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eMedicine Specialties > Pulmonology > Obstructive Airways Diseases
Bronchiectasis
Ethan E Emmons, MD,Assistant Chief, Pulmonary Disease and Critical Care Medicine, Brooke Army Medical Center
Updated: Apr 16, 2009
Introduction
Background
Bronchiectasis is an uncommon disease that results in the abnormal and permanent distortion of one or more of the conducting
bronchi or airways, most often secondary to an infectious process. First described by Laennec in 1819, later detailed by Sir William
Osler in the late 1800s, and further defined by Reid in the 1950s, bronchiectasis has undergone significant changes in regard to its
prevalence, etiology, presentation , and treatment.1
Bronchiectasis can be categorized as a chronic obstructive pulmonary lung disease manifested by airways that are inflamed and
easily collapsible, resulting in air flow obstruction with shortness of breath, impaired clearance of secretions often with disabling
cough, and occasionally hemoptysis. Severe cases can result in progressive impairment with respiratory failure. 2,3
Bronchiectasis most often presents as (1) a focal process involving a lobe , segment, or subsegment of the lung or (2) a diffuseprocess involving both lungs. The former is by far the most common presentation of bronchiectasis, while the latter is most often
associated with systemic illnesses, such as cystic fibrosis (CF), sinopulmonary disease, or both.
Diagnosis is usually based on a compatible clinical history of chronic respiratory symptoms, such as a daily cough and viscid sputum
production, and characteristic radiographic findings on CT scans, such as bronchial wall thickening and luminal dilatation.
Pathophysiology
Bronchiectasis is an abnormal dilation of the proximal and medium-sized bronchi (>2 mm in diameter) caused by weakening or
destruction of the muscular and elastic components of the bronchial walls. Affected areas may show a variety of changes, including
transmural inflammation, edema, scarring, and ulceration, among other findings. Distal lung parenchyma may also be damaged
secondary to persistent microbial infection and frequent postobstructive pneumonia. Bronchiectasis can be congenital or acquired but
is most often the latter. 1
Congenital bronchiectasis usually affects infants and children and results from developmental arrest of the bronchial tree. The more
commonly acquired forms occur in adults and older children and require an infectious insult, impairment of drainage , airway
obstruction, and/or a defect in host defense. The tissue is also damaged in part by the host response of neutrophilic proteases,
inflammatory cytokines, nitric oxide, and oxygen radicals. This results in damage to the muscular and elastic components of the
bronchial wall. Additionally, peribronchial alveolar tissue may be damaged, resulting in diffuse peribronchial fibrosis.4
The result is abnormal bronchial dilatation with bronchial wall destruction and transmural inflammation. The most important functional
finding of altered airway anatomy is severely impaired clearance of secretions from the bronchial tree.
Impaired clearance of secretions causes colonization and infection with pathogenic organisms, contributing to the common purulent
expectoration observed in patients with bronchiectasis. The result is further bronchial damage and a vicious cycle of bronchialdamage, bronchial dilation, impaired clearance of secretions, recurrent infection, and more bronchial damage.5
In 1950, Reid characterized bronchiectasis as cylindrical, cystic, or varicose in nature.6
Cylindrical bronchiectasis involves diffuse mucosal edema, with resultant bronchi that are dilated minimally but have straight,
regular outlines that end squarely and abruptly (see Media File 1).
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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.
Cystic or saccular bronchiectasis has ulceration with bronchial neovascularization and a resultant ballooned appearance that
may have air-fluid levels (see Media File 2).
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Cystic and cylindrical bronchiectasis of the right lower lobe on a posterior-anterior chest radiograph.
Varicose bronchiectasis has a bulbous appearance with a dilated bronchus and interspersed sites of relative constriction and,
potentially, obstructive scarring. The latter may subsequently result in postobstructive pneumonitis and additional parenchyma
damage (see Media File 3).
Varicose bronchiectasis with alternating areas of bronchial dilatationand constriction.
Frequency
United States
Currently no systematic data are available to detail the incidence or prevalence of bronchiectasis. A general theory is that the
emergence of vaccines and antibiotics in the 20th century has resulted in a decline in the rate of bronchiectasis .7
The best data available suggest that the prevalence of bronchiectasis mirrors the socioeconomic conditions of the population under
study, being significantly less prevalent in areas where immunizations and antibiotics are readily available. Bronchiectasis is relatively
uncommon in the United States, with a prevalence of approximately 100,000 cases based on data from the 1980s. That said, the
number of bronchiectasis cases in the United States that are associated with atypical mycobacteria or other environmental factors
reportedly has increased.8,9,10,11
Bronchiectasis may be underdiagnosed because it is no longer included in survey data and often goes unreported. The exception is
bronchiectasis associated with CF; the latter occurs with a prevalence of 1 in 2500 white births. CF is the largest single cause of
chronic lung infections and bronchiectasis in industrialized nations.12 Native Americans in Alaska comprise a subgroup with higher
than expected prevalence, with a 4-fold higher rate of bronchiectasis than the general population.13 Overall, identifying the true
incidence remains a challenge, given the lack of specific symptoms and lack of readily available noninvasive screening tests for
population studies.
International
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Bronchiectasis remains a major cause of morbidity in less-developed countries, especially in countries with limited access to medical
care and antibiotic therapy.14,15
Mortality/Morbidity
Mortality is difficult to estimate given the difficulty in identifying prevalence and the lack of definitive studies. A study of 400 patients in
1940, prior to widespread antibiotic use, revealed a mortality rate of greater than 30%, with most patients dying within 2 years and at
an age of younger than 40 years.16A retrospective study in 1981, after the widespread use of antibiotics, reported a mortality rate of
13%.17
A more recent study from Finland identified 842 patients aged 35-74 years with bronchiectasis and followed them for 8-13 years.
These patients were also compared with asthma and chronic obstructive pulmonary disease (COPD) controls. The mortality rate was
not found to be significantly different among the 3 groups (bronchiectasis, asthma, COPD), with mortality rates of 28%, 20%, and
38% respectively.18,19
Currently, mortality is more often related to progressive respiratory failure and cor pulmonale than to uncontrolled infection. Life-
threatening hemoptysis may also occur but is uncommon.
Additional complications include chronic bronchial infection, recurrent pneumonia, empyema, pneumothorax, and lung abscess.
Amyloidosis and metastatic abscesses occurred in the preantibiotic era but are rarely observed today.
Race
No racial predilection exists other than those that may be associated with socioeconomic status.
Sex
Evidence suggests that non CF-related bronchiectasis is more common and more virulent in women, particularly slender white
women older than 60 years. In these patients, bronchiectasis is often caused by primary Mycobacterium avium complex (MAC)
infection and has been called the Lady Windermere syndrome, named after a character in a novel by Oscar Wilde .20,21,22
Age
In the preantibiotic era and in today's less-developed countries, symptoms usually began in the first decade of life. Today , the age of
onset, except for those with CF, has moved into adulthood .23
Although limited, epidemiologic studies suggest that persons aged 60-80 years have the highest frequency of bronchiectasisagain
likely from the rise in atypical mycobacterial infections. The differences in prevalence between age groups are a direct reflection of
the differences in prevalence of the underlying causes of bronchiectasis, lung disease, and/or chronic infections.24
The eMedicine Pediatrics article Bronchiectasis may be of interest.
Clinical
History
In clinical practice, the classic manifestations of bronchiectasis are cough and daily mucopurulent sputum production, often lasting
months to years. Blood-streaked sputum or hemoptysis may result from airway damage associated with acute infection.
A rare variant known as dry bronchiectasis manifests by episodic hemoptysis with little-to-no sputum production. Dry bronchiectasis i
usually a sequela of tuberculosis and is found in the upper lobes.
Although patients often report repetitive pulmonary infections that require antibiotics over several years, a single episode of a severe
infection may result in bronchiectasis , often occurring in childhood.25 These include tuberculosis, pertussis, or severe bacterial
pneumonia. Today, CF is the most common cause of bronchiectasis in children and young adults.7
Less specific symptoms include dyspnea, pleuritic chest pain, wheezing, fever, weakness, and weight loss.
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Patients may relate multiple episodes of bronchitis or pulmonary infections, which are exacerbations of bronchiectasis and
often require antibiotics. These acute bacterial infections are often heralded by the onset of increased sputum production over
baseline, increased viscidity of sputum, and, occasionally, a foul odor of the sputum. Rarely , low-grade fever may occur.
Patients may experience an increase in generalized constitutional symptoms, such as fatigue and malaise, as well as
increased dyspnea, shortness of breath, wheezing, or pleuritic pain.
In bronchiectasis, secondary infection, or poorly treated pneumonia, the discrete pathogens are often unknown, but most
patients relate a history of childhood infections that may include tuberculosis, pertussis, orMycoplasma species infection.25
Most individuals have never smoked (55%) or have smoked too little to account for their degree of cough, findings of
obstruction on spirometry testing, and daily sputum production.
Bronchiectasis is a morphologic diagnosis and may exist with relatively few symptoms.
Chronic productive cough is prominent26 occurring in up to 98% of patients. Sputum is typically produced on a daily basis in
greater than 70% of patients, with one study reporting production in 96% of patients.27
Some patients only produce sputum with acute upper respiratory tract infections, but otherwise they have quiescent
disease.
Sputum is typically mucoid and without a rancid odor; however, during infectious exacerbations, sputum becomes
purulent and may develop an offensive odor.
In the past, total daily sputum amount has been used to characterize the severity of bronchiectasis, with less than 10
mL defined as mild bronchiectasis, 10-150 mL defined as moderate bronchiectasis, and greater than 150 mL defined
as severe bronchiectasis. Today, bronchiectasis is most often classified by radiographic findings.
In patients with CF, the volume of sputum produced is generally much greater than that associated with other etiologies
of bronchiectasis.
Hemoptysis occurs in 56-92% of patients with bronchiectasis. Hemoptysis may be massive and life threatening secondary to
bronchial artery bleeding.7,27,28
Hemoptysis is more commonly observed in dry bronchiectasis, although this presentation of bronchiectasis is rare.
Hemoptysis is generally mild and manifested by blood flecks in the patient's usual purulent sputum. This is often the
factor that leads patients to consult a physician.
Bleeding usually originates from dilated bronchial arteries, which contain blood at systemic (rather than pulmonary)
pressures. Therefore, massive hemoptysis may occur but is rarely a cause of death.
Dyspnea may occur in as many as 72% of patients but is not a universal finding. A 2006 review reported a rate of 62%.27
Dyspnea typically occurs in patients with extensive bronchiectasis observed on chest radiographs.
Marked dyspnea is more likely to be secondary to a concomitant illness, such as chronic bronchitis or emphysema.
Wheezing is commonly reported and may be due to airflow obstruction following destruction of the bronchial tree. Similar to
dyspnea, it may also be secondary to concomitant conditions such as asthma.
Pleuritic chest pain is an intermittent finding, occurring in 19-46% of patients.27 It is most commonly secondary to chronic
coughing but also occurs in the setting of acute exacerbation.
Fatigue is commonly reported (73% of patients).27
Weight loss often occurs in patients with severe bronchiectasis.
This is believed to be secondary to increased caloric requirements associated with the increased work of coughing and
clearing secretions.
Weight loss suggests advanced disease but is not diagnostic of bronchiectasis.
Fever may occur in the setting of acute infectious exacerbations.
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Also of interest is that urinary incontinence occurs more frequently in women with bronchiectasis versus age-matched controls
(47% vs 12%).29 The etiology of this is unclear.
Physical
Findings are nonspecific and may be attributed to other conditions. Most commonly, crackles, rhonchi, wheezing, and inspiratory
squeaks may be heard upon auscultation. General findings may include digital clubbing, cyanosis , plethora, wasting, and weight loss
Nasal polyps and signs of chronic sinusitis may also be present. In advanced disease, the physical stigmata of cor pulmonale may be
observed.
Crackles and rhonchi are often observed in association with active infections and acute exacerbations.
Crackles are nonspecific and may occur in as many as 73% of patients. 27
Scattered wheezing may be heard in approximately one third of patients. Wheezing may be due to airflow obstruction from
secretions, destruction of the bronchial tree leading to airway collapsibility, or concomitant conditions.7,27
Digital clubbing is an inconsistent finding in approximately 2-3% of patients. 27 It is more frequent in patients with moderate-to-
severe bronchiectasis.
Cyanosis and plethora are rare findings secondary to polycythemia from chronic hypoxia.
Wasting and weight loss are suggestive of advanced disease but are not diagnostic of bronchiectasis.
In severe cases, findings are consistent with cor pulmonale. Right-sided heart failure may be observed, including peripheral
edema, hepatomegaly, and hypoxia. This can ultimately lead to progressive respiratory failure.30
Causes
Primary infections
Bronchiectasis may be the sequela of a variety of necrotizing infections that are either poorly treated or not treated at
all and are not occurring in the setting of another associated condition. This was particularly common in developed
countries prior to the widespread use of antibiotics25 and today remains an important cause of bronchiectasis in
developing countries, where antibiotics are used inconsistently.14,15
Typical offending organisms that have been known to cause bronchiectasis include Klebsiella species, Staphylococcus
aureus, Mycobacterium tuberculosis, Mycoplasma pneumoniae, nontuberculous mycobacteria, measles virus, pertussis
virus, influenza virus, herpes simplex virus, and certain types of adenovirus.7,25
Infection with respiratory syncytial virus in childhood may also result in bronchiectasis .
MAC infection deserves special mention for its propensity to occur in the setting of human immunodeficiency virus (HIV
and in hosts who are immunocompetent.31 MAC infection has been observed especially in women who are
nonsmokers; are older than 60 years; and have a consistent history, positive acid-fast bacilli on sputum smear, and a
CT scan with small regular nodules and findings of bronchiectasis.8,11,22
Once a patient develops bronchiectasis, many of these same organisms colonize the damaged bronchi and may result
in ongoing damage and episodic infectious exacerbations. The organisms found most typically include Haemophilus
species (47-55% of patients) and Pseudomonas species (18-26% of patients).32,33Although not a primary cause of bronchiectasis, patients with non -CF bronchiectasis often develop chronic bronchial
infection with Pseudomonas aeruginosa via a mechanism involving biofilm formation and the release of virulence
factors. This suggests that Pseudomonas species may promote disease progression and may be related to worsening
lung function and increased morbidity and mortality.34
Bronchial obstruction
Focal postobstructive bronchiectasis may occur in a number of clinical settings (eg, endobronchial tumors,
broncholithiasis, bronchial stenosis from infections, encroachment of hilar lymph nodes, foreign body aspiration).
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Right-middle lobe syndrome is a specific type of bronchial obstruction that may result in bronchiectasis . It results from
an abnormal angulation of the lobar bronchus at its origin, predisposing it to obstruction, subsequent infection , and
development of bronchiectasis.
Aspiration
In adults, foreign body aspiration often takes place in the setting of altered mental status and involves unchewed food.
Patients may also aspirate chewed materials from the stomach, including food, acid, and microorganisms.
After aspiration, a postobstructive pneumonia may occur, with subsequent development of focal bronchiectasis.
Bronchiectasis may also develop in the setting of chronic aspiration. Further recognized is that a history ofgastroesophageal reflux is a risk factor for aspiration and that the organism Helicobacter pylorimay be playing a role in
the development of bronchiectasis in this group of patients.35,36,37
Cystic fibrosis
CF and its variants are likely the most common cause of bronchiectasis in the United States and other industrialized
nations. CF is an autosomal recessive disease affecting approximately 1 in 2,500 whites and 1 in 17,000 blacks in the
United States.38 Estimates indicate 10,000 adults in the United States in 2005 would have CF, and this would comprise
40% of the total CF population .39
CF is a multisystem disorder that affects the chloride transport system in exocrine tissues, primarily secondary to a
defect in the CF transmembrane regulator (CFTR) protein. Multiple genetic variants exist , and the importance of
patients that have genetic heterozygous mutations remains to be elucidated. However, a reasonable assumption is that
CF can be divided into 2 groups of patients: (1) those with classic disease that is readily diagnosed based on clinical
and laboratory data and (2) those with less severe disease that manifests later in life and who have ambiguous genetic
testing results.40,41,42
The major pulmonary finding in CF is bronchiectasis, which is an almost universal feature of this disease. It may be the
sole feature of CF in adults or those with genetic variations of the disease.
Bronchiectasis associated with CF is believed to occur secondary to mucous plugging of proximal airways and chronic
pulmonary infection, especially with mucoid P aeruginosa.43
Young syndrome44
Young syndrome is clinically similar to CF and may represent a genetic variant of the disease. It is most commonly see
in North American males and is a leading cause of male infertility.
Patients have bronchiectasis (often predominant in the lower lobes), sinusitis, and obstructive azoospermia, but they
are not affected with the other findings of CF.
It is most often observed in middle-aged men.
The pathogenesis of bronchiectasis is believed to be similar to that of CF.
The criterion standard for diagnosis is electron microscopic analysis of the structure of the cilia .
Primary ciliary dyskinesia
Primary ciliary dyskinesia is a group of inherited disorders that may affect 1 in 15,000-30,000 persons. It is manifested
by immotile or dyskinetic cilia and/or sperm. This may lead to poor mucociliary clearance, recurrent pulmonary
infections, and, ultimately, bronchiectasis.45,46
A variant of this condition, initially described by Kartagener, encompassed the clinical triad of situs inversus, nasalpolyps or sinusitis, and bronchiectasis in the setting of immotile cilia of the respiratory tract.47
Allergic bronchopulmonary aspergillosis48
Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to inhaled Aspergillus antigen that is
characterized by bronchospasm, bronchiectasis, and immunologic evidence of a reaction to Aspergillus species.
ABPA should be suspected in patients with a productive cough who also have a long history of asthma-type symptoms
that do not respond to conventional therapy.
Bronchiectasis is believed to be secondary to airway plugging by viscid secretions containing hyphae ofAspergillus
species. The resulting bronchiectasis is thin-walled and affects the central and medium-sized airways.
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Sarcoidosis may cause bronchiectasis by a variety of mechanisms, including parenchymal scarring, endobronchial
granulomatous inflammation, or extrinsic compression of bronchi .73
Traction bronchiectasis: Traction bronchiectasis is distortion of the airways secondary to mechanical traction on the bronchi
from fibrosis of the surrounding lung parenchyma. Although the airways may become dilated in this situation, the other
manifestations of bronchiectasis are lacking.
Toxic gas exposure: Exposure to toxic gas may often cause irreversible damage to the bronchial airways and cystic
bronchiectasis. Commonly suspected agents include chlorine gas and ammonia.
Differential Diagnoses
Alpha1-Antitrypsin Deficiency Gastroesophageal Reflux Disease
Asthma Pneumonia, Aspiration
Bronchitis Pneumonia, Bacterial
Chronic Bronchitis Tuberculosis
Chronic Obstructive Pulmonary Disease
Emphysema
Empyema, Pleuropulmonary
Other Problems to Be Considered
Cystic Fibrosis
Workup
Laboratory Studies
In a typical patient, bronchiectasis is suspected based on the clinical presentation, especially if purulent sputum is present and other
conditions, such as pneumonia and lung abscess, have been ruled out. A sputum analysis may be used to further strengthen clinical
suspicion. Radiographic studies, specifically CT scanning, then may be used to confirm the diagnosis. Once the diagnosis is
confirmed, additional laboratory testing may be useful to determine the underlying cause. Although many causes are untreatable,
identifying treatable conditions is paramount. In a significant percentage of patients, no readily identifiable cause is found. The choice
of laboratory tests may vary and should be tailored to the individual patient and clinical situation.
A sputum analysis may reinforce the diagnosis of bronchiectasis and add significant information regarding potential etiologies.
Once sputum is allowed to settle, the examination may reveal Dittrich plugs, small white or yellow concretions.
A Gram stain and culture result may reveal evidence of microorganisms, including mucoid Pseudomonas species and
Escherichia coli, which suggest CF but are not diagnostic. Chronic bronchial infection with nonmucoid P aeruginosa is
becoming much more common in patients with non-CF bronchiectasis.
The presence of eosinophils and golden plugs containing hyphae suggestsAspergillus species, although this finding
alone is not diagnostic of ABPA .
Perform a smear and culture of sputum for mycobacteria and fungi. Atypical mycobacterial infection is a common causeof bronchiectasis in the older population, especially in those with underlying structural lung disease.
CBC count is often abnormal in patients with bronchiectasis and may be useful.
Typical findings are nonspecific and include anemia and an elevated white blood cell count with an increased
percentage of neutrophils. An increased percentage of eosinophils is one criterion for ABPA.
Alternatively, polycythemia secondary to chronic hypoxia may be observed in advanced cases .
Quantitative immunoglobulin levels, including IgG subclasses, IgM, and IgA, are useful to exclude hypogammaglobulinemia .
Note, however, that on rare occasions, bronchiectasis may be seen in patients with antibody production deficiency but normal
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to low-normal IgG levels. In situations such as these, evaluating antibody response to Haemophilus influenzae and
pneumococcal vaccines may be useful.
Quantitative AAT levels are used to rule out ATT deficiency.
Pilocarpine iontophoresis (sweat test) was the criterion standard test to evaluate for CF. However , genetic analysis has now
become standard and may be performed to look for evidence of mutations consistent with CF and to look for potential
variants, such as Young syndrome.38
Aspergillus precipitins and serum total IgE levels are important in making the diagnosis of ABPA .
Rheumatoid factor and/or other autoimmune screening tests may be performed in the appropriate clinical setting.
Imaging Studies
High-resolution CT (HRCT) scanning is the criterion standard for the diagnosis of bronchiectasis.74,75,76
Additionally, the anatomical distribution of bronchiectasis may be important in helping diagnose any associated condition or
cause of bronchiectasis.
Bronchiectasis as a result of infection generally involves the lower lobes, the right-middle lobe, and the lingula.
Right-middle lobe involvement alone suggests right-middle lobe syndrome, an anatomic dysfunction, or a neoplastic
cause with secondary mechanical obstruction.Bronchiectasis caused by CF, M tuberculosis, or chronic fungal infections tends to affect the upper lobes, although this
is not universal in the former .
ABPA also affects the upper lobes but usually involves the central bronchi, whereas most other forms of bronchiectasis
involve distal bronchial segments.
Posterior-anterior and lateral chest radiographs should be obtained in all patients.
Expected general findings include increased pulmonary markings, honeycombing, atelectasis, and pleural changes.
Specific findings may include linear lucencies and parallel markings radiating from the hila (tram tracking) in cylindrical
bronchiectasis, dilated bronchi in varicose bronchiectasis, and clustered cysts in cystic bronchiectasis.
In occasional patients, the diagnosis of bronchiectasis may be based on chest radiograph findings alone in the context
of the appropriate clinical setting.
CT scanning (see Media File 4), particularly HRCT scanning of the chest, has replaced bronchography as the defining
modality of bronchiectasis.
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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 exacerbation, broad-spectrum antibacterial agents are generally preferred. However, if time and the clinical
situation allows, then sampling 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 amoxicillin , tetracycline, trimethoprim-
sulfamethoxazole, a newer macrolide (eg, azithromycin80 or clarithromycin81,82 ), a second-generation cephalosporin, or
one of the fluoroquinolones. In general, the duration 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 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.
Additionally, 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 dailyantibiotics 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.83,84
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.85 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.86,87,88,89,90 Gentamicin91 and colistin92 have also been used.
Bronchial hygiene
With its tenacious sputum and defects in clearance of mucus, good bronchial hygiene is paramount in the treatment of
bronchiectasis. Postural drainage with percussion and vibration is used to loosen and mobilize secretions. Other
devices available to assist with mucus clearance include flutter devices93,94 intrapulmonic percussive ventilation
devices, and incentive spirometry. 95 However, consistent benefits from these techniques are lacking and vary with
patient motivation and knowledge. A new device called the Vest system is a pneumatic compression device worn by the
patient periodically throughout the day and is relatively technique independent.
Nebulization with sodium chloride solutions appears to be beneficial, particularly in patients with CF-related
bronchiectasis.96,97,98 Mucolytics, such as acetylcysteine, are also often tried but a universal benefit does not seem to
exist. However, maintaining adequate general hydration, which may improve the viscidity of secretions, is important.
Aerosolized recombinant DNase, which breaks down by-product DNA from neutrophils , has been shown to benefit
patients with CF.99,100 However, improvement has not been definitively shown in patients with bronchiectasis from
other causes.101
Bronchodilators
Bronchodilators, including beta-agonists and anti-cholinergics, may help some patients with bronchiectasis , presumably
reversing bronchospasm associated with airway hyperreactivity and improving mucociliary clearance.102,103,104
Treatment with inhaled bronchodilators may be appropriate, although good, large, randomized clinical trials looking at
their use in bronchiectasis have not been performed.
Anti-inflammatory medication
The rationale is to modify the inflammatory response caused by the microorganisms associated with bronchiectasis and
subsequently reduce the amount of tissue damage. Inhaled corticosteroids105 oral corticosteroids106 leukotriene
inhibitors107 and nonsteroidal anti-inflammatory agents108 have all been examined. Although evidence tends to support
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some benefit from the use of these agents, findings are not universally definitive. One study reported that inhaled
corticosteroids are beneficial to patients with bronchiectasis compared with a placebo, particularly patients with
associated P aeruginosa infections109 while another study showed improvement in quality-of-life scores.110
A practical approach is to use tapering oral corticosteroids and antibiotics in the acute exacerbation 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.
Kapur et al report that the evidence supporting the use of inhaled steroids in adults with stable bronchietasis is
insufficient.111
Surgical Care
Surgery is an important adjunct to therapy in some patients with advanced or complicated disease.112 Surgical resection for
bronchiectasis can be performed with acceptable morbidity and mortality in patients of any age .88,113,114 In general, surgery
should be reserved for patients who have focal disease that is poorly controlled by antibiotics. The involved bronchiectatic site
should be completely resected for optimal symptom control. Other indications for surgical intervention may include the
following:
Reduction of acute infective episodes
Reduction of excessive sputum production
Massive hemoptysis (Alternatively, bronchial artery embolization may be attempted for the control of hemoptysis .)
Foreign body or tumor removal
Consideration in the treatment of MAC orAspergillus 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.
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 forced expiratory volume in 1
second (FEV1) falls below 30% of the predicted value. Female patients and younger patients may need to be considered even
sooner.
Consultations
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.
Medication
No specific medical therapy exists for the treatment of bronchiectasis . Therapy is focused on the treatment of infectious
exacerbations that the patient commonly experiences, most commonly in the form of an acute bronchitis-type syndrome. 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 thisarticle and is found elsewhere in eMedicine. See Cystic Fibrosis and Mycobacterium Avium-Intracellulare.
The remainder of this section focuses on the most widely accepted and commonly used medications in the treatment of acute
infectious processes associated with bronchiectasis. These medications include antibiotics, beta-agonists, inhaled corticosteroids, and
expectorants. Other more controversial medications have been previously mentioned in this article for completeness but are not
discussed here.
Antibiotics
These are the mainstays of treatment of patients with bronchiectasis and infectious exacerbations. The route of antibiotic
administration varies with the overall clinical condition, with most patients doing well on outpatient regimens. Some patients benefit
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from a set regimen of antibiotic therapy, such as therapy for 1 week of every month. The choice of antibiotic is provider dependent,
but, in general, the antibiotic chosen should have a reasonable spectrum of coverage, including the most common gram-positive and
gram-negative organisms.
Treatment of the patient who is more ill or the patient with CF often requires intravenous anti-Pseudomonas species coverage with an
aminoglycoside, most often in combination with an antipseudomonal synthetic penicillin or cephalosporin. Aerosolized tobramycin has
been found effective in patients with CF.
Clarithromycin (Biaxin)
Semisynthetic macrolide antibiotic that reversibly binds to P site of 50S ribosomal subunit of susceptible organisms and may inhibit
RNA-dependent protein synthesis by stimulating dissociation of peptidyl t-RNA from ribosomes, causing bacterial growth inhibition.
Dosing
Adult
500 mg PO bid for 7-14 d
Pediatric
Not established
Interactions
Toxicity increases with coadministration of fluconazole and pimozide; effects decrease and adverse GI effects may increase with
coadministration of rifabutin or rifampin; may increase toxicity of anticoagulants, cyclosporine, tacrolimus, digoxin, carbamazepine,
ergot alkaloids, triazolam, and HMG-CoA reductase inhibitors
Plasma levels of certain benzodiazepines may increase, prolonging CNS depression; arrhythmias and increases in QTc intervals
occur with disopyramide; coadministration with omeprazole may increase plasma levels of both agents; decreases metabolism of
repaglinide, thus increasing serum levels and effects
Contraindications
Documented hypersensitivity; coadministration of pimozide
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Coadministration with ranitidine or bismuth citrate is not recommended with CrCl
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Day 1: 500 mg PO
Days 2-5: 250 mg/d PO
Pediatric
Day 1: 10 mg/kg PO once; not to exceed 500 mg/d
Days 2-5: 5 mg/kg/d PO; not to exceed 250 mg/d
Interactions
Aluminum- and magnesium-containing antacids reduce peak serum levels; medications not reported to interact with azithromycin butwith other macrolides, suggesting careful monitoring, include warfarin, theophylline, digoxin, ergotamine , triazolam, carbamazepine ,
terfenadine, cyclosporine, and phenytoin; effects reduced with coadministration of aluminum and/or magnesium antacids;
nephrotoxicity and neurotoxicity may occur when coadministered with cyclosporine
Contraindications
Documented hypersensitivity; do not administer with pimozide
Precautions
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Site reactions can occur with IV route; bacterial or fungal overgrowth may result with prolonged antibiotic use; may increase hepatic
enzymes and cholestatic jaundice; caution in patients with impaired hepatic function, prolonged QT intervals, or pneumonia; caution
in patients who are hospitalized, geriatric, or debilitated
Trimethoprim and sulfamethoxazole (Septra, Bactrim)
Synthetic combination antibiotic. Each tab contains 80 mg of trimethoprim and 400 mg of sulfamethoxazole. Rapidly absorbed after
oral administration.
Mechanism of action involves blockage of 2 consecutive steps in biosynthesis of nucleic acids and proteins needed by many
microorganisms. Coverage for common forms of both gram-positive and gram-negative organisms, including susceptible strains ofStreptococcus pneumoniae and H influenzae.
Indicated in treatment of acute and chronic bronchitic symptoms in patients with bronchiectasis.
Dosing
Adult
2 tab PO q12h for 14 d; if used monthly, alternatively may be administered as 10-d course
Pediatric
2 months: 8 mg/kg TMP and 40 mg/kg SMZ PO per 24 h, administered in 2 divided doses q12h for 10 d
Interactions
May have drug-to-drug interactions with thiazide diuretics, warfarin, phenytoin, and methotrexate; may interact with a serum
methotrexate assay and may interfere with Jaffe alkaline picrate reaction assay for creatinine
Contraindications
Documented hypersensitivity; megaloblastic anemia due to folate deficiency; pregnancy at term; breastfeeding mothers; infants
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Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Discontinue at first appearance of rash or sign of adverse reaction; obtain CBC count frequently; discontinue therapy if significant
hematologic changes occur; goiter , diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses
may cause bone marrow depression (if signs occur, administer 5-15 mg/d leucovorin); caution in folate deficiency (eg, patients with
chronic alcoholism, elderly patients, those receiving anticonvulsant therapy, those with malabsorption syndrome); hemolysis may
occur in individuals who are G-6-PD deficient; patients with AIDS may not tolerate or respond to TMP-SMZ; caution in renal orhepatic impairment (perform urinalyses and renal function tests during therapy); administer fluids to prevent crystalluria and stone
formation
Doxycycline (Doryx, Vibra-Tabs, Vibramycin)
Broad-spectrum, synthetically derived bacteriostatic antibiotic in the tetracycline class. Almost completely absorbed, concentrates in
bile, and is excreted in urine and feces as a biologically active metabolite in high concentrations.
Inhibits protein synthesis and, thus, bacterial growth by binding to 30S and possibly 50S ribosomal subunits of susceptible bacteria.
May block dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.
Alternative agent for patients who cannot be given macrolides or penicillins.
Dosing
Adult
100 mg PO bid for 10 days
Pediatric
8 years: 2-5 mg/kg/d PO qd or divided bid; not to exceed 200 mg/d
Interactions
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; tetracyclines can
increase hypoprothrombinemic effects of anticoagulants; tetracyclines can decrease effects of oral contraceptives, causingbreakthrough bleeding and increased risk of pregnancy
Contraindications
Documented hypersensitivity; severe hepatic dysfunction
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drugserum level determinations in prolonged therapy; tetracycline use during tooth development (last half of pregnancy through age 8 y)
can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines
Levofloxacin (Levaquin)
Fluoroquinolones should be used empirically in patients likely to develop exacerbation due to resistant organisms to other antibiotics.
Rapidly becoming a popular choice in pneumonia. This is the L stereoisomer of the D/L parent compound ofloxacin, the D form being
inactive. Good monotherapy with extended coverage against Pseudomonas species and excellent activity against pneumococcus.
Agent acts by inhibition of DNA gyrase activity . PO form has bioavailability that reportedly is 99%.
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Dosing
Adult
500 mg PO/IV qd
Pediatric
18 years: Administer as in adults
Interactions
Antacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones;
cimetidine may interfere with metabolism of fluoroquinolones; reduces therapeutic effects of phenytoin; probenecid may increase
serum concentrations
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
In prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal
function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Tobramycin for inhalation (TOBI)
Aminoglycoside specifically developed for administration with a nebulizer system.
When inhaled, concentrated in airways where antibacterial effect exerted by disrupting protein synthesis.
Active against wide range of gram-negative organisms, including P aeruginosa. Indicated for treatment of patients with CF and P
aeruginosa infection.
Dosing
Adult
300-mg dose administered via a nebulizer; recommended treatment regimen is repeated cycles of 28 d of medication q12h, followed
by 28 d off
Pediatric
6 years: Administer as in adults
Interactions
Increases effects of neuromuscular blockers and potentiates effect of extended-spectrum penicillins; concurrent administration with
amphotericin B, cephalosporins, and loop diuretics increases risk of nephrotoxicity
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
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D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Although not reported to cause same complications as IV aminoglycosides, caution when prescribing to patients with known or
suspected renal, auditory, vestibular, or neuromuscular dysfunction
Gentamicin (Gentacidin, Garamycin)
Water-soluble injectable antibiotic of aminoglycoside group. Acts by inhibiting normal protein synthesis; active against variety of
pathogenic organisms, including P aeruginosa.
When treating Pseudomonas species, often used in combination with an antipseudomonal synthetic penicillin or cephalosporin.
In patients with bronchiectasis, gentamicin (or other aminoglycosides) may be indicated in setting of severe respiratory tract infection
or in patients with CF.
Dosing regimens are numerous; adjust dose based on CrCl and changes in volume of distribution. May be administered IV /IM.
Dosing
Adult
3 mg/kg/d IV divided tid in normal renal function; once-a-day dosing also effective; follow each regimen by at least a trough level
drawn on the third or fourth dose (0.5 h before dosing); may draw a peak level 0.5 h after 30-min infusion
Pediatric
6-7.5 mg/kg/d IV divided q8h
Interactions
Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity ;
aminoglycosides enhance effects of neuromuscular blocking agents, thus prolonged respiratory depression may occur;
coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying
degrees may occur (monitor regularly)
Contraindications
Documented hypersensitivity; nondialysis-dependent renal insufficiency
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Narrow therapeutic index (not intended for long-term therapy); caution in impaired renal function and neuromuscular disorders
because may aggravate muscle weakness; serious adverse effects of vestibular and auditory branches of eighth cranial nerve may
occur
Amikacin (Amikin)
Irreversibly binds to 30S subunit of bacterial ribosomes; blocks recognition step in protein synthesis; causes growth inhibition. For
gram-negative bacterial coverage of infections resistant to gentamicin and tobramycin. Effective against P aeruginosa. Use patient's
IBW for dosage calculation. The same principles of drug monitoring for gentamicin apply to amikacin.
Dosing
Adult
10-15 mg/kg/d IV/IM divided bid/tid; not to exceed 1.5 g/d regardless of higher BW
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Pediatric
Neonates: Dose variable to postconceptional and postnatal age
Children: 15-22.5 mg/kg/d IV/IM divided q8h
Interactions
Coadministration with other aminoglycosides, penicillins, cephalosporins, and amphotericin B increases nephrotoxicity; enhances
effects of neuromuscular blocking agents; causes respiratory depression; irreversible hearing loss may occur with coadministration of
loop diuretics
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Not intended for long-term therapy; caution in patients with renal failure (not on dialysis), hypocalcemia, myasthenia gravis, and
conditions that depress neuromuscular transmission
Inhaled Beta Agonist
Although no long-term studies have been performed with inhaled beta-agonists, these medications are routinely used in patients with
bronchiectasis for multiple reasons. Bronchiectasis may cause an obstructive defect on pulmonary function testing that may respond
to inhaled beta-agonists. Many older patients with bronchiectasis often have a concomitant illness, such as chronic obstructive
pulmonary disease, that responds to inhaled beta-agonists. Finally, in the acute infectious bronchitic exacerbation that occurs in
patients with bronchiectasis, patients may develop transient obstructive airway physiology that may be improved with an inhaled beta
agonist. Along these same lines, many patients are started on inhaled steroids for long-term airway stabilization, but the efficacy of
these medications in bronchiectasis is questionable, and any effect simply may be secondary to the treatment of other concomitant
obstructive airway diseases.
Salmeterol (Serevent Diskus)
By relaxing the smooth muscles of the bronchioles in conditions associated with bronchitis, emphysema, asthma, or bronchiectasis,
salmeterol can relieve bronchospasms. Effect also may facilitate expectoration.
Shown to improve symptoms and morning peak flows. May be useful when bronchodilators are used frequently. More studies are
needed to establish the role for these agents.
When administered at high or more frequent doses than recommended, incidence of adverse effects is higher. The bronchodilating
effect lasts >12 h. Used on a fixed schedule in addition to regular use of anticholinergic agents.
Dosing
Adult
Serevent Diskus: 1 inhalation (50 mcg) bid at least 12 h apart
Pediatric
4 years: Administer as in adults
Interactions
Concomitant use of beta-blockers may decrease bronchodilating and vasodilating effects of beta agonists; concurrent administration
with methyldopa may increase pressor response; coadministration with oxytocic drugs may result in severe hypotension; ECG
changes and hypokalemia resulting from diuretics may worsen when coadministered
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Contraindications
Documented hypersensitivity; angina, tachycardia, and cardiac arrhythmias associated with tachycardia
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Not indicated to treat acute asthmatic symptoms; black box FDA warning describes that long-term use may result in increased
asthma morbidity and mortality, use only as additional therapy for patients not adequately controlled on other asthma-controller
medications (eg, low- to medium-dose inhaled corticosteroids) or patients whose disease severity clearly warrants initiation of
treatment with 2 maintenance therapies, including salmeterol
Albuterol sulfate (Proventil, Ventolin, Airet)
Relatively selective beta2-adrenergic bronchodilator that, when inhaled, has the effect of causing relaxation of bronchial smooth
muscle and inhibiting release of mediators of immediate hypersensitivity from cells, especially mast cells. Administered in metered -
dose aerosol unit for oral inhalation; indicated for prevention and relief of bronchospasm from any cause, including those observed in
patients with bronchiectasis.
Dosing
Adult
Acute symptoms: 2 inhalations repeated q4-6h
Pediatric
12 years: Administer as in adults
Interactions
Do not use other sympathomimetic aerosol bronchodilators concomitantly with albuterol; effects on vascular system may bepotentiated by MAOIs or TCAs; beta-receptor blocking agents and albuterol inhibit effect of each other; may lower serum potassium
level and be additive to other drugs that also lower serum potassium level
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in cardiovascular disorders, including coronary artery disease, hypertension, convulsive disorders, hyperthyroidism, and
diabetes mellitus
Inhaled corticosteroids
Studies, although most have been small in numbers, have shown benefit in the use of inhaled steroids. 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 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 more recent study b
Tsang et al showed benefit of inhaled fluticasone in patients with chronic P aeruginosa infection and bronchiectasis.109 The optimal
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dosing of inhaled corticosteroid therapy remains to be determined. No significant studies of oral steroid therapy in patients with
bronchiectasis have been performed.
Beclomethasone dipropionate (Beconase AQ Intranasal)
Inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease number and activity of
inflammatory cells, in turn decreasing airway hyperresponsiveness. Readily absorbed through nasopharyngeal mucosa and GI tract.
Has a weak HPA axis inhibitory potency when applied topically.
Most reliable during pregnancy because has been in use for many years with no significant problems observed. May decreasenumber and activity of inflammatory cells, resulting in decreased nasal inflammation.
Various dose preparations are available and must be titrated in conjunction with other medications patient is taking; most inhaled PO
medications have effect in 24 h.
Dosing
Adult
504-840 mcg/d (42 mcg per actuation, 12-20 puffs qd) inhaled PO divided tid/qid
Pediatric
336-672 mcg/d (42 mcg per actuation, 8-16 puffs qd) inhaled PO divided tid/qid
Interactions
Coadministration with ketoconazole may increase plasma levels but does not appear to be clinically significant
Contraindications
Documented hypersensitivity, bronchospasm, status asthmaticus, other types of acute episodes of asthma
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Coughing, upper respiratory tract infection, and bronchitis may occur
Fluticasone propionate (Flovent)
May decrease number and activity of inflammatory cells, in turn decreasing airway hyperresponsiveness. Also has vasoconstrictive
activity.
Applied as nasal spray. Particularly effective in allergic and vasomotor rhinosinusitis and rhinosinusitis medicamentosa. Used as
prophylaxis for nasal polyps. Plasma concentrations very low following intranasal administration in recommended doses. Advise
patients to administer spray toward the lateral nasal wall, avoiding irritation to septum or having drug run down back of pharynx.
Has a weak HPA axis inhibitory potency when applied topically. Studies concerning bioavailability are established; should be
considered first line when treating pediatric patients. Not systemically absorbed like other nasal steroids (ie, beclomethasone).
Should use nasal steroid spray with fluticasone propionate to help buffer the nose and prevent complications from the spray, such as
nasal drying, epistaxis, and, in long-term use, septal perforation.
Dosing
Adult
110-220 mcg (110 mcg per actuation) inhaled PO bid
Pediatric
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Administer as in adults
Interactions
Coadministration with ketoconazole may increase plasma levels but does not appear to be clinically significant
Contraindications
Documented hypersensitivity; bronchospasm, status asthmaticus, and other types of acute episodes of asthma
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Coughing, upper respiratory tract infection, and bronchitis may occur
Expectorants
One of the hallmarks of bronchiectasis is a chronic, thick, viscid sputum production. In bronchiectasis, it is extremely difficult for the
body's natural mucociliary clearance mechanisms to adequately clear the sputum produced. Although definitive evidence is lacking,
expectorants are expected to increase respiratory tract fluid secretions and to help loosen phlegm and bronchial secretions. By
reducing the viscosity of secretions, this increases the efficacy of mucociliary clearance system. Expectorants are often found in
combination with decongestants, which may provide some patients additional relief.
Guaifenesin (Mucinex)
Contains 600 mg of guaifenesin in a sustained-release formulation intended for oral administration. Increases respiratory tract fluid
secretions and helps to loosen phlegm and bronchial secretions.
Humibid LA and guaifenesin are indicated for patients with bronchiectasis complicated by tenacious mucus and/or mucous plugs.
Dosing
Adult
600-1200 mg PO q12h; not to exceed 2400 mg/d
Pediatric
12 years: Administer as in adults
Interactions
May increase renal clearance of urate and lower serum uric acid levels; may interfere with urine laboratory tests for 5-
hydroxyindoleacetic acid and urine testing for catecholamines
Contraindications
Documented hypersensitivity
Precautions
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
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When prescribing medication that may suppress cough, important to identify cause of the cough so that suppression does not
increase risk of clinical or physiologic complications
Follow-up
Further Inpatient Care
Base the need for admission on the severity of exacerbations, the need for intravenous antibiotics, and other comorbid
conditions.
Further Outpatient Care
A pulmonologist or other practitioner skilled in caring for patients with bronchiectasis should be consulted.
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.
Inpatient & Outpatient Medications
Antibiotics and bronchodilators are used.
Other medications are based on the patient's comorbid conditions.
Complications
In the preantibiotic era, mortality was high and patients most often died within 5 years from the onset of symptoms .
Today, survival is long, and common complications include recurrent pneumonia requiring hospitalization, empyema, lung
abscess, hemoptysis, progressive respiratory failure, and cor pulmonale.
Progressive respiratory failure and cor pulmonale are the most common causes of pulmonary-related mortality in
bronchiectasis. One study found age older than 65 years and prior use of long-term oxygen therapy to be risk factors for a
poor outcome in patients with bronchiectasis who were admitted to an ICU for respiratory failure.30
Prognosis
Overall, the prognosis is good, but it varies with the underlying or predisposing condition. Bronchiectasis associated with CF
may carry a worsened prognosis. A registry study performed in Finland reported no increased mortality in patients with
bronchiectasis versus patients with asthma or COPD.18
In general, patients do well if they are compliant with all treatment regimens and practice routine preventive medicine
strategies.
Patient Education
For excellent patient education resources, visit eMedicine's Lung and Airway Center. Also, see eMedicine's patient education
article Chronic Obstructive Pulmonary Disease (COPD).
Miscellaneous
Medicolegal Pitfalls
Medicolegal pitfalls center around failure to adequately investigate the possible etiology of a patient's bronchiectasis and, thus
potentially to miss a treatable cause.
Specifically, ABPA, atypical mycobacterial infections, immunodeficiency states, and autoimmune diseases are causes
of bronchiectasis that may be treated effectively once diagnosed.
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CF, Young syndrome, primary ciliary dyskinesia, and AAT deficiency require aggressive treatment and management
once diagnosed and genetic counseling for the patients and their families. Likewise, congenital abnormalities should be
identified for the patient and their family as such.
Foreign body obstruction needs to be excluded as an etiology in all patients.
Multimedia
Media file 1: 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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Media file 2: Cystic and cylindrical bronchiectasis of the right lower lobe on a posterior-anterior chest radiograph.
Media file 3: Varicose bronchiectasis with alternating areas of bronchial dilatation and constriction.
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Media file 4: This CT scan depicts areas of both cystic bronchiectasis and varicose bronchiectasis.
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