Approach Considerations
The goals of therapy are to improve symptoms, to reduce complications, to control exacerbations, and to reduce morbidity and mortality. Early recognition is essential in bronchiectasis and associated conditions. Additionally, management of underlying conditions, which may include the use of intravenous immunoglobulin or intravenous alpha1-antitrypsin (AAT) therapy, is essential to the overall treatment.
Antibiotics and chest physiotherapy are the mainstay modalities. Other modalities (beyond those for specific associated conditions) may include bronchodilators, corticosteroid therapy, dietary supplementation, and oxygen or surgical therapies. Admitting patients with severe exacerbations of bronchiectasis to the hospital and treating them with intravenous antibiotics, bronchodilators, aggressive physiotherapy, and supplemental nutrition is not uncommon.
Aggressively pursue and treat any associated or known causal condition of the bronchiectasis. The scope of therapies for these associated medical conditions, such as mycobacterial disease and CF, is beyond the scope of this article. See Cystic Fibrosis and Mycobacterium Avium-Intracellulare.
Supportive Treatment
The following general measures are recommended:
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Smoking cessation
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Avoidance of second-hand smoke
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Adequate nutritional intake with supplementation, if necessary
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Confirmation of immunizations for measles, rubeola, and pertussis
Oxygen therapy is reserved for patients who are hypoxemic with severe disease and end-stage complications, such as cor pulmonale.
Patients with cystic fibrosis (CF) should be cared for at specialized CF treatment centers that address all aspects of the disease, including nutritional and psychologic aspects.
Antibiotic Therapy
Antibiotics have been the mainstay of treatment for more than 40 years. Oral, parenteral, and aerosolized antibiotics are used, depending on the clinical situation.
In acute exacerbations, broad-spectrum antibacterial agents are generally preferred. However, if time and the clinical situation allows, sampling of respiratory secretions during an acute exacerbation may allow treatment with antibiotics based on specific species identification.
Acceptable choices for the outpatient who is mild to moderately ill include any of the following:
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Amoxicillin
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Tetracycline
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Trimethoprim-sulfamethoxazole
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A second-generation cephalosporin
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A fluoroquinolone
In general, the duration of antibiotic therapy for mild to moderate illness is 7-10 days.
For patients with moderate-to-severe symptoms, parenteral antibiotics, such as an aminoglycoside (gentamicin, tobramycin) and an antipseudomonal synthetic penicillin, a third-generation cephalosporin, or a fluoroquinolone, may be indicated. Patients with bronchiectasis from CF are often infected with mucoid Pseudomonas species, and, as such, tobramycin is often the drug of choice for acute exacerbation.
Infection with Mycobacterium avium complex (MAC) provides special treatment challenges. For the treatment of MAC in the setting of bronchiectasis, the American Thoracic Society recommends a 3- to 4-drug treatment regimen with clarithromycin, rifampin, ethambutol, and possibly streptomycin that is continued until the patient's culture results are negative for 1 year. The typical duration of therapy may be 18-24 months.
Regular antibiotic regimens
Some patients with chronic bronchial infections may need regular antibiotic treatment to control the infectious process. Some clinicians prefer to prescribe antibiotics on a regular basis or for a set number of weeks each month.
The oral antibiotics of choice are the same as those mentioned previously. Potential regimens include daily antibiotics for 7-14 days of each month, alternating antibiotics for 7-10 days with antibiotic-free periods of 7-10 days, or a long-term daily dose of antibiotics. For patients with severe CF and bronchiectasis, intermittent courses of intravenous antibiotics are sometimes used. [89, 90]
Aerosolized antibiotics
In the past several years, the nebulized route of antibiotic administration has received more attention because it is capable of delivering relatively high concentrations of drugs locally with relatively few systemic adverse effects. [91] This is particularly beneficial in treating patients with chronic infection from P aeruginosa. Currently, inhaled tobramycin is the most widely used nebulized treatment for patients with bronchiectasis from either CF or non-CF causes of bronchiectasis. [92, 93, 94, 95, 96] Gentamicin [97] and colistin [98] have also been used.
No significant studies have examined the long-term use of inhaled antibiotics in patients with non-CF bronchiectasis. A study by Govan et al found sustained long-term benefit (12 mo) of inhaled gentamicin in this subgroup, along with an acceptable side effect profile. [99] Optimal dosing regimen of inhaled gentamicin still needs to be elucidated.
Bronchial Hygiene
Good bronchial hygiene is paramount in the treatment of bronchiectasis, because of the tenacious sputum and defects in clearance of mucus in these patients. Postural drainage with percussion and vibration is used to loosen and mobilize secretions.
Devices available to assist with mucus clearance include flutter devices, [100, 101] intrapulmonic percussive ventilation devices, and incentive spirometry. [102] Although consistent benefits from these techniques are lacking and vary with patient motivation and knowledge, a review did report improvement in patients’ cough-related quality of life scores. [103]
A relatively new device called the "Vest" system is a pneumatic compression device/vest that is worn by the patient periodically throughout the day. It is essentially technique independent and has variable success, especially in patients with CF. Significant controlled trials have not been performed in patients with non-CF bronchiectasis.
Nebulization with concentrated (7%) sodium chloride solutions appears to be beneficial, particularly in patients with CF-related bronchiectasis. [104, 105, 106] Mucolytics, such as acetylcysteine, are also often tried but do not appear to be universally beneficial. However, maintaining adequate general hydration, which may improve the viscidity of secretions, is important.
Aerosolized recombinant DNase has been shown to benefit patients with CF. [107, 108] This enzyme breaks down DNA released by neutrophils, which accumulates in the airways in response to chronic bacterial infection. However, improvement has not been definitively shown in patients with bronchiectasis from other causes. [109]
Bronchodilator Therapy
Bronchodilators, including beta-agonists and anticholinergics, may help some patients with bronchiectasis, presumably reversing bronchospasm associated with airway hyperreactivity and improving mucociliary clearance. [110, 111, 112] High-quality, large, randomized clinical trials of bronchodilator treatment in bronchiectasis have not been performed, however.
Anti-inflammatory Therapy
The rationale of anti-inflammatory therapy is to modify the inflammatory response caused by the microorganisms associated with bronchiectasis and subsequently reduce the amount of tissue damage. Inhaled corticosteroids, [113] oral corticosteroids, [114] leukotriene inhibitors, [115] and nonsteroidal anti-inflammatory agents [115] have all been examined.
Although some evidence suggests a benefit from the use of these agents, some evidence does not, and findings are not universally definitive. [116] One study reported that inhaled corticosteroids are beneficial compared with placebo in patients with bronchiectasis, particularly those with associated P aeruginosa infections. [117]
A double-blind, placebo controlled 6-week crossover study with 20 patients using beclomethasone dipropionate (750 mcg bid) showed reduced mean sputum volume and improved forced expiratory volume in 1 second (FEV1) at 6 weeks. A similar study of 24 patients using fluticasone propionate (500 mcg bid) showed reduced sputum leukocyte density and reduced levels of inflammatory mediators but no change in pulmonary function.
A study by Tsang et al showed benefit of inhaled fluticasone in patients with chronic P aeruginosa infection and bronchiectasis. [117] Another study showed improvement in quality-of-life scores with inhaled steroids in patients with steady-state bronchiectasis. [118]
Azithromycin has known anti-inflammatory properties and long-term use has been studied in patients with both CF and non-CF bronchiectasis. In non-CF patients, azithromycin has been shown to decrease exacerbations and improve spirometry and microbiologic profiles. [119, 120] In CF patients a meta-analysis suggests that it improves lung function, especially in those patients colonized with Pseudomonas. [106]
A practical approach is to use tapering oral corticosteroids and antibiotics for acute exacerbations and to consider inhaled corticosteroids for daily use in patients with significant obstructive physiology on pulmonary function testing and evidence of reversibility suggesting airway hyperreactivity. However, Kapur et al reported that the evidence supporting the use of inhaled steroids in adults with stable bronchiectasis is insufficient. [121]
Adjunctive Surgical Resection
Surgery is an important adjunct to therapy in some patients with advanced or complicated disease. [122] Surgical resection for bronchiectasis can be performed with acceptable morbidity and mortality in patients of any age. [94, 123, 124]
In general, surgery should be reserved for patients who have focal disease that is poorly controlled by antibiotics. The involved bronchiectatic sites should be completely resected for optimal symptom control. Other indications for surgical intervention may include the following:
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Reduction of acute infective episodes
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Reduction of excessive sputum production
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Massive hemoptysis (Alternatively, bronchial artery embolization may be attempted for the control of hemoptysis.)
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Foreign body or tumor removal
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Consideration in the treatment of MAC or Aspergillus species infections
Complications of surgical intervention include empyema, hemorrhage, prolonged air leak, and persistent atelectasis.
Patient selection plays an important role in perioperative mortality rates, which may be as low as 1% in the surgical treatment of segmental or even multisegmental bronchiectasis.
Lung Transplantation
Single- or double-lung transplantation has been used as treatment of severe bronchiectasis, predominantly when related to CF. In general, consider patients with CF and bronchiectasis for lung transplantation when FEV1 falls below 30% of the predicted value. Female patients and younger patients may need to be considered sooner.
Consultations
A pulmonologist or other practitioner skilled in caring for patients with bronchiectasis should be consulted. All patients with CF should be referred to a regional center with the resources and trained personnel to care for patients with CF, including nutritional and psychological care.
Long-Term Monitoring
The interval of follow-up care is determined by the patient's clinical condition and associated conditions or causes. Patients with CF should optimally be monitored at a center specialized in the care of CF.
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Cylindrical bronchiectasis with signet-ring appearance. Note that the luminal airway diameter is greater than the diameter of the adjacent vessel.
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Cystic and cylindrical bronchiectasis of the right lower lobe on a posterior-anterior chest radiograph.
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Varicose bronchiectasis with alternating areas of bronchial dilatation and constriction.
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This CT scan depicts areas of both cystic bronchiectasis and varicose bronchiectasis.
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- Overview
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- DDx
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- Approach Considerations
- Sputum Analysis
- CBC Count
- Quantitative Immunoglobulin levels
- Quantitative Alpha1-Antitrypsin Levels
- Pilocarpine Iontophoresis (Sweat Test)
- Aspergillus Precipitins and Serum Total IgE levels
- Autoimmune Screening Tests
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