Approach
Treatment for all patients involves maintenance therapy and treatment of acute exacerbations, with attention to the specific organism involved. For a select group of patients, surgery may be indicated. Patients with severe disease or a resistant organism (typically Pseudomonas) are likely to require intravenous antibiotics during acute exacerbations. For severe, progressive disease lung transplantation should be considered.
The goal of treatment is to decrease baseline clinical symptoms of cough, sputum production, and dyspnea, to improve quality of life, to decrease the number of acute exacerbations, and to prevent progression of airway damage.[67] The particular organism present in the sputum, specifically Pseudomonas, and the frequency of exacerbations determine antibiotic treatment choices. Those with low risk of developing a Pseudomonas infection have few, if any, exacerbations and may require less maintenance therapy.
Although the treatment of children and adolescents with bronchiectasis is broadly similar to that of adults, there are several important differences. Early treatment is particularly important to harness the potential to reverse lung damage and therefore prevent progression to adult bronchiectasis. Bacterial pathogens in children vary, as do their natural lung microbiome, immunologic responses, and treatment outcomes. Therapies need to be tailored to be developmentally appropriate (e.g., in the case of airway-clearance techniques). Children require ongoing support and supervision from their parents or caregivers, who need to be involved in treatment discussions and decisions and taught how to use medications and equipment.[9]
The number of studies assessing treatments for noncystic fibrosis (non-CF) bronchiectasis is increasing, therefore evidence for specific treatments in this area is building. More randomized controlled trials are needed with an endpoint focussed on reducing exacerbations and improving quality of life, rather than reducing sputum volume. Additionally, treatment of any identified underlying disease (e.g., CF) or comorbid disease (e.g., gastroesophageal reflux disease) is paramount. For detailed information on the treatment of these conditions, see Cystic fibrosis, Gastroesophageal reflux disease, and Disorders of infant feeding.
Maintenance therapy for all patients
Airway clearance is impaired in patients with bronchiectasis, and bronchial hygiene to loosen secretions and enhance their removal is the mainstay of maintenance therapy.[45][68] A systematic review of airway clearance techniques in people with non-CF bronchiectasis concluded that airway clearance techniques seem to be safe in people with stable bronchiectasis. They may be associated with improvements in sputum expectoration, selected measures of lung function, symptoms, and health-related quality-of-life scores. However, it is not clear what role they play in the management of acute bronchiectasis.[69]
Airway clearance therapy
Maintenance of oral hydration helps to improve mucociliary clearance.
Techniques include percussion, breathing, or coughing strategies (including active cycle of breathing, directed coughing, and autogenic drainage); positioning and postural drainage; positive expiratory pressure (PEP) devices; and vibration with the use of oscillatory devices such as the flutter valve and vibration vest. These techniques may be used alone or in combination.[69] British Thoracic Society guidelines recommend that adults with bronchiectasis are offered active cycle of breathing techniques or oscillating PEP, with gravity-assisted positioning to enhance their effectiveness.[45] However, there is insufficient evidence that any airway clearance technique is better than any other.[68][70][71] 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.[72] Regular airway clearance is also recommended for children/adolescents. It should be individualized according to their age and developmental stage and reviewed at least biannually by a respiratory physical therapist with pediatric expertise. Children with asthma-type responses may benefit from using a short-acting bronchodilator prior to airway clearance therapy.[9]
Airway clearance is generally recommended for 15 to 30 minutes, 2 or 3 times daily. Many of these interventions require the assistance of a caregiver. The therapy is time-consuming, and patients often find the process unpleasant.[68] Patient preference should weigh heavily in which technique is chosen. Data from a US bronchiectasis and nontuberculosis mycobacteria patient registry show 59% of patients used airway clearance techniques when clinically stable and these patients were more likely to be those with severe disease. One year later, over half of the patients who used airway clearance techniques at baseline were no longer using them.[73]
Diet and exercise
A healthy diet and exercise are recommended in all patients, including vitamin D supplementation.[9][74] A higher body mass index has been shown to correlate with beneficial outcome in adult patients.[75]
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.[76] 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.[77] Patients who participated in exercise training soon after an exacerbation did not show any benefits.[76] Exercise training is often offered as part of a pulmonary rehabilitation program, combined with patient education and training in self-management, and delivered on an outpatient basis or remotely via telerehabilitation. A systematic review of telerehabilitation in chronic respiratory diseases found similar outcomes to traditional pulmonary rehabilitation with no safety issues identified; however, the studies were predominately in COPD patients.[78]
In children and adolescents, evidence for formal exercise programs is lacking, and it is recommended that exercise is encouraged on an ongoing basis as part of an active lifestyle.[9]
Self-management plans
Self-management plans aim to increase patients’ confidence in managing their own condition. The British Thoracic Society recommends they are considered in all people with bronchiectasis and has published a template action plan that provides patients with information on maintenance therapy, monitoring their symptoms, recognizing 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.[45] 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.[79]
Maintenance therapy for select patients
Select patients may benefit from any or all of the following:
Early eradication of Pseudomonas aeruginosa
Long-term antibiotics for chronic bacterial infection
Mucoactive agents
Inhaled corticosteroids
Bronchodilators.
Early eradication of P aeruginosa
Repeated exacerbations, a recent history of antibiotic use, and CF are risk factors for P aeruginosa infection. Case records suggest that early, aggressive antibiotic therapy may eradicate P aeruginosa infection in some patients with bronchiectasis, but this is yet to be confirmed directly in a prospective, randomized controlled trial.
Two studies have investigated whether eradication treatment in adults with bronchiectasis can improve clinical outcomes versus the patient’s baseline.[80][81] A retrospective observational study compared different eradication regimens in 30 patients with bronchiectasis not due to CF: intravenous antibiotics (12 patients), intravenous antibiotics followed by oral ciprofloxacin (13 patients), or oral ciprofloxacin alone (5 patients); 25 of the 30 patients then received nebulized colistin for 3 months. Initially, P aeruginosa was eradicated from sputum in 80% of patients, although this reduced to 54% at follow-up (median time 6.2 months). After eradication treatment, the rate of exacerbations fell from 3.93 per year to 2.09 per year; 20 out of 30 patients reported a clinical improvement, although lung function was not changed.[80]
Another study compared different eradication regimens in a 15-month, single-masked, randomized study involving 35 patients with bronchiectasis not due to CF. Following the first isolation of P aeruginosa in sputum, patients received 14 days of intravenous ceftazidime and tobramycin. Patients then received 3 months of either nebulized tobramycin twice a day or placebo. In the first month, 91% of patients in the tobramycin group were free of P aeruginosa, while this proportion was 77% in the placebo group. After 12 months of follow-up, these figures were 55% and 29%, respectively. The study also showed that the median time to recurrence of P aeruginosa was higher in the tobramycin group compared with the placebo group, and that the number of exacerbations and hospital admissions were lower in the tobramycin group compared with the placebo group.[81]
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 that is extrapolated from the two studies described above.[65]
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.[45]
There is some evidence that including a nebulized antibiotic in eradication treatment for P aeruginosa is more efficacious than intravenous treatment alone.[65]
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:[65]
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),
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
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.
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.[65]
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 nebulized colistimethate, gentamicin, or tobramycin.[45]
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.[9]
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).[9]
Antibiotic choices will depend on patient factors, Pseudomonas susceptibility profile, and availability of antibiotics.[9] 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.[9]
Guidelines from the BTS also recommend offering eradication treatment to adult patients with bronchiectasis who have a clinical deterioration and a new growth of methicillin-resistant Staphylococcus aureus (MRSA).[45] However, guidelines from the ERS suggest not offering eradication treatment to adult patients with bronchiectasis following new isolation of pathogenic microorganisms other than P aeruginosa.[65]
Long-term antibiotics
Most patients with bronchiectasis are chronically infected with bacteria, even during the stable state, and their bacterial load correlates with markers of inflammation. Prolonged use of antibiotics (4 weeks or more) has been associated with a reduced risk of exacerbations and hospitalizations in patients with bronchiectasis, although there are concerns regarding the increased risk of emerging drug resistance.[9][82][83]
[ 
]
Guidelines on bronchiectasis from the 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 physical therapy, with or without mucoactive treatment, then the BTS guideline recommends the following:[45]
If P aeruginosa infection is present, then a long-term inhaled antipseudomonal antibiotic or a long-term macrolide is recommended. If other potentially pathogenic microorganisms are present, then a long-term macrolide or a long-term oral or inhaled targeted antibiotic is recommended. If no pathogens are isolated, then a long-term macrolide is recommended.
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.
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 2-3 months.
The BTS guideline for long-term macrolides in adults recommends that patients with 3 or more exacerbations per year 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.[84]
The 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.[65]
For children and adolescents, the ERS recommends at least 6 months of macrolide antibiotics for non-CF bronchiectasis and recurrent exacerbations (>1 hospitalized or ≥3 nonhospitalized exacerbations in the previous 12 months). Patients should be monitored to ensure that the antibiotics remain clinically beneficial.[9] 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).[9]
The results of several studies suggest that oral macrolide antibiotics are of therapeutic benefit in bronchiectasis patients with or without Pseudomonas.[85][86][87]
[ ]
Benefits include a small improvement in forced expiratory volume in the first second of expiration (FEV₁), a decrease in sputum volume, and a decreased exacerbation rate. However, vigilance is required.[88]
An individual patient data meta-analysis of long-term macrolide treatment in adult patients with bronchiectasis concluded that macrolides led to a clinically significant improvement in all patients, including those for whom macrolides are not currently recommended as first-line treatment, such as patients with fewer than 3 exacerbations per year, or those with Pseudomonas infection.[87]
Long-term treatment with azithromycin was associated with a significantly lower exacerbation rate compared with erythromycin in an adjusted indirect treatment comparison of macrolides in non-CF bronchiectasis in adults.[89]
An option to reduce the risk of developing antibiotic resistance is to stop long-term macrolides for a period of time each year, such as over the summer.[84]
A Cochrane review evaluating intermittent prophylactic antibiotics in bronchiectasis found that, in adults who have frequent chest infections, long‐term antibiotic treatment given at 14‐day on/off intervals slightly reduces the frequency of chest infections, with little to no difference in serious adverse events. However, antibiotic resistance was increased.[90]
Presence of mycobacteria in the sputum necessitates prompt discontinuation of macrolide monotherapy to minimize 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.[85] 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.[84] Adults should also have liver function tests at baseline, 1 month after starting macrolides, and thereafter every 6 months while on therapy.[84] 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.[45] Amoxicillin and amoxicillin/clavulanate are also alternative options.[45]
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.[9][91]
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.[92] The Food and Drug Administration has also issued warnings about the increased risk of aortic dissection, significant hypoglycemia, and mental health adverse effects in patients taking fluoroquinolones.[93][94]
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.[95][96] They are associated with the emergence of bacterial resistance.[97]
Sensitivity must be confirmed prior to initiation of inhaled antibiotics. Bronchodilators should be used prior to administration of inhaled antibiotics.
Mucoactive agents
Mucoactive agents refer to medications that may act directly on the clearance of mucus from the airways. The medications can be categorized by their mode of action, including expectorants (which may aid and/or induce cough), mucolytics (which thin mucus), mucokinetics (which increase mucociliary clearance), and mucoregulators (which suppress the mechanisms that underlie chronic hypersecretion of mucus).[45]
Use of nebulized hyperosmolar agents, such as hypertonic saline, promotes mucus clearance by inducing coughing (i.e., they are expectorants). Nebulized hypertonic saline has been shown to reduce inflammatory mediators, improve sputum bacteriology, and improve quality-of-life scores.[98][99] Hypertonic saline may cause chest tightness and wheezing in some patients. Addition of hyaluronic acid may improve tolerability.[100] Bronchodilators should be used prior to administration of nebulized hyperosmolar agents.
Mannitol is of uncertain clinical benefit.[101][102] It failed to reduce exacerbation rates in a large, randomized controlled trial.[103][104]
[ ]
Guidelines from the BTS recommend considering the use of humidification with sterile water or normal saline to facilitate airway clearance in adults with bronchiectasis.[45]
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.[9]
Guidelines from the ERS 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.[65] Guidelines from the BTS suggest considering a trial of mucoactive treatment in adults with bronchiectasis who have difficulty with sputum expectoration.[45] The BTS guidelines also suggest performing an airway reactivity challenge test when inhaled mucoactive treatment is first given, and considering pretreatment with a bronchodilator before inhaled or nebulized mucoactive treatments, particularly where bronchoconstriction is likely.[45]
rhDNase, which is a mucolytic, is not recommended in patients with bronchiectasis as it increases frequency of exacerbations in bronchiectasis.[45][65] However, other mucolytic agents may be beneficial in a subset of adult patients. These include acetylcysteine, erdosteine (not available in the US), carbocisteine (not available in the US), and bromhexine.[45][105][106][107]
Inhaled corticosteroids
Inhaled corticosteroids have been shown to decrease 24-hour sputum volume and to lessen exacerbations.[108] Large and long-term supportive studies, however, are lacking, and inhaled corticosteroids are not routinely recommended in patients without other indications.[9][45][65][109] There is also concern that inhaled corticosteroids may increase the risk of infection in bronchiectasis, particularly with nontuberculous mycobacterial organisms.[110] However, oral corticosteroids are recommended by the BTS in patients with bronchiectasis and active allergic bronchopulmonary aspergillosis.[45] The ERS suggests that inhaled corticosteroids may be beneficial in children and adolescents with bronchiectasis and eosinophilic airway inflammation.[9]
Bronchodilators
Nebulized bronchodilators (e.g., albuterol) 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 coexisting COPD or asthma should follow guideline recommendations for COPD or asthma.[9][45]
Scoring of disease severity
The Bronchiectasis Severity Index (BSI) is a clinical scoring system, validated in the UK and Europe, that may be used to stratify severity of chronic disease into mild, moderate, or severe in adult patients. This may help with understanding prognosis and guiding treatment. Variables taken into account by the BSI include age, body mass index, FEV₁ % predicted, hospital admission within the past 2 years, number of exacerbations in the previous 12 months, Medical Research Council breathlessness score, Pseudomonas colonization, colonization with other organisms, and radiologic severity. Another scoring system, FACED, is also available, but it does not take exacerbations into account, so the BSI remains the preferred scoring system.[45][111][112][113]
Treatment of exacerbations
An acute exacerbation typically presents as worsening of cough, change in sputum color, increase in sputum volume, fever, and/or malaise. 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 dyspnea and/or hypoxia for any duration should be considered as having a severe exacerbation, and they require immediate treatment.[9]
Antibiotics are the mainstay of treatment and should be selected for their activity against likely pathogens. Where possible, antibiotic choice should be based on culture and sensitivity from sputum samples.[45] Testing for viral infection should be considered in patients with exacerbations, as viral infections are also implicated in exacerbations.[45]
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 S aureus, depending on the culture results.[45]
For adults with mild to moderate underlying disease and a low likelihood of P aeruginosa, oral antibiotic treatment is appropriate. In patients with mild to moderate underlying disease and a known (but not first or new) 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.
The BTS 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 MRSA, first-line oral options include doxycycline, rifampin, 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.[45]
For those patients suitable for outpatient care, oral antibiotic treatment (e.g., amoxicillin, clarithromycin, or trimethoprim/sulfamethoxazole) is appropriate for 14 days. For children and adolescents, the empiric antibiotic of choice is oral amoxicillin/clavulanate but, as with adults, the antibiotic should be chosen according to airway cultures and previous hypersensitivity reactions.[9]
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.[45] 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 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 H 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.[9] As with adults, the antibiotic should be chosen according to airway cultures and previous hypersensitivity reactions.[9]
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. Concurrent treatment of the underlying infection is essential. There is no evidence to support the use of systemic corticosteroids in the treatment of acute exacerbations in either mild or severe disease with or without Pseudomonas species.
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 hypoglycemia, mental health adverse effects, and other musculoskeletal or nervous system effects.[92][93][94]
Treatment of refractory disease
Surgical resection is considered in patients with localized disease whose symptoms are not controlled by optimal medical treatment:[45]
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 postoperative complications.[114][115]
Surgery may be indicated for massive hemoptysis and, possibly, in the treatment of focal nontuberculous mycobacteria (NTM) or Aspergillus species.
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.[9] As with adults, benefits are greater when disease is localized and not due to pathology that is likely to recur (e.g., immunodeficiency).
Treatment of respiratory failure
The evidence regarding administration of noninvasive ventilation is poor, but there may be some benefit in patients with severe ventilatory failure. Domiciliary noninvasive 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 hospitalization. Long-term oxygen therapy should be considered for patients with bronchiectasis and respiratory failure, using the same eligibility criteria as for COPD.[45] Oxygen saturation must be carefully monitored to prevent hypercapnic respiratory failure.
Lung transplantation
Referral for lung transplantation should be considered in patients with bronchiectasis aged 65 years or younger if their FEV₁ is <30% and they have significant clinical instability or if they have a rapidly progressive respiratory deterioration despite optimal medical management.[45]
Earlier transplant referral should be considered in bronchiectasis patients with poor lung function and the following additional factors: massive hemoptysis, severe secondary pulmonary hypertension, intensive care admissions, or respiratory failure (particularly if requiring noninvasive ventilation).[45]
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