eMedicine Specialties > Pulmonology > Obstructive Airways Diseases
Bronchiectasis
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).
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 patients63,64,65,66 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.
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Overview: Bronchiectasis |
| Differential Diagnoses & Workup: Bronchiectasis |
| Treatment & Medication: Bronchiectasis |
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Further Reading
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
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






Overview: Bronchiectasis