eMedicine Specialties > Pulmonology > Obstructive Airways Diseases

Bronchiectasis

Ethan E Emmons, MD,, 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 diffuse process 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 bronchial damage, 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).

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).

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 parenchymal damage (see Media File 3).

Varicose bronchiectasis with alternating areas of bronchial dilatation and 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

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.¹⁶ A 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 bronchiectasis—again 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 is 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.
• 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, or Mycoplasma 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 prominent,²⁶ 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.
• 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).^32,33
  • Although 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).
  • 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 of gastroesophageal reflux is a risk factor for aspiration and that the organism Helicobacter pylori may 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 seen 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, nasal polyps 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 of Aspergillus species. The resulting bronchiectasis is thin-walled and affects the central and medium-sized airways.
  • CT scanning of the chest exhibits central airway bronchiectasis, differentiating this condition from other causes of bronchiectasis.
  • Other features of ABPA include eosinophilia, elevated immunoglobulin E (IgE) levels, and dramatic responses to corticosteroids.
• Immunodeficiency states
  • Immunodeficiency states may occur in the setting of congenital and acquired immunodeficiency. The most common congenital conditions (albeit rare) involve B-lymphocyte functions, specifically hypogammaglobulinemia. The latter may involve an immunoglobulin G (IgG) subclass deficiency; X-linked agammaglobulinemia; or selective immunoglobulin A (IgA), immunoglobulin M (IgM), or IgE deficiency.^49,50,51,52
  • Patients with hypogammaglobulinemia usually present in childhood with repeated sinus or pulmonary infections, although it has been diagnosed in adults who did not have a history of repeated infections. Establishing the diagnosis is important because gammaglobulin replacement may reduce the number of infections and resultant lung injury.
  • HIV disease, with resultant acquired immunodeficiency syndrome (AIDS), has been implicated in the development of bronchiectasis and demonstrates the accelerated bronchial damage that may occur from repeated infections in patients who are immunosuppressed. Bronchiectasis in HIV infection has occurred with and without obvious preceding pulmonary infection and may occur secondary to immunologic dysfunction from the HIV disease itself.^31,53,54
• Congenital anatomic defects and connective-tissue disorders
  • Bronchopulmonary sequestration is a congenital abnormality classified as either intralobar or extralobar and results in chronic lower respiratory tact infections that lead to bronchiectasis.
  • Williams-Campbell syndrome (congenital cartilage deficiency) is the absence of cartilage from lobar to first- to second-generation segmental airways that results in extensive peripheral bronchiectasis.⁵⁵
  • Mounier-Kuhn syndrome (tracheobronchomegaly) is a rare disorder characterized by dilation of the trachea and segmental bronchi (central bronchiectasis).⁵⁶
  • Swyer-James syndrome (unilateral hyperlucent lung) likely is a developmental disturbance that leads to unilateral bronchiolitis, hyperinflation, and, in some cases, bronchiectasis.
  • Yellow-nail syndrome is rare. It results in exudative pleural effusions.⁵⁷
  • Marfan syndrome is a connective-tissue disorder in which the general consensus is weakness of the connective tissue of the bronchial wall predisposes to bronchiectasis.⁵⁸
• Alpha1-antitrypsin (AAT) deficiency⁵⁹
  • Bronchiectasis has been noted to occur in this rare condition, both in patients with true AAT deficiency and in patients with heterozygous phenotypes.^60,61,62
  • The pathogenesis of bronchiectasis in this setting is unclear, but it is believed that the AAT abnormalities make patients more susceptible to respiratory tract infections and subsequent bronchial damage.
• Autoimmune diseases and idiopathic inflammatory disorders
  • Rheumatoid arthritis is associated with bronchiectasis in a reported 3.2-35% of patients^63,64,65,66 and, in one series, was associated with an unfavorable prognosis.⁶⁷ The pathology of bronchiectasis may be increased susceptibility to infections in these patients. Pulmonary disease may occur prior to the onset of the rheumatic process.
  • With Sjögren syndrome, bronchiectasis has been noted in these patients and may be secondary to increased viscosity of mucus with poor airway clearance.⁶⁸
  • Ankylosing spondylitis is associated with bronchiectasis, but in small numbers.⁶⁹
  • Systematic lupus erythematosus may present with a variety of pulmonary pathology, including bronchiectasis, which was reported in 21% of patients in one series.⁷⁰
  • In relapsing polychondritis, bronchiectasis appears to be secondary to primary bronchial damage with resultant recurrent infection.⁷¹
  • With inflammatory bowel disease, bronchiectasis has been seen in both ulcerative colitis and Crohn disease. The etiology remains unclear. Pulmonary symptoms may occur prior to the onset of bowel disease.⁷²
  • 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

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 suggests Aspergillus 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 cause of 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 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.

This CT scan depicts areas of both cystic bronchiectasis and varicose bronchiectasis.

• Its reported sensitivity and specificity are 84-97% and 82-99%, respectively, although it may be higher at referral centers.⁷⁷
• Additional advantages of HRCT scanning include noninvasiveness, avoidance of possible allergic reactions to contrast media, and information regarding other pulmonary processes.
• The 3 forms of bronchiectasis in the Reid classification can be visualized by HRCT.⁶
  • Cylindrical bronchiectasis has parallel tram track lines, or it may have a signet-ring appearance composed of a dilated bronchus cut in a horizontal section with an adjacent pulmonary artery representing the stone.
  • Observe the ratio of size of the bronchus lumen versus the size of its adjacent vessel. A luminal airway diameter 1-1.5 times the adjacent vessel is normal. A diameter greater than 1.5 times the adjacent vessel is suggestive of bronchiectasis.
  • Varicose bronchiectasis has irregular or beaded bronchi, with alternating areas of dilatation and constriction.
  • Cystic bronchiectasis has large cystic spaces and a honeycomb appearance. These cystic changes are in contrast to the blebs of emphysema, which have thinner walls and are not accompanied by proximal airway abnormalities.
• Also see the eMedicine Radiology article Bronchiectasis.

Other Tests

• Pulmonary function tests
  • Pulmonary function test results may be normal or abnormal and may reflect underlying comorbidities as well as providing information regarding predisposing conditions.
  • They are useful in obtaining a functional assessment of the patient, as well as allowing for objective determination of the deterioration of a patient's pulmonary function when baseline studies are available.
  • The most common abnormality is an obstructive airway defect. This may even be found in patients without a prior smoking history.
  • Obstruction is not usually reversible with bronchodilator therapy, although a subgroup of patients may develop hyperreactive airways in conjunction with their bronchiectasis that will respond to bronchodilators.
  • Restriction may be observed in patients with severe advanced disease secondary to scarring and atelectasis, but this is not common. Traction bronchiectasis most often occurs in the setting of a restrictive lung defect from underlying fibrosis.
• Electron microscopy: Perform electron microscopic examination of sperm and respiratory epithelium to observe for evidence of primary ciliary structural abnormalities and dyskinesia.

Procedures

• Bronchography, although once common, is now used rarely, having been replaced by HRCT scanning.⁷⁵
  • Bronchography is performed by instilling contrast material via a catheter or a bronchoscope and performing plain radiographic imaging. It should only be performed at facilities and by operators skilled in its use.
  • In current practice, it is only of potential value in confirming the location of focal bronchiectasis and in excluding disease elsewhere in the setting of possible surgical resection.
  • This procedure carries the risk of acute bronchoconstriction.
• Bronchoscopy is generally not helpful in diagnosing bronchiectasis, but it may be useful in identifying underlying abnormalities, such as tumors, foreign bodies, or other lesions. Bronchoscopy with bronchoalveolar lavage may be used to obtain specimens for staining and culture when a primary infectious etiology or a secondary infection is suspected.

Treatment

Medical Care

The goals of therapy are to improve symptoms, to reduce complications, to control exacerbations, and to reduce morbidity and mortality. Early recognition is essential in bronchiectasis and associated conditions. Additionally, management of underlying conditions, which may include the use of intravenous immunoglobulin or intravenous AAT therapy, is essential to the overall treatment.

Antibiotics and chest physiotherapy are the mainstay modalities. Other modalities (beyond those for specific associated conditions) may include bronchodilators, corticosteroid therapy, dietary supplementation, and oxygen or surgical therapies. Admitting patients with severe exacerbations of bronchiectasis to the hospital and treating them with intravenous antibiotics, bronchodilators, aggressive physiotherapy, and supplemental nutrition is not uncommon.

Available treatment guidelines include Chronic cough due to bronchiectasis: ACCP evidence-based clinical practice guidelines and Pulmonary rehabilitation: joint ACCP/AACVPR evidence-based clinical practice guidelines.

• General therapy
  • Patients should stop smoking.
  • Patients should avoid second-hand smoke.
  • Patients should have adequate nutritional intake with supplementation, if necessary.
  • Immunizations for influenza and pneumococcal pneumonia are recommended.^78,79
  • Immunizations for measles, rubeola, and pertussis should be confirmed.
  • Oxygen therapy is reserved for patients who are hypoxemic with severe disease and end-stage complications, such as cor pulmonale.
  • Patients with CF should be cared for at specialized CF treatment centers that address all aspects of the disease, including nutritional and psychologic aspects.
• Antibiotics
  • 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 clarithromycin^81,82 ), 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 daily antibiotics for 7-14 days of each month, alternating antibiotics for 7-10 days with antibiotic-free periods of 7-10 days, or a long-term daily dose of antibiotics. For patients with severe CF and bronchiectasis, intermittent courses of intravenous antibiotics are sometimes used.^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 devices,^93,94 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 corticosteroids,¹⁰⁵ oral corticosteroids,¹⁰⁶ leukotriene inhibitors,¹⁰⁷ and nonsteroidal anti-inflammatory agents¹⁰⁸ have all been examined. Although evidence tends to support 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 infections,¹⁰⁹ 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 sites 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 or Aspergillus species infections
• Complications of surgical intervention include empyema, hemorrhage, prolonged air leak, and persistent atelectasis.
• Patient selection plays an important role in perioperative mortality rates, which may be as low as 1% in the surgical treatment of segmental or even multisegmental bronchiectasis.
• 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 this article 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 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 <25 mL/min; give half dose or increase dosing interval if CrCl <30 mL/min; diarrhea may be sign of pseudomembranous colitis; superinfections may occur with prolonged or repeated antibiotic therapies

Azithromycin (Zithromax)

An azalide, a subclass of the macrolide antibiotics. Following oral administration, absorbed rapidly and widely distributed throughout body. Mechanism of action is interference with microbial protein synthesis. Effective against a wide range of organisms, including the most common gram-positive and gram-negative organisms. Has additional coverage of so-called atypical infections, such as Chlamydia, Mycoplasma, and Legionella species. Indicated for treatment of patients with mild-to-moderate infections, including acute bronchitic infections that may be observed with bronchiectasis.

Dosing

Adult

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 but with 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 of Streptococcus 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: Not recommended
>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 <2 mo

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

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 or hepatic 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: Not recommended
>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, causing breakthrough 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 drug serum 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%.

Dosing

Adult

500 mg PO/IV qd

Pediatric

<18 years: Not recommended
>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: Not recommended
>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

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; non–dialysis-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

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: Not established
>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

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: Not established
>12 years: Administer as in adults

Interactions

Do not use other sympathomimetic aerosol bronchodilators concomitantly with albuterol; effects on vascular system may be potentiated 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 by Tsang et al showed benefit of inhaled fluticasone in patients with chronic P aeruginosa infection and bronchiectasis.¹⁰⁹ The optimal 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 decrease number 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

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

<2 years: 12 mg/kg/d PO in 6 divided doses
2-6 years: 300 mg PO q12h; not to exceed 600 mg/d
6-12 years: 600 mg PO q12h; not to exceed 1200 mg/d
>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

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.
  • 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.

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.

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

References

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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.

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Keywords

bronchiectasis, bronchial dilatation, dry bronchiectasis, bronchial dilation, dilated bronchus, dilated bronchi, dilatated bronchus, dilatated bronchi, infected bronchi, bronchial infection, chronic obstructive pulmonary lung disease, COPD, bronchial tree infection, cylindrical bronchiectasis, cystic bronchiectasis, varicose bronchiectasis, bronchitis, chronic bronchitis, pulmonary infection, bronchial obstruction, cystic fibrosis, CF, Young syndrome, primary ciliary dyskinesia, allergic bronchopulmonary aspergillosis, bronchopulmonary aspergillosis, alpha-1 antitrypsin deficiency, alpha1-antitrypsin deficiency, rheumatic disease, traction bronchiectasis, congenital bronchiectasis, hemoptysis, bronchial obstruction, chronic aspiration

Contributor Information and Disclosures

Author

Ethan E Emmons, MD,, Chief, Pulmonary Disease and Critical Care Medicine, Brooke Army Medical Center
Ethan E Emmons, MD, is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Medical Editor

Helen M Hollingsworth, MD, Director, Adult Asthma and Allergy Services, Associate Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, Boston Medical Center
Helen M Hollingsworth, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, and Massachusetts Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Daniel R Ouellette, MD, FCCP, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Pulmonary Disease and Critical Care Medicine Service, Henry Ford Health System
Daniel R Ouellette, MD, FCCP is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society
Disclosure: Boehringer Ingleheim Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

I would like to acknowledge my wife and children who everyday motivate me to be a better man and father.

Clinical trials

• Bronchiectasis and Long Term Azithromycin Treatment
• A Phase II , Placebo-Controlled Study to Assess Efficacy of 28 Day Oral AZD9668 in Patients With Bronchiectasis
• Evaluation of the Quality of Life Questionnaire-Bronchiectasis in Patients With Bronchiectasis
• A Study to Determine the Safety and Tolerability of Arikace™ Versus Placebo in Patients Who Have Bronchiectasis
• The Long Term Effect of Inhaled Hypertonic Saline (6%) in Patients With Non Cystic Fibrosis Bronchiectasis

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