR

Adults

clarithromycin: 500 mg orally twice daily

OR

Adults

trimethoprim/sulfamethoxazole: 160/800 mg orally twice daily

OR

Adults

ciprofloxacin: 500-750 mg orally twice daily

OR

Adults

linezolid: 600 mg orally twice daily

OR

Children and adolescents

amoxicillin/clavulanate: children <3 months of age: 30 mg/kg/day orally given in 2 divided doses; children ≥3 months of age and body weight <40 kg: 25-45 mg/kg/day orally given in 2 divided doses, or 20-40 mg/kg/day orally given in 3 divided doses; children ≥3 months of age and body weight ≥40 kg and adolescents: 500-875 mg orally twice daily, or 250-500 mg orally three times daily

More

Plus – increased airway clearance

Back
ACUTE
|
acute exacerbation: mild to moderate underlying disease
|
Pseudomonas aeruginosa not isolated, or chronically infected with Pseudomonas aeruginosa
Plus – 

increased airway clearance

Treatment recommended for ALL patients in selected patient group

Airway clearance to clear mucus, with or without bronchodilators, is important and should be increased in frequency for patients of any disease severity during the treatment of exacerbations.

Plus – continued maintenance therapy

Back
ACUTE
|
acute exacerbation: mild to moderate underlying disease
|
Pseudomonas aeruginosa not isolated, or chronically infected with Pseudomonas aeruginosa
Plus – 

continued maintenance therapy

Treatment recommended for ALL patients in selected patient group

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72] 

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at least 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

The British Thoracic Society (BTS) recommends self-management plans are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help.[46] 

Nebulised bronchodilators given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46] Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10] 

Use of nebulised hyperosmolar agents, such as hypertonic saline, promotes mucus clearance by inducing coughing. Nebulised hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[96][97] It may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[98] Bronchodilators should be used prior to administration of nebulised hyperosmolar agents. 

Guidelines from the BTS recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[46] 

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10] 

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105] 

acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics

1st line – antibiotic eradication therapy

Back
ACUTE
|
acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics
|
first or new isolation of Pseudomonas aeruginosa
1st line – 

antibiotic eradication therapy

An acute exacerbation typically presents as worsening of cough, change in sputum colour, increase in sputum volume, fever, and/or malaise. Severity of underlying disease in adults can be scored using the Bronchiectasis Severity Index (BSI).[46][109][110][111]

In children and adolescents, an acute exacerbation can be defined as increased respiratory symptoms (predominantly cough with or without increased sputum volume and/or purulence) for 3 days or more. For children and adolescents with immunodeficiency a shorter time frame is used. Children with dyspnoea and/or hypoxia for any duration should be considered as having a severe exacerbation, and they require immediate treatment.[10]

The European Respiratory Society (ERS) recommends all adult patients should be offered eradication antibiotic therapy on a first or new detection of P aeruginosa although it notes that this recommendation is based on very low-quality evidence.[79]

The British Thoracic Society (BTS) also recommends eradication antibiotic treatment in adult patients with bronchiectasis associated with clinical deterioration and a new growth of P aeruginosa. If a new growth of P aeruginosa is detected in the context of stable bronchiectasis, then the BTS guideline recommends discussing the risks and benefits of eradication treatment with the patient, compared with clinical observation alone.[46] 

There is some evidence that including a nebulised antibiotic in eradication treatment for P aeruginosa is more efficacious than intravenous treatment alone.[79] 

For adult patients with a first or new isolation of P aeruginosa, the ERS outlines some commonly used treatment approaches, but notes that there is no clear evidence to support one regimen over another. The ERS outlines three suggested eradication regimens, which are all for a total duration of 3 months: (1) an oral fluoroquinolone (such as ciprofloxacin) for an initial 2-week period followed by intravenous antibiotics (e.g., a beta-lactam plus an aminoglycoside), followed by inhaled antibiotics (e.g., colistimethate, tobramycin, or gentamicin); (2) intravenous antibiotics (e.g., a beta-lactam plus an aminoglycoside) for an initial 2-week period, followed by inhaled antibiotics (e.g., colistimethate, tobramycin, or gentamicin); or (3) a 2-week initial phase of oral fluoroquinolone or intravenous antibiotics, plus inhaled antibiotics (e.g., ciprofloxacin plus inhaled colistimethate) followed by continued inhaled antibiotics alone.[79] After each phase, the ERS guideline recommends repeating sputum sampling and only moving to the next step if the culture is positive for P aeruginosa.[79] 

Cefepime may be used for adult patients with known P aeruginosa resistant to fluoroquinolones. Other intravenous options for adult patients with P aeruginosa include ceftazidime, piperacillin/tazobactam, aztreonam, and meropenem. Combination therapy may be needed in certain patients with known P aeruginosa, and advice should be sought from an infectious disease specialist regarding selection of a suitable regimen.

The BTS guideline on bronchiectasis in adults recommends oral ciprofloxacin for 2 weeks as first-line treatment. As second-line treatment, the guideline recommends an intravenous antipseudomonal beta-lactam antibiotic, with or without an intravenous aminoglycoside, for 2 weeks, followed by 3 months of nebulised colistimethate, gentamicin, or tobramycin.[46] 

For children and adolescents with a confirmed first or new isolation of P aeruginosa, the ERS recommends a stepwise treatment approach depending on whether the child is symptomatic. For asymptomatic children, oral ciprofloxacin and/or inhaled antibiotics for 2 weeks are recommended first, followed by inhaled antibiotics for 4-12 weeks (e.g., colistimethate, tobramycin). This should be followed by a repeat specimen from the child’s lower airway, if possible. If P aeruginosa is still present, or if the child becomes symptomatic, then the child should receive treatment as per symptomatic children.[10] 

For children with increased symptoms from baseline, intravenous antibiotics are recommended for 2 weeks (e.g., piperacillin/tazobactam or ceftazidime plus tobramycin) followed by inhaled antibiotics for 4-12 weeks (e.g., colistimethate, tobramycin).[10] Antibiotic choices will depend on patient factors, Pseudomonas susceptibility profile, and availability of antibiotics.[10] Inhaled antibiotics should be followed by a repeat specimen from the child’s lower airway, if possible. If P aeruginosa is still present, clinicians should consider repeating intravenous antibiotics, followed by inhaled antibiotics, at least once.[10]

Consult your local protocols for guidance on suitable eradication therapy regimens.

Plus – increased airway clearance

Back
ACUTE
|
acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics
|
first or new isolation of Pseudomonas aeruginosa
Plus – 

increased airway clearance

Treatment recommended for ALL patients in selected patient group

Airway clearance to clear mucus, with or without bronchodilators, is important and should be increased in frequency for patients of any disease severity during the treatment of exacerbations.

Plus – continued maintenance therapy

Back
ACUTE
|
acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics
|
first or new isolation of Pseudomonas aeruginosa
Plus – 

continued maintenance therapy

Treatment recommended for ALL patients in selected patient group

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72] 

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at least 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

The British Thoracic Society (BTS) recommends self-management plans are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help.[46] 

Nebulised bronchodilators given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46] Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10] 

Use of nebulised hyperosmolar agents, such as hypertonic saline, promotes mucus clearance by inducing coughing. Nebulised hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[96][97] It may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[98] Bronchodilators should be used prior to administration of nebulised hyperosmolar agents. 

Guidelines from the BTS recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[46] 

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10] 

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105] 

1st line – short-term intravenous antibiotic

Back
ACUTE
|
acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics
|
Pseudomonas aeruginosa not isolated, or chronically infected with Pseudomonas aeruginosa
1st line – 

short-term intravenous antibiotic

An acute exacerbation typically presents as worsening of cough, change in sputum colour, increase in sputum volume, fever, and/or malaise. Severity of underlying disease in adults can be scored using the Bronchiectasis Severity Index (BSI).[46][109][110][111]

In children and adolescents, an acute exacerbation can be defined as increased respiratory symptoms (predominantly cough with or without increased sputum volume and/or purulence) for 3 days or more. For children and adolescents with immunodeficiency a shorter time frame is used. Children with dyspnoea and/or hypoxia for any duration should be considered as having a severe exacerbation, and they require immediate treatment.[10]

Antibiotics are the mainstay of treatment and should be selected for their activity against likely pathogens. Examples of potentially suitable regimens are listed above.

Patients with severe disease or a resistant organism (typically Pseudomonas) are likely to require intravenous antibiotics during acute exacerbations. In the context of P aeruginosa, this would be in a patient known to be chronically infected with P aeruginosa; a first or new isolation of P aeruginosa would prompt eradication therapy. Intravenous antibiotics should also be considered when patients are particularly unwell or have failed to respond to oral therapy, which is most likely in patients with P aeruginosa.[46]

An appropriate initial choice would be an antibiotic with coverage against prior culture results.

Cefepime may be used for adult patients with known P aeruginosa resistant to fluoroquinolones. Other intravenous options for adult patients with P aeruginosa include ceftazidime, piperacillin/tazobactam, aztreonam, and meropenem. Combination therapy may be needed in certain patients with known P aeruginosa, and advice should be sought from an infectious disease specialist regarding selection of a suitable regimen.

Vancomycin and linezolid are appropriate for adult patients with methicillin-resistant Staphylococcus aureus (MRSA). Gentamicin, if used, should be used cautiously with close monitoring of renal function and serum levels. Vancomycin also requires monitoring of serum levels.

Intravenous ceftriaxone is an appropriate second-line treatment for adult patients with Haemophilus influenzae, and those with coliforms such as Klebsiella and Enterobacter.

Children and adolescents with a severe exacerbation and/or who are not responding to oral antibiotics are likely to require intravenous antibiotics.[10] As with adults, the antibiotic should be chosen according to airway cultures and previous hypersensitivity reactions.[10] Ceftriaxone or cefotaxime may be suitable options.

If a patient is already taking long-term antibiotics, but experiences a further exacerbation, then the author of this topic recommends continuing the maintenance antibiotic unless there are drug-drug interactions that preclude co-administration, or if there are substantial changes in antibiotic susceptibility.

Treatment course: 14 days.

Primary options

Adults

cefepime: 2 g intravenously every 12 hours

OR

Adults

vancomycin: 1 g intravenously every 8-12 hours

OR

Adults

gentamicin: 3-6 mg/kg/day intravenously given in divided doses every 8 hours

OR

Adults

ceftriaxone: 2 g intravenously every 24 hours

OR

Adults

ceftazidime: 2 g intravenously every 8 hours

OR

Adults

piperacillin/tazobactam: 4.5 g intravenously every 6-8 hours

More

OR

Adults

aztreonam: 2 g intravenously every 8 hours

OR

Adults

meropenem: 2 g intravenously every 8 hours

OR

Adults

linezolid: 600 mg intravenously every 12 hours

OR

Children

ceftriaxone: 50-100 mg/kg/day intravenously given in divided doses every 12-24 hours, maximum 4 g/day

OR

Children

cefotaxime: 75-200 mg/kg/day intravenously given in divided doses every 6-8 hours, maximum 12 g/day

Plus – increased airway clearance

Back
ACUTE
|
acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics
|
Pseudomonas aeruginosa not isolated, or chronically infected with Pseudomonas aeruginosa
Plus – 

increased airway clearance

Treatment recommended for ALL patients in selected patient group

Airway clearance to clear mucus, with or without bronchodilators, is important and should be increased in frequency for patients of any disease severity during the treatment of exacerbations.

Plus – continued maintenance therapy

Back
ACUTE
|
acute exacerbation: severe underlying disease or not responding/resistant to initial antibiotics
|
Pseudomonas aeruginosa not isolated, or chronically infected with Pseudomonas aeruginosa
Plus – 

continued maintenance therapy

Treatment recommended for ALL patients in selected patient group

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72] 

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at least 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

The British Thoracic Society (BTS) recommends self-management plans are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help.[46] 

Nebulised bronchodilators given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46] Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10] 

Use of nebulised hyperosmolar agents, such as hypertonic saline, promotes mucus clearance by inducing coughing. Nebulised hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[96][97] It may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[98] Bronchodilators should be used prior to administration of nebulised hyperosmolar agents. 

Guidelines from the BTS recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[46] 

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10] 

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105] 

3 or more exacerbations per year despite maintenance therapy

1st line – reassess physiotherapy ± mucoactive treatment

Back
ACUTE
|
3 or more exacerbations per year despite maintenance therapy
1st line – 

reassess physiotherapy ± mucoactive treatment

Guidelines from the British Thoracic Society (BTS) include recommendations on the stepwise management of bronchiectasis in adults. If the patient experiences 3 or more exacerbations per year despite maintenance treatment (e.g., treatment of any underlying cause, airway clearance techniques, pulmonary rehabilitation, vaccination), then the patient should have their physiotherapy reassessed and mucoactive treatment should be considered. Mucoactive treatment could consist of humidification with sterile water or normal saline to facilitate airway clearance.[46]

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10]

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105]

Plus – continued maintenance therapy

Back
ACUTE
|
3 or more exacerbations per year despite maintenance therapy
Plus – 

continued maintenance therapy

Treatment recommended for ALL patients in selected patient group

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72] 

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at least 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

The British Thoracic Society recommends self-management plans are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help.[46] 

Nebulised bronchodilators given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46] Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10] 

Consider – long-term antibiotic

Back
ACUTE
|
3 or more exacerbations per year despite maintenance therapy
Consider – 

long-term antibiotic

Additional treatment recommended for SOME patients in selected patient group

Guidelines on bronchiectasis from the British Thoracic Society (BTS) include recommendations on the use of inhaled and long-term antibiotics in adults. If the patient experiences 3 or more exacerbations per year despite maintenance treatment (e.g., treatment of any underlying cause, airway clearance techniques, pulmonary rehabilitation, vaccination), and despite reassessment of physiotherapy, with or without mucoactive treatment, then the BTS guideline recommends the following: (1) if P aeruginosa infection is present, then a long-term inhaled antipseudomonal antibiotic or a long-term macrolide is recommended; if other potentially pathogenic micro-organisms are present, then a long-term macrolide or long-term oral or inhaled targeted antibiotic is recommended; if no pathogens are isolated, then a long-term macrolide is recommended; consult an infectious disease specialist for guidance on targeted antibiotic selection and dose; (2) if the patient is still experiencing 3 or more exacerbations per year despite the above treatment, then the BTS bronchiectasis guideline recommends a long-term macrolide plus a long-term inhaled antibiotic; (3) if the patient is still experiencing 5 or more exacerbations per year despite long-term macrolide plus long-term inhaled antibiotic treatment, then the BTS guideline recommends considering regular intravenous antibiotics every 2 to 3 months; consult an infectious disease specialist for guidance on intravenous antibiotic selection and dose.[46]

The BTS guideline for long-term macrolides in adults recommends that patients who have been offered macrolides to reduce exacerbation rates should have their treatment continued for a minimum of 6 months. Macrolides can also be considered to improve quality of life but may require a long course (e.g., 1 year) before a significant clinical response is seen.[82] 

The European Respiratory Society (ERS) makes similar recommendations about offering long-term (≥3 months) antibiotic treatment in adults with bronchiectasis. However, it does not make a recommendation about regular intravenous antibiotics.[79]

For children and adolescents, the ERS recommends at least 6 months of macrolide antibiotics for non-cystic fibrosis bronchiectasis and recurrent exacerbations (>1 hospitalised or ≥3 non-hospitalised exacerbations in the previous 12 months). Patients should be monitored to ensure that the antibiotics remain clinically beneficial.[10] An ECG is not necessary in all children/adolescents but a detailed cardiac history should be taken prior to starting therapy. Macrolides should not be used in children/adolescents with contraindications to macrolides (e.g., abnormal ECG, abnormal liver function tests, azithromycin hypersensitivity).[10]

There are concerns regarding the increased risk of emerging drug resistance with prolonged antibiotics.[10][80][81] [ Cochrane Clinical Answers logo ] An option to reduce the risk of developing resistance is to stop long-term macrolides for a period of time each year, such as over the summer.[82]

Presence of mycobacteria in the sputum necessitates prompt discontinuation of macrolide monotherapy to minimise the risk of resistance developing. Macrolides are associated with increased risk of cardiovascular death and other serious adverse events in people who do not have bronchiectasis, and the available data cannot exclude a similar risk in patients with bronchiectasis.[83] Use caution when prescribing macrolides in patients with a history of cardiac disease or other conditions that may increase the risk of QT interval prolongation (this includes patients receiving other drugs that prolong the QT interval or cause electrolyte imbalances). The BTS guideline for long-term macrolides in adults recommends an ECG to assess the QTc interval prior to starting therapy and again after 1 month.[82] Adults should also have liver function tests at baseline, 1 month after starting macrolides, and thereafter every 6 months while on therapy.[82] The dose, specific macrolide, and duration of therapy (months to indefinitely) are not completely established. Doxycycline can be considered as an alternative if patients are intolerant to macrolides, or if they are ineffective.[46] Amoxicillin and amoxicillin/clavulanate are also alternative options.[46]

The long-term use of fluoroquinolones in the treatment of respiratory infections in patients with bronchiectasis may mask active pulmonary tuberculosis. Vigilant mycobacterial surveillance is important in this patient population.[10][89]

In November 2018, the European Medicines Agency (EMA) completed a review of serious, disabling, and potentially irreversible adverse effects associated with systemic and inhaled fluoroquinolone antibiotics. These adverse effects include tendonitis, tendon rupture, arthralgia, neuropathies, and other musculoskeletal or nervous system effects. As a consequence of their review, the EMA now recommends that fluoroquinolone antibiotics be restricted for use in serious, life-threatening bacterial infections only.[90] The US Food and Drug Administration has also issued warnings about the increased risk of aortic dissection, significant hypoglycaemia, and mental health adverse effects in patients taking fluoroquinolones.[91][92]

Inhaled antibiotics (e.g., gentamicin, tobramycin, colistimethate) for at least 1 month are well tolerated and may significantly reduce bacterial load and decrease exacerbation frequency in adult patients with bronchiectasis. They are associated with the emergence of bacterial resistance.[93][94][95] 

Sensitivity must be confirmed prior to initiation of inhaled antibiotics. Bronchodilators should be used prior to administration of inhaled antibiotics.

Primary options

Children and adults

azithromycin: children: consult specialist for guidance on dose; adults: 250-500 mg orally three times weekly, or 250 mg orally once daily

OR

Children and adults

erythromycin base: children: consult specialist for guidance on dose; adults: 250 mg orally twice daily

OR

Adults

tobramycin inhaled: adults: 300 mg inhaled via nebuliser twice daily; give in cycles of 28 days on and then 28 days off

OR

Adults

gentamicin: adults: 80 mg inhaled via nebuliser twice daily

OR

Adults

colistimethate sodium: adults: 75 mg inhaled via nebuliser every 12 hours

More

OR

Adults

doxycycline: 100 mg orally once daily

OR

Adults

amoxicillin: 250 mg orally twice daily

OR

Adults

amoxicillin/clavulanate: 250 mg orally twice daily

More

Secondary options

Adults

tobramycin inhaled: 300 mg inhaled via nebuliser twice daily; give in cycles of 28 days on and then 28 days off

or

gentamicin: 80 mg inhaled via nebuliser twice daily

or

colistimethate sodium: 75 mg inhaled via nebuliser every 12 hours

More

-- AND --

azithromycin: 250 mg orally three times weekly

or

erythromycin base: 250 mg orally twice daily

Consider – surgery

Back
ACUTE
|
3 or more exacerbations per year despite maintenance therapy
Consider – 

surgery

Additional treatment recommended for SOME patients in selected patient group

Surgical resection is considered in patients with localised disease whose symptoms are not controlled by optimal medical treatment.[46]

It is appropriate for patients with severe focal disease in one or both lobes of a lung and with limited success with antibiotic therapy. Complete resection of the bronchiectatic area is associated with the best results. Advanced age and renal failure are associated with increased post-operative complications.[112][113]

Surgery may be indicated for massive haemoptysis and, possibly, in the treatment of focal non-tuberculous mycobacteria (NTM) or Aspergillus species.

Referral for lung transplantation should be considered in patients with bronchiectasis aged 65 years or younger if their forced expiratory volume in the first second of expiration (FEV₁) is <30% and they have significant clinical instability or if they have a rapidly progressive respiratory deterioration despite optimal medical management.[46]

Earlier transplant referral should be considered in bronchiectasis patients with poor lung function and the following additional factors: massive haemoptysis, severe secondary pulmonary hypertension, intensive care admissions, or respiratory failure (particularly if requiring non-invasive ventilation).[46]

Surgery is rarely carried out in children/adolescents with bronchiectasis and is only considered when maximal medical therapy has failed and the patient’s quality of life is severely compromised.[10] As with adults, benefits are greater when disease is localised and not due to pathology that is likely to recur (e.g., immunodeficiency).

Consider – treatment of respiratory failure

Back
ACUTE
|
3 or more exacerbations per year despite maintenance therapy
Consider – 

treatment of respiratory failure

Additional treatment recommended for SOME patients in selected patient group

The evidence regarding administration of non-invasive ventilation is poor, but there may be some benefit in patients with severe ventilatory failure. Domiciliary non-invasive ventilation with humidification should be considered for patients with bronchiectasis and respiratory failure associated with hypercapnia, especially where this is associated with symptoms or recurrent hospitalisation. Long-term oxygen therapy should be considered for patients with bronchiectasis and respiratory failure, using the same eligibility criteria as for COPD.[46] Oxygen saturation must be carefully monitored to prevent hypercapnic respiratory failure.

ONGOING

first or new isolation of Pseudomonas aeruginosa at outpatient review

1st line – antibiotic eradication therapy

Back
ONGOING
|
first or new isolation of Pseudomonas aeruginosa at outpatient review
1st line – 

antibiotic eradication therapy

The European Respiratory Society (ERS) recommends all adult patients should be offered eradication antibiotic therapy on a first or new detection of P aeruginosa, although it notes that this recommendation is based on very low-quality evidence.[79]

The British Thoracic Society (BTS) also recommends eradication antibiotic treatment in adult patients with bronchiectasis associated with clinical deterioration and a new growth of P aeruginosa. If a new growth of P aeruginosa is detected in the context of stable bronchiectasis, the BTS guideline recommends discussing the risks and benefits of eradication treatment with the patient, compared with clinical observation alone.[46] 

There is some evidence that including a nebulised antibiotic in eradication treatment for P aeruginosa is more efficacious than intravenous treatment alone.[79]

For adult patients with a first or new isolation of P aeruginosa, the ERS outlines some commonly used treatment approaches, but notes that there is no clear evidence to support one regimen over another. The ERS outlines three suggested eradication regimens, which are all for a total duration of 3 months: (1) an oral fluoroquinolone (such as ciprofloxacin) for an initial 2-week period followed by intravenous antibiotics (e.g., a beta-lactam plus an aminoglycoside), followed by inhaled antibiotics (e.g., colistimethate, tobramycin, or gentamicin); (2) intravenous antibiotics (e.g., a beta-lactam plus an aminoglycoside) for an initial 2-week period, followed by inhaled antibiotics (e.g., colistimethate, tobramycin, or gentamicin); or (3) a 2-week initial phase of oral fluoroquinolone or intravenous antibiotics, plus inhaled antibiotics (e.g., ciprofloxacin plus inhaled colistimethate) followed by continued inhaled antibiotics alone.[79] After each phase, the ERS guideline recommends repeating sputum sampling and only moving to the next step if the culture is positive for P aeruginosa.[79]

Cefepime may be used for adult patients with known P aeruginosa resistant to fluoroquinolones. Other intravenous options for adult patients with P aeruginosa include ceftazidime, piperacillin/tazobactam, aztreonam, and meropenem. Combination therapy may be needed in certain patients with known P aeruginosa, and advice should be sought from an infectious disease specialist regarding selection of a suitable regimen.

The BTS guideline on bronchiectasis in adults recommends oral ciprofloxacin for 2 weeks as first-line treatment. As second-line treatment, the guideline recommends an intravenous antipseudomonal beta-lactam antibiotic, with or without an intravenous aminoglycoside, for 2 weeks, followed by 3 months of nebulised colistimethate, gentamicin, or tobramycin.[46] 

For children and adolescents with a confirmed first or new isolation of P aeruginosa, the ERS recommends a stepwise treatment approach depending on whether the child is symptomatic. For asymptomatic children, oral ciprofloxacin and/or inhaled antibiotics for 2 weeks are recommended first, followed by inhaled antibiotics for 4-12 weeks (e.g., colistimethate, tobramycin). This should be followed by a repeat specimen from the child’s lower airway, if possible. If P aeruginosa is still present, or if the child becomes symptomatic, then the child should receive treatment as per symptomatic children.[10] 

For children with increased symptoms from baseline, intravenous antibiotics are recommended for 2 weeks (e.g., piperacillin/tazobactam or ceftazidime plus tobramycin) followed by inhaled antibiotics for 4-12 weeks (e.g., colistimethate, tobramycin).[10] Antibiotic choices will depend on patient factors, Pseudomonas susceptibility profile, and availability of antibiotics.[10] Inhaled antibiotics should be followed by a repeat specimen from the child’s lower airway, if possible. If P aeruginosa is still present, clinicians should consider repeating intravenous antibiotics, followed by inhaled antibiotics, at least once.[10]

Consult your local protocols for guidance on suitable eradication therapy regimens.

Plus – continued maintenance therapy

Back
ONGOING
|
first or new isolation of Pseudomonas aeruginosa at outpatient review
Plus – 

continued maintenance therapy

Treatment recommended for ALL patients in selected patient group

A healthy diet and exercise are recommended for all patients, including vitamin D supplementation.[10][71] A higher body mass index has been shown to correlate with a beneficial outcome in adults.[72] 

Exercise is considered a form of airway clearance. One Cochrane systematic review found that adult patients with stable bronchiectasis had improved exercise capacity and quality of life immediately after exercise training lasting at least 4 weeks, but found limited benefits on cough-related quality-of-life and psychological symptoms.[73] There was insufficient evidence to show any longer-term benefits, although the frequency of exacerbations at 1 year was reduced with exercise training in one study.[74] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[73] Exercise training is often offered as part of a pulmonary rehabilitation programme, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. In children and adolescents, evidence for formal exercise programmes is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[10]

The British Thoracic Society (BTS) recommends self-management plans are considered in all bronchiectasis patients and provides a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognising exacerbations, and when and how to seek medical help.[46] 

Nebulised bronchodilators given before therapy with mucoactive agents may improve tolerability, especially in patients with concurrent asthma or COPD, although the evidence for their use is weak. Treatment with bronchodilators in patients with bronchiectasis and co-existing COPD or asthma should follow guideline recommendations for COPD or asthma.[10][46] Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[10] 

Use of nebulised hyperosmolar agents, such as hypertonic saline, promotes mucus clearance by inducing coughing. Nebulised hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[96][97] It may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[98] Bronchodilators should be used prior to administration of nebulised hyperosmolar agents. 

Guidelines from the BTS recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[46] 

In children and adolescents with bronchiectasis, the routine use of mucoactive agents is not recommended. This includes recombinant human deoxyribonuclease (rhDNase), bromhexine, mannitol, and hypertonic saline. In selected patients with more severe disease, inhaled mannitol or hypertonic saline may be considered, with the first dose taken under medical supervision. If tolerated, the use of mannitol or hypertonic saline may improve quality of life and increase expectoration. A short-acting bronchodilator should be used prior to inhaling mannitol or hypertonic saline.[10] 

Guidelines from the European Respiratory Society suggest offering long-term mucoactive treatment (≥3 months) to adults with bronchiectasis who have difficulty in expectorating sputum and poor quality of life, where symptoms are not controlled by standard airway clearance techniques.[79] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[46] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pre-treatment with a bronchodilator before inhaled or nebulised mucoactive treatments, particularly where bronchoconstriction is likely.[46] 

Although rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine, carbocisteine, and bromhexine.[46][103][104][105] 

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