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Bronchiectasis is "irreversible, abnormal dilatation of the bronchi." [1, 2] Common etiologies for
bronchiectasis are congenital abnormalities, recurrent infection, and airway obstruction. The
tethering effect of pulmonary fibrosis can also dilate the airways, causing traction bronchiectasis.
[3, 4, 5]
Involved bronchi are dilated, inflamed, and easily collapsible, resulting in airflow
obstruction and impaired clearance of secretions.
Bronchiectasis is a final common pathway for many diseases with diverse fundamental causes.
Knowledge of the root cause of bronchiectasis in a particular patient is more helpful for
treatment than an understanding of the generic subject of bronchiectasis. Bronchiectasis is
associated with a wide range of disorders, but it usually results from necrotizing bacterial
infections, such as infections caused by the Staphylococcus or Klebsiella species or Bordetella
pertussis. Unfortunately, in about half the cases, the specific cause of bronchiectasis remains
undetermined. [6]
Hemoptysis is common and may occur in as many as 50% of patients. Episodic hemoptysis with
little to no sputum production (dry bronchiectasis) is usually a sequela of tuberculosis. However,
massive hemoptysis may occur; bleeding usually originates in dilated bronchial arteries, which
contain blood at systemic (rather than pulmonary) pressures.
Diagnosis of bronchiectasis is based on a clinical history of daily viscid sputum production and
characteristic computed tomography (CT) scan findings. (See the images below.) [7, 8]
bronchiectasis.
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Pathophysiology
Bronchiectasis is not caused by a single factor, but is a complex interplay between repeated
inflammation and a defective immune response to inflammation. [9] Many congenital or acquired
abnormalities can prevent normal airway defense and repair, resulting in infection. If the initial
reaction to infection is ineffective, the immune response is increased and prolonged, potentially
causing a vicious circle of bronchial injury and increased inflammation that leads to
bronchiectasis. [10] Bronchial damage includes variable amounts of bronchomalacia or fibrosis
and disturbance of mucociliary function.
Examples of defective cellular or humoral immunity that predispose to bronchiectasis occur all
along the immune response pathway. King et al found that adult patients with bronchiectasis had
significantly lower levels of immunoglobulin G3 (IgG3), B-cell lymphocytes, and T-helper
lymphocytes. [11] In about one third of adult bronchiectasis patients, King et al also found a
significantly diminished neutrophil oxidative burst. [11] Numerous examples of diminished
immunity have been cited to account for susceptibility to bronchiectasis, including primary
immune deficiencies, positive HIV status, female sex, rheumatoid arthritis, and ulcerative colitis.
[6, 12, 13, 14, 15]
Ordinarily, pathogens are trapped by bronchial mucus and removed by the mucociliary elevator.
Some patients, such as patients with cystic fibrosis or Young syndrome, have an altered mucous
viscosity that impedes effective mucous removal. [20, 21] Others, such as patients with primary
ciliary dyskinesia, have a defective anatomic mechanism for mucous clearance. [22, 23]
Altered anatomy can play a role in recurrent infections. A congenital abnormality that affects
normal anatomy, such as communicating bronchopulmonary foregut malformation, tracheal
bronchus, or accessory cardiac bronchus can precipitate bronchiectasis. [24] Patients with
Williams-Campbell syndrome have a congenital cartilage defect that predisposes them to
bronchiectasis, [25] while patients with Mounier-Kuhn syndrome (tracheobronchomegaly) have
deficient tracheobronchial smooth muscle and elastic fibers. [26]
Inhalational lung injury from smoke or other noxious gases often is the initial event in the cycle
ending in bronchiectasis. [27] Central bronchial obstruction can be caused by foreign body,
broncholith, tumor, or lymphadenopathy, leading to bronchiectasis. [28, 29, 30] Patients with end-
stage fibrosis can develop traction bronchiectasis and bronchiolectasis. Common examples
include usual interstitial pneumonitis, tuberculosis, sarcoidosis, and radiation fibrosis. High
negative pleural pressures and increased elastic recoil in pulmonary fibrosis interact to enlarge
the bronchi. [31] The dilated bronchi are typically distorted with a beaded appearance. [32]
Depending on the distribution of fibrosis, traction bronchiectasis can be regional or widespread.
Traction bronchiectasis is restricted to the areas of end-stage fibrosis and particularly affects
peripheral bronchi, which lack cartilage support. [31]
Epidemiology
Incidence in the United States
In adults, the prevalence of bronchiectasis trends upward with increasing age. [33, 34] The
prevalence of bronchiectasis in patients aged 65 years and older is increasing over time, possibly
related to improved diagnosis and/or access to care. [35]
Weykert et al used a retrospective cohort design to estimate the prevalence of bronchiectasis in
US adults. [33] They found a prevalence of bronchiectasis ranging from 4.2 cases per 100,000
persons (18-34 y) to 271.8 cases per 100,000 persons (75 y and older). [33]
Seitz, et al evaluated a sample of Medicare claims from patients aged 65 years and older.
Incidence internationally
Contrary to the case in the Western world, bronchiectasis is regarded as common in the East,
though prevalence statistics are sparse. [36] Although the prevalence of pediatric bronchiectasis
has generally been declining in the more developed world, numerous regional studies suggest
that pediatric bronchiectasis remains a significant problem in the developing world. [37] Chang et
al found a particularly high prevalence of bronchiectasis in aboriginal children aged 15 years and
younger in Central Australia of 14.7 cases per 1,000 persons. [38] In a health screening program
for adults in Seoul, Korea, the prevalence of bronchiectasis was 9.1%. [39]
Race-related incidence
For whites, cystic fibrosis is the most common life-threatening recessive genetic problem. [40]
Cystic fibrosis occurs in about 1 in 3,200 white live births, 1 in 15,000 African American live
births, and 1 in 31,000 Asian American live births. [40]
Sex-related incidence
Overall, bronchiectasis unrelated to cystic fibrosis occurs more frequently and is more severe in
women. [15] Although cystic fibrosis has an equal distribution between males and females, the
diagnosis may be delayed in females, which could explain why females tend to die younger. [43]
Rheumatoid arthritis, [44] sarcoidosis, [45] and Sjgren syndrome all have a female predominance
and predispose to bronchiectasis.
Mycobacterium avium complex has generally been found more frequently in older women. [42, 48,
49, 50]
Women in the United States are diagnosed with nontuberculous mycobacterial disease 1.4
times more frequently than men among Medicare patients older than 65 years. [42] With various
study methodologies and geographic locations, sex ratios for nontuberculous mycobacterial
disease can vary considerably. [51]
Age-related incidence
In adults, the prevalence of bronchiectasis trends upward with increasing age. [33, 34] A review of
nontuberculous mycobacteria-associated hospitalizations showed a significant upward trend with
increasing age for males and females. [52]
Mortality/morbidity
Pediatric bronchiectasis in the developing world carries a higher rate of morbidity and mortality,
at least partially related to poorer nutrition. [37] Hemoptysis and chronic respiratory failure are
also more common in the developing world. [37]
Quality of life can be assessed by the St Georges Respiratory Questionnaire or the Leicester
Cough Questionnaire. [53] Frequency of exacerbations of symptoms can be used to judge
effectiveness of treatment, and exercise capacity can be evaluated by the incremental shuffle
walk test or the 6-minute walking test. [53]
Mortality risk is correlated with numerous demographic, pulmonary function, and quality-of-life
variables. [54] CT imaging variables with the highest mortality risk include extent of
bronchiectasis, severity of dilatation, and bronchial wall thickness. [54] Surgical mortality for
bronchiectasis ranges from 0-2.2% and morbidity is 18-23%. [55]
Patients with cystic bronchiectasis are at risk for developing a mycetoma within a dilated
bronchus.
Hemoptysis is common and may occur in as many as 50% of patients. Episodic hemoptysis with
little to no sputum production (dry bronchiectasis) is usually a sequela of tuberculosis. However,
massive hemoptysis may occur; bleeding usually originates in dilated bronchial arteries, which
contain blood at systemic (rather than pulmonary) pressures.
Anatomy
Main anatomic types of bronchiectasis are as follows [2] :
Cylindrical
Varicoid
Saccular
[57]
Mounier-Kuhn syndrome First to fourth order bronchi
Clinical details
The classic clinical presentation for a patient with bronchiectasis is intermittent or daily, often
purulent, sputum production. This is often accompanied by other symptoms such as dyspnea,
fatigue, weight loss, chest pain, hemoptysis, and clubbing of the fingers. [59, 60, 61, 62, 63] Patients may
develop respiratory failure or right-sided heart failure. [59] This clinical presentation should
prompt further investigation by chest CT.
Differentials
Alpha1-Antitrypsin Deficiency
Aspiration Pneumonia
Asthma
Bacterial Pneumonia
Bronchitis
Emphysema
Tuberculosis
Preferred examination
Chest radiography is usually the first imaging examination, but the findings are often nonspecific
and the images may appear normal. [1] High-resolution computed tomography (HRCT) scanning
recently was the imaging modality of choice for demonstrating or ruling out bronchiectasis and
its extent (see the images below). HRCT scanning also helps clinicians to evaluate the status of
the surrounding lung tissue and exclude other lesions such as neoplasms. [2]
ectatic bronchi. The patient has cystic fibrosis, which was diagnosed in and treated
since childhood.
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This high-resolution computed tomography scan through the upper lung
bronchiectasis.
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This high-resolution computed tomography scan in a 13-year-old female
fluid-filled dilated bronchi in a 65-year-old man with bronchiectasis in the left lower
lobe.
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Bronchography was the classic modality used and, until the advent of HRCT scanning, was the
only imaging method to demonstrate bronchiectasis. Bronchography is performed by instilling
an iodine-based contrast material via a catheter or bronchoscope, but it is rarely, if ever,
performed today, as HRCT scanning has replaced it as the diagnostic modality of choice. HRCT
scanning is noninvasive and has a sensitivity of 96% and a specificity of 93%. [3]
Volumetric multidetector CT acquisition of the chest is now the preferred examination for the
diagnosis of bronchiectasis. Compared with the previous criterion standard of high-resolution
CT (HRCT), volumetric CT offers several improvements. [64, 65]
Equivalent reconstructed HRCT images can be generated from the volumetric data set without
additional scanning. [66] In addition, volumetric CT allows multiplanar reconstructions, which
improve assurance of diagnosis, at least partially by permitting better assessment of absence of
tapering of bronchi. [67, 68] By including the entire lung volume, volumetric CT increases the
sensitivity of detection for smaller regions of bronchiectasis and permits more confident
exclusion of the diagnosis. [64, 65, 68, 69]
Volumetric CT can be acquired in a single breath-hold. This reduces breathing artifact, which
can be confused with bronchiectasis. [64, 70] Since the initial demonstration of improvement in
diagnostic accuracy with multidetector row CT, CT technology has progressed remarkably with
the addition of more detectors and substantial improvements in scanner speed. These
improvements have solidified the superiority of volumetric CT as the preferred examination.
Although volumetric CT has numerous advantages, the radiation dose is higher. Radiation dose
can be mitigated by several techniques. [71] This is particularly important in pediatric patients,
because of their increased radiation sensitivity. Radiologists should use modified protocols
especially suited for children. [71, 72] Limited HRCT imaging could be appropriate in some patients
for follow-up imaging in order to reduce the radiation dose.
The premise of a preferred examination for bronchiectasis implies that the requesting physician
already knows the diagnosis, but bronchiectasis is only definitively diagnosed on chest CT.
Bronchiectasis could be the suspected diagnosis triggering the request. However, as a practical
matter, physicians request examinations in response to patient presentations, relying on the
radiologist to protocol the examination to differentiate among possible conditions that could
present similarly. [73, 74, 75, 76, 77] Most chest CT protocols are likely to diagnose bronchiectasis, if
present. Moreover, unsuspected bronchiectasis is often diagnosed on chest CT obtained for
unrelated reasons. Once bronchiectasis is diagnosed, follow-up chest CT can be tailored for
optimal assessment and follow-up of bronchiectasis, along with any other conditions.
Limitations of techniques
In general, if a volumetric study of the chest is obtained without significant motion artifact in an
adult patient, there should be no important limitation to the identification or exclusion of
bronchiectasis. However, the operational definition of bronchiectasis is "irreversible, abnormal
dilatation of the bronchi." [1, 2] Reversible bronchial dilatation has been identified rarely in adults.
[78]
Gaillard et al reviewed follow-up CT examinations in 22 noncystic fibrosis pediatric patients
with bronchial dilatation. In 6 patients, the bronchial dilatation resolved completely and in 8
patients there was improvement. [79] For patients with complete reversal of bronchial dilatation, a
single study would be insufficient.
Chest radiographs may be negative in patients with minor to moderate disease. Many abnormal
radiographic findings may be nonspecific, and confirmation using HRCT scanning may be
required.
Bronchography is rarely indicated because it is invasive and is associated with allergic reactions
to the contrast material. Bronchography also carries the risk of acute bronchoconstriction.
Radiography
Chest radiography helps to identify serious disease, and it was once the standard imaging
modality. [4] However, the radiographs may depict no abnormalities, or the findings may be
nonspecific in patients with less-severe disease. [5]
Various abnormal radiographic findings have been described as follows (see the images below)
[80, 81, 82]
:
Parallel line opacities (tram tracks) caused by dilated, thickened bronchi are
seen along their length
Increased size and loss of definition of the pulmonary vessels in the affected
areas as a result of peribronchial fibrosis
old woman with chronic cough since childhood. Nodules are present in the
left upper lung; the right upper lung was similarly involved.
chest radiograph shows overaeration and increased markings over the heart.
Bronchography
Introduced in 1922, bronchography was the investigation of choice until the introduction of
HRCT scanning in the mid 1980s. Currently, bronchography is rarely used. Bronchography is
performed by instilling contrast material via a catheter or bronchoscope under fluoroscopic
control and plain radiographic imaging. The procedure is unpleasant for the patient and is also
associated with temporary impairment of ventilation, as well as allergic and foreign body
reactions to the contrast medium. In addition, interpretation of bronchographic images is
difficult, owing to underfilling and retained secretions.
Degree of confidence
The accuracy of plain radiographic findings in the diagnosis of bronchiectasis is unknown,
because the findings are variable and nonspecific and depend on the severity and extent of the
bronchiectasis. However, good correlation exists between the severity of disease as seen on plain
images and HRCT scans. Chest radiographic findings may be normal or nonspecific in patients
with less severe disease. The prevalence of signs of bronchiectasis at radiography correlates with
the severity of bronchiectasis at HRCT, although radiography is less sensitive and less specific.
[83]
False positives/negatives
Many plain radiographic findings are nonspecific and may be seen in patients with idiopathic
pulmonary fibrosis, sarcoidosis, histiocytosis X, rheumatoid lung, and other chronic interstitial
lung disorders.
Computed Tomography
On HRCT scans in patients with bronchiectasis, the internal bronchial diameter may be greater
than that of the adjacent artery, and there may be a lack of bronchial tapering (the same diameter
as the parent branch for >2 cm). The bronchi may be within 1 cm of costal pleura or abut the
mediastinal pleura (more specific but less sensitive than an increased ratio), and bronchial wall
thickening may be seen (in 68% of patients). A cystic cluster of thin-walled cystic spaces may be
present, often with air-fluid levels. (See the image below.)
bronchiectasis.
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In cylindrical bronchiectasis, bronchi coursing horizontally are seen as parallel lines, and
vertically oriented bronchi are seen as circular lucencies that are larger than the adjacent
pulmonary artery (signet-ring appearance). (See the image below.)
Varicose bronchiectasis may be seen as nonuniform bronchial dilatation. Other findings include
the following:
Tracheomegaly
Fluid-filled bronchi are revealed as tubular or branching structures when they course
horizontally or are revealed as nodules when they are perpendicular to the plane of the CT scan
section (see the image below).
fluid-filled dilated bronchi in a 65-year-old man with bronchiectasis in the left lower
lobe.
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CT indications of bronchiectasis include the following [1, 58, 84, 85, 86] :
Bronchial thickening
Air trapping
Degree of confidence
Except for extremely obese patients and examinations compromised by motion, volumetric
imaging of the chest provides a very high degree of confidence to confirm or deny the diagnosis
of bronchiectasis. HRCT scanning has a sensitivity of 96% and a specificity of 93%, [3] as
compared with bronchography.
Bronchial measurements may vary with the use of different WLs and WWs. [19]
Some patients without bronchiectasis have a 1.49:1 bronchus-to-artery ratio; however, the ratio
is reliable only if it is greater than 1.5. If the ratio is less than 1.5, other signs, such as bronchial
wall thickening and lack of tapering, should be present for the diagnosis of bronchiectasis.
Bronchial wall thickening is optimally seen with a WW of 1000 HU and a WL of 700 HU;
higher WL and other WW readings are associated with artifactual wall thickening. [20] This
finding is not specific and is also seen in patients with asthma and in those who smoke.
False positives/negatives
The variability of the bronchus-to-artery ratio at high altitudes and in patients with pulmonary
hypertension may result in an overdiagnosis because of vasoconstriction in these conditions. The
bronchial diameter relative to the adjacent pulmonary artery also increases with increasing
altitude. [88]
In patients with consolidation, dilated bronchi may not be seen. Cardiac and respiratory artifacts
may obscure the results or mimic subtle bronchiectasis in the left lower lobe. Rarely,
histiocytosis X and cavitating pulmonary masses mimic cystic bronchiectasis. Traction
bronchiectasis occurs in patients with interstitial fibrosis and results from fibrous tethering of the
bronchial wall. Traction bronchiectasis is not a true bronchial disorder.
The patients age should be considered, since the bronchoarterial ratio increases with age. [89] In
rare instances in adults, but more frequently in pediatric patients, bronchiectasis can be
reversible.
A dilated, cystic bronchus should be distinguished from a bulla, since a bronchial cyst has a
perceptible wall, while a bulla does not.
At times, severely dilated bronchi with associated volume loss can simulate honeycombing. [84]
While HRCT remains the primary mode of assessment for bronchiectasis in adults, for cystic
fibrosis patients and for young patients who may need repeated follow-up examinations, MRI
yields useful information.
Teufel et al compared HRCT and 1.5-Tesla MRI using a very short echo time in 51 patients with
cystic fibrosis. For these patients, both CT and MRI were able to detect bronchiectasis, mucous
plugging, and peribronchial thickening, with MRI strongly correlating with the CT findings. [93]
Montella et al compared chest MRI with HRCT in 50 noncystic fibrosis pediatric patients, and
both modalities demonstrated bronchiectasis in approximately 72% of those subjects. Other
findings in chronic pediatric lung disease were also assessed, with results supporting the use of
chest MRI as a reasonable alternative to CT. [94]
Although CT has better spatial resolution and shows morphology in more detail than MRI, MRI
is superior for assessment of functional changes of altered hemodynamics and perfusion. [90] ] [3]
He MRI may be used for evaluation of pulmonary ventilation and function. [] [95]
Degree of confidence
Degree of confidence of MRI depends on many factors, such as study protocol, image artifacts,
and severity and location of disease.
False negatives/positives
Multiple imaging sequences may be necessary to discriminate partial mucous plugging from a
severely thickened bronchial wall. [90]
Nuclear Imaging
Patients with bronchiectasis can suffer from chronic productive cough, recurrent infections, and
hemoptysis. V/Q scanning can be useful in determining whether surgical resection is appropriate
therapy, especially for hemoptysis. In one series, 23 of 66 patients treated surgically had
hemoptysis as a symptom. V/Q scanning demonstrated undiminished perfusion in cylindrical
bronchiectasis, but areas of cystic or mixed cystic and cylindrical bronchiectasis showed
perfusion defects. If a patient has a scan showing less than 10% perfusion of a bronchiectatic
region, those patients can benefit from surgical resection of that nonfunctional region. [96]
Degree of confidence
The purpose of a V/Q scan is to determine perfused versus nonperfused areas of lung rather than
to make a diagnosis of bronchiectasis. Different diseases can cause nonperfused areas of lung, so
V/Q is used in conjunction with CT or MRI.
Angiography
The vasculature, pulmonary parenchyma, and airways can be assessed with Multidetector CT
angiography. In disorders with chronic lung inflammation, including bronchiectasis, abnormal
collateral systemic vessels form in the affected parts of the lung. These collateral bronchial
arteries appear as tortuous vessels and can bleed. Occasionally, nonbronchial systemic arteries or
pulmonary arteries bleed. Multiplanar reformatted images are used for identifying the origins
and courses of these vessels.
Bronchiectasis, chronic bronchitis, lung malignancy, tuberculosis, and chronic fungal infection
are some of the most common underlying causes of hemoptysis and are easily detected with CT
angiography.
Results from multidetector CT angiography can be used to direct therapeutic angiography for
bronchial or pulmonary arterial embolization or surgical resection.
Degree of confidence
Occasionally, an examination is limited by artifact from patient motion, data depletion from a
very large patient, or timing of contrast bolus. Otherwise, multidetector row CT usually
demonstrates the vasculature and the pulmonary parenchyma well.
False positives/negatives
No large series using current CT techniques has been published for hemoptysis assessment. In a
series of 22 patients, using 16-detector row CT, bronchial (100%) and nonbronchial (62%)
arteries causing hemoptysis were visible, with most traceable throughout their length. [98]
Substantial technological advances in CT since then allow more detailed visualization.