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Bronchiectasis: [Print] - eMedicine Pulmonology

August 30, 2009 9:40:24 PMhttp://emedicine.medscape.com/article/296961-print

emedicine.medscape.com

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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1Bronchiectasis: [Print] - eMedicine Pulmonology


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


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


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


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


Precautions

Pregnancy


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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


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


Precautions

Pregnancy


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


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


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


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


Precautions

Pregnancy


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


22/34



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


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


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


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


Precautions

Pregnancy


Precautions


24/34



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.


25/34



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.


26/34



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.


27/34



Media file 4: This CT scan depicts areas of both cystic bronchiectasis and varicose bronchiectasis.

References

1. Luce JM. Bronchiectasis. In: Murray JF, Nadel JA, eds. Textbook of Respiratory Medicine. 2nd ed. Philadelphia, Pa: WB

Saunders and Co; 1994:1398-1417.

2. Ip MS, Lam WK. Bronchiectasis and related disorders. Respirology. Jun 1996;1(2):107-14. [Medline].

3. Kolbe J, Wells AU. Bronchiectasis : a neglected cause of respiratory morbidity and mortality. Respirology. Dec 1996;1(4)

:221-5. [Medline].

4. Morrissey D. Pathogenesis of Bronchiectasis. Clin Chest Med. 2007;28:289-296.

5. Cole PJ. A new look at the pathogenesis, management of persistent bronchial sepsis: A 'viscious circle' hypothesis and its

logical therapeutic connotations. In: Davies RJ. Strategies for the Management of Chronic Bacterial Sepsis. Oxford: Medicine

Publishing Foundation; 1984:1-20.

6. Reid LM. Reduction in bronchial subdivision in bronchiectasis. Thorax. Sep 1950;5(3):233-47. [Medline].

7. Barker AF. Bronchiectasis . N Engl J Med. May 2 2002;346(18):1383-93. [Medline].

8. Koh WJ, Kwon OJ. Bronchiectasis and non-tuberculous mycobacterial pulmonary infection. Thorax. May 2006 ;61(5):458;author reply 458. [Medline].

9. Wickremasinghe M, Ozerovitch LJ, Davies G, et al. Non -tuberculous mycobacteria in patients with

bronchiectasis. Thorax. Dec 2005;60(12):1045-51. [Medline].

10. Kennedy TP, Weber DJ. Nontuberculous mycobacteria. An underappreciated cause of geriatric lung disease.Am J Respir Cri

Care Med. Jun 1994;149(6):1654-8. [Medline].

11. Wickremasinghe M, Wells A, Daview G, et al. Nontuberculous mycobacteria in patients with

bronchiectasis. Thorax. 2005;60:1045-1051.


28/34



12. Nikolaizik WH, Warner JO. Aetiology of chronic suppurative lung disease.Arch Dis Child. Feb 1994;70(2):141-2. [Medline].

13. Singleton R, Morris A, Redding G, Poll J, Holck P, Martinez P. Bronchiectasis in Alaska Native children: causes and clinical

courses. Pediatr Pulmonol. Mar 2000;29(3):182-7. [Medline].

14. Maxwell GM, Elliott RB, McCoy WT, Langsford WA. Respiratory infections in Australian Aboriginal children: a clinical and

radiological study. Med J Aust. Nov 30 1968;2(22):990-3. [Medline].

15. Chang A, Grimwood K, Mulholland E, et al. Bronchiectasis in indigenous children in remote Australian communities. Med J

Aust. 2002;117:200-204.

16. Perry K, King D. Bronchiectasis , a study of prognosis based on a follow-up of 400 patients.Am Rev Tuber. 1941;40:53.

17. Ellis DA, Thornley PE, Wightman AJ, Walker M, Chalmers J, Crofton JW. Present outlook in bronchiectasis : clinical and socia

study and review of factors influencing prognosis. Thorax. Sep 1981;36(9):659-64. [Medline].

18. Keistinen T, Saynajakangas O, Tuuponen T, Kivela SL. Bronchiectasis: an orphan disease with a poorly-understood

prognosis. Eur Respir J. Dec 1997;10(12):2784-7. [Medline].

19. Saynajakangas O, Keistinen T, Tuuponen T, Kivela SL. Bronchiectasis in Finland: trends in hospital treatment. Respir

Med. Aug 1997;91(7):395-8. [Medline].

20. Wallace RJ Jr. Mycobacterium avium complex lung disease and women. Now an equal opportunity disease. Chest. Jan 1994;

105(1):6-7. [Medline].

21. Iseman MD, Buschman DL, Ackerson LM. Pectus excavatum and scoliosis. Thoracic anomalies associated with pulmonary

disease caused by Mycobacterium avium complex.Am Rev Respir Dis. Oct 1991;144(4):914-6. [Medline].

22. Reich JM, Johnson RE. Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe

pattern. The Lady Windermere syndrome. Chest. Jun 1992;101(6):1605-9. [Medline].

23. Nicotra MB, Rivera M, Dale AM, Shepherd R, Carter R. Clinical, pathophysiologic, and microbiologic characterization of

bronchiectasis in an aging cohort. Chest. Oct 1995;108(4):955-61. [Medline].

24. Morrissey BM, Harper RW. Bronchiectasis: sex and gender considerations. Clin Chest Med. Jun 2004;25(2):361-72. [Medline]

25. Pasteur M, Helliwell S, Houghton S, et al. An investigation into causitive factors in patients with bronchiectasis. Am J Respir

Crit Care Med. 2000;162:1277-1284.

26. Rosen MJ. Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines. Chest. Jan 2006;129(1

Suppl):122S-131S. [Medline].

27. King PT, Holdsworth SR, Freezer NJ, Villanueva E, Holmes PW. Characterisation of the onset and presenting clinical features

of adult bronchiectasis. Respir Med. Dec 2006;100(12):2183-9. [Medline].

28. Flume PA, Yankaskas JR, Ebeling M, Hulsey T, Clark LL. Massive hemoptysis in cystic fibrosis. Chest. Aug 2005;128(2)

:729-38. [Medline].

29. Prys-Picard CO, Niven R. Urinary incontinence in patients with bronchiectasis. Eur Respir J. Apr 2006 ;27(4):866-7. [Medline].

30. Dupont M, Gacouin A, Lena H, et al. Survival of patients with bronchiectasis after the first ICU stay for respiratory

failure. Chest. May 2004;125(5):1815-20. [Medline].

31. Holmes AH, Trotman-Dickenson B, Edwards A, Peto T, Luzzi GA. Bronchiectasis in HIV disease. Q J Med. Nov-Dec 1992;

85(307-308):875-82. [Medline].

32. Angrill J, Augusti C, de Celis R, et al. Bacterial colonization in patients with bronchiectasis : microbiological pattern and risk

factors. Thorax. 2002;57:15-19.


29/34



33. King PT, Holdsworth SR, Freezer NJ, Villanueva E, Holmes PW. Microbiologic follow-up study in adult bronchiectasis. Respir

Med. Aug 2007;101(8):1633-8. [Medline].

34. Davies G, Wells AU, Doffman S, Watanabe S, Wilson R. The effect of Pseudomonas aeruginosa on pulmonary function in

patients with bronchiectasis. Eur Respir J. Nov 2006;28(5):974-9. [Medline].

35. Tsang KW, Lam SK, Lam WK, Karlberg J, Wong BC, Hu WH. High seroprevalence of Helicobacter pylori in active

bronchiectasis.Am J Respir Crit Care Med. Oct 1998;158(4):1047-51. [Medline].

36. Tsang KW, Lam WK, Kwok E, Chan KN, Hu WH, Ooi GC. Helicobacter pylori and upper gastrointestinal symptoms inbronchiectasis. Eur Respir J. Dec 1999;14(6):1345-50. [Medline].

37. De Groote M, Huitt G, Fulton K, et al. Retrospective analysis of aspiration risk and genetic predisposition in bronchiectasis

patients with and without non-tuberculous mycobacteria infection.Am J Respir Crit Care Med. 2003;163:A763.

38. National Institutes of Health. Genetic testing for cystic fibrosis. National Institutes of Health Consensus Development

Conference Statement on genetic testing for cystic fibrosis.Arch Intern Med. Jul 26 1999;159(14):1529-39. [Medline].

39. Yankaskas JR, Marshall BC, Sufian B, Simon RH , Rodman D. Cystic fibrosis adult care: consensus conference

report. Chest. Jan 2004;125(1 Suppl):1S-39S. [Medline].

40. Kerem E, Corey M, Kerem BS, et al. The relation between genotype and phenotype in cystic fibrosis--analysis of the most

common mutation (delta F508). N Engl J Med. Nov 29 1990;323(22):1517-22. [Medline].

41. Groman JD, Meyer ME, Wilmott RW, Zeitlin PL, Cutting GR . Variant cystic fibrosis phenotypes in the absence of CFTR

mutations. N Engl J Med. Aug 8 2002;347(6):401-7. [Medline].

42. Drumm ML, Konstan MW, Schluchter MD, Handler A, Pace R, Zou F. Genetic modifiers of lung disease in cystic fibrosis. N

Engl J Med. Oct 6 2005;353(14):1443-53. [Medline].

43. Li Z, Kosorok MR, Farrell PM, Laxova A, West SE, Green CG. Longitudinal development of mucoid Pseudomonas aeruginosa

infection and lung disease progression in children with cystic fibrosis. JAMA. Feb 2 2005;293(5):581-8. [Medline].

44. Handelsman DJ, Conway AJ, Boylan LM, Turtle JR. Young 's syndrome. Obstructive azoospermia and chronic sinopulmonary

infections. N Engl J Med. Jan 5 1984;310(1):3-9. [Medline].

45. Sturgess JM, Thompson MW, Czegledy-Nagy E, Turner JA. Genetic aspects of immotile cilia syndrome.Am J Med

Genet. Sep 1986;25(1):149-60. [Medline].

46. Noone PG, Leigh MW, Sannuti A, et al. Primary ciliary dyskinesia: diagnostic and phenotypic features.Am J Respir Crit Care

Med. Feb 15 2004;169(4):459-67. [Medline].

47. Lillington GA. Dyskinetic cilia and Kartagener's syndrome. Bronchiectasis with a twist. Clin Rev Allergy Immunol. Aug 2001;

21(1):65-9. [Medline].

48. Morrissey B, Louie S. Allergic bronchopulmonary aspergillosis: an evolving challenge in asthma. In: Gershwin M, Albertson T,

eds. Bronchial Asthma: A Guide for Practical Understanding and Treatment. 5th ed. Totowa, NJ: Humana

Press; 2006:279-309.

49. Vendrell M, de Gracia J, Rodrigo MJ, et al. Antibody production deficiency with normal IgG levels in bronchiectasis of

unknown etiology. Chest. Jan 2005;127(1):197-204. [Medline].

50. De Gracia J, Rodrigo MJ, Morell F, et al. IgG subclass deficiencies associated with bronchiectasis.Am J Respir Crit Care

Med. Feb 1996;153(2):650-5. [Medline].

51. Thickett KM, Kumararatne DS, Banerjee AK, Dudley R, Stableforth DE. Common variable immune deficiency: respiratory

manifestations, pulmonary function and high-resolution CT scan findings. QJM. Oct 2002;95(10):655-62. [Medline].


30/34



52. Notarangelo LD, Plebani A, Mazzolari E, Soresina A, Bondioni MP. Genetic causes of bronchiectasis: primary immune

deficiencies and the lung. Respiration. 2007;74(3):264-75. [Medline].

53. Stover DE, White DA, Romano PA, Gellene RA, Robeson WA. Spectrum of pulmonary diseases associated with the acquired

immune deficiency syndrome.Am J Med. Mar 1985;78(3):429-37. [Medline].

54. McGuinness G, Naidich DP, Garay S, Leitman BS , McCauley DI. AIDS associated bronchiectasis: CT features. J Comput

Assist Tomogr. Mar-Apr 1993;17(2):260-6. [Medline].

55. Jones VF, Eid NS, Franco SM, Badgett JT , Buchino JJ. Familial congenital bronchiectasis: Williams-Campbellsyndrome. Pediatr Pulmonol. Oct 1993;16(4):263-7. [Medline].

56. Woodring JH, Howard RS 2nd, Rehm SR. Congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a report of 10 cases

and review of the literature. J Thorac Imaging. Apr 1991;6(2):1-10. [Medline].

57. Cordasco EM Jr, Beder S, Coltro A, Bavbek S, Gurses H, Mehta AC. Clinical features of the yellow nail syndrome. Cleve Clin

J Med. Jul-Aug 1990;57(5):472-6. [Medline].

58. Wood JR, Bellamy D, Child AH, Citron KM. Pulmonary disease in patients with Marfan syndrome. Thorax. Oct 1984;39(10)

:780-4. [Medline].

59. Shin MS, Ho KJ. Bronchiectasis in patients with alpha 1-antitrypsin deficiency. A rare occurrence?. Chest. Nov 1993;104(5)

:1384-6. [Medline].

60. Chan E, Feldman N, Chmura K. Do mutations of the alpha-1-antitrypsin gene predispose to non-tuberculous mycobacterial

infection?.Am J Respir Crit Care Med. 2004;169:A132.

61. Parr DG, Guest PG, Reynolds JH, Dowson LJ, Stockley RA. Prevalence and impact of bronchiectasis in alpha1-antitrypsin

deficiency.Am J Respir Crit Care Med. Dec 15 2007;176(12):1215-21. [Medline].

62. Cuvelier A, Muir JF, Hellot MF, Benhamou D, Martin JP, Benichou J. Distribution of alpha (1)-antitrypsinalleles in patients with

bronchiectasis. Chest. Feb 2000;117(2):415-9. [Medline].

63. Walker WC. Pulmonary infections and rheumatoid arthritis. Q J Med. Apr 1967;36(142):239-51. [Medline].

64. Perez T, Remy-Jardin M, Cortet B. Airways involvement in rheumatoid arthritis: clinical, functional, and HRCT findings.Am J

Respir Crit Care Med. May 1998;157(5 Pt 1):1658-65. [Medline].

65. McMahon MJ, Swinson DR, Shettar S, Wolstenholme R, Chattopadhyay C, Smith P. Bronchiectasis and rheumatoid arthritis:

a clinical study.Ann Rheum Dis. Nov 1993;52(11):776-9. [Medline].

66. McMahon MJ, Swinson DR, Shettar S, et al. Bronchiectasis and rheumatoid arthritis: a clinical study.Ann Rheum

Dis. Nov 1993;52(11):776-9. [Medline].

67. Swinson DR, Symmons D, Suresh U, Jones M, Booth J. Decreased survival in patients with co-existent rheumatoid arthritis

and bronchiectasis. Br J Rheumatol. Jun 1997;36(6):689-91. [Medline].

68. Robinson DA, Meyer CF. Primary Sjogren 's syndrome associated with recurrent sinopulmonary infections and

bronchiectasis. J Allergy Clin Immunol. Aug 1994;94(2 Pt 1):263-4. [Medline].

69. Casserly IP, Fenlon HM, Breatnach E, Sant SM. Lung findings on high-resolution computed tomography in idiopathic

ankylosing spondylitis--correlation with clinical findings, pulmonary function testing and plain radiography. Br J

Rheumatol. Jun 1997;36(6):677-82. [Medline].

70. Fenlon HM, Doran M, Sant SM, Breatnach E. High -resolution chest CT in systemic lupus erythematosus.AJR Am J

Roentgenol. Feb 1996;166(2):301-7. [Medline].


31/34



71. Tillie-Leblond I, Wallaert B, Leblond D, Salez F, Perez T, Remy-Jardin M. Respiratory involvement in relapsing polychondritis.

Clinical, functional, endoscopic, and radiographic evaluations. Medicine (Baltimore). May 1998 ;77(3):168-76. [Medline].

72. Camus P, Colby TV. The lung in inflammatory bowel disease. Eur Respir J. Jan 2000;15(1):5-10. [Medline].

73. Rockoff SD, Rohatgi PK. Unusual manifestations of thoracic sarcoidosis.AJR Am J Roentgenol. Mar 1985;144(3)

:513-28. [Medline].

74. Tiddens HA. Chest computed tomography scans should be considered as a routine investigation in cystic fibrosis. Paediatr

Respir Rev. Sep 2006;7(3):202-8. [Medline].

75. Young K, Aspestrand F, Kolbenstvedt A. High resolution CT and bronchography in the assessment of bronchiectasis.Acta

Radiol. Nov 1991;32(6):439-41. [Medline].

76. Smith IE, Flower CD. Review article: imaging in bronchiectasis. Br J Radiol. Jul 1996;69(823):589-93. [Medline].

77. Hansell DM. Bronchiectasis. Radiol Clin North Am. Jan 1998;36(1):107-28. [Medline].

78. Chang C, Singleton R, Morris P and et al. Pneumococcal vaccines for children and adults with bronchiectasis. The Cochrane

Database of Systematic Reviews. 2008;3.

79. Chang CC, Morris PS, Chang AB. Influenza vaccine for children and adults with bronchiectasis. Cochrane Database Syst

Rev. 2008;3:CD006218. [Medline].

80. Davies G, Wilson R. Prophylactic antibiotic treatment of bronchiectasis with azithromycin. Thorax. Jun 2004;59(6)

:540-1. [Medline].

81. Tagaya E, Tamaoki J, Kondo M, Nagai A. Effect of a short course of clarithromycin therapy on sputum production in patients

with chronic airway hypersecretion. Chest. Jul 2002;122(1):213-8. [Medline].

82. Yalin E, Kiper N, Ozcelik U, Dogru D, Firat P, Sahin A. Effects of claritromycin on inflammatory parameters and clinical

conditions in children with bronchiectasis. J Clin Pharm Ther. Feb 2006;31(1):49-55. [Medline].

83. Evans DJ, Bara AI, Greenstone M. Prolonged antibiotics for purulent bronchiectasis. Cochrane Database Syst Rev. 2003;(4)

:CD001392. [Medline].

84. Evans DJ, Greenstone M. Long-term antibiotics in the management of non-CF bronchiectasis--do they improve

outcome?. Respir Med. Jul 2003 ;97(7):851-8. [Medline].

85. Rubin BK. Aerosolized antibiotics for non-cystic fibrosis bronchiectasis. J Aerosol Med Pulm Drug Deliv. Mar 2008;21(1)

:71-6. [Medline].

86. Barker AF, Couch L, Fiel SB, et al. Tobramycin solution for inhalation reduces sputum Pseudomonas aeruginosa density in

bronchiectasis.Am J Respir Crit Care Med. Aug 2000;162(2 Pt 1):481-5. [Medline].

87. Bilton D, Henig N, Morrissey B, Gotfried M. Addition of inhaled tobramycin to ciprofloxacin for acute exacerbations of

Pseudomonas aeruginosa infection in adult bronchiectasis. Chest. Nov 2006;130(5):1503-10. [Medline].

88. Haciibrahimoglu G, Fazlioglu M, Olcmen A, Gurses A, Bedirhan MA. Surgical management of childhood bronchiectasis due to

infectious disease. J Thorac Cardiovasc Surg. May 2004;127(5):1361-5. [Medline].

89. Drobnic ME, Sune P, Montoro JB, Ferrer A, Orriols R. Inhaled tobramycin in non-cystic fibrosis patients with bronchiectasis

and chronic bronchial infection with Pseudomonas aeruginosa.Ann Pharmacother. Jan 2005;39(1):39-44. [Medline].

90. Scheinberg P, Shore E. A pilot study of the safety and efficacy of tobramycin solution for inhalation in patients with severe

bronchiectasis. Chest. Apr 2005;127(4):1420-6. [Medline].


32/34



91. Lin H, Cheng H, Wang C, et al. Inhaled gentamicin reduces airway neutrophil activity and mucus secretion in

bronchiectasis.Am J Respir Crit Care Med. 1999;155:2024-2029.

92. Steinfort DP, Steinfort C. Effect of long-term nebulized colistin on lung function and quality of life in patients with chronic

bronchial sepsis. Intern Med J. Jul 2007;37(7):495-8. [Medline].

93. Patterson JE, Hewitt O, Kent L, Bradbury I, Elborn JS, Bradley JM. Acapella versus 'usual airway clearance' during acute

exacerbation in bronchiectasis: a randomized crossover trial. Chron Respir Dis. 2007;4(2):67-74. [Medline].

94. Eaton T, Young P, Zeng I, Kolbe J. A randomized evaluation of the acute efficacy, acceptability and tolerability of flutter andactive cycle of breathing with and without postural drainage in non-cystic fibrosis bronchiectasis. Chron Respir Dis. 2007;4(1)

:23-30. [Medline].

95. Langenderfer B. Alternatives to percussion and postural drainage. A review of mucus clearance therapies: percussion and

postural drainage, autogenic drainage, positive expiratory pressure, flutter valve, intrapulmonary percussive ventilation, and

high-frequency chest compression with the ThAIRapy Vest . J Cardiopulm Rehabil. Jul-Aug 1998;18(4):283-9. [Medline].

96. [Best Evidence] Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC . Mucus clearance and lung

function in cystic fibrosis with hypertonic saline. N Engl J Med. Jan 19 2006;354(3):241-50. [Medline].

97. [Best Evidence] Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB. A controlled trial of long -term inhaled

hypertonic saline in patients with cystic fibrosis. N Engl J Med. Jan 19 2006;354(3):229-40. [Medline].

98. Wills P, Greenstone M. Inhaled hyperosmolar agents for bronchiectasis. Cochrane Database Syst Rev. 2008;3.

99. Fuchs HJ, Borowitz DS, Christiansen DH , et al. Effect of aerosolized recombinant human DNase on exacerbations of

respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J

Med. Sep 8 1994;331(10):637-42. [Medline].

100. Paul K, Rietschel E, Ballmann M, Griese M, Worlitzsch D, Shute J. Effect of treatment with dornase alpha on airway

inflammation in patients with cystic fibrosis.Am J Respir Crit Care Med. Mar 15 2004;169(6):719-25. [Medline].

101. O'Donnell AE, Barker AF, Ilowite JS , Fick RB. Treatment of idiopathic bronchiectasis with aerosolized recombinant human

DNase I. rhDNase Study Group. Chest. May 1998;113(5):1329-34. [Medline].

102. Franco F, Sheikh A, Greenstone M. Short acting beta-2 agonists for bronchiectasis. Cochrane Database Syst Rev. 2003;(3)

:CD003572. [Medline].

103. Sheikh A, Nolan D, Greenstone M. Long-acting beta-2-agonists for bronchiectasis. Cochrane Database Syst Rev. 2001;(4)

brochiectasis

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