BRONCHIECTASIS

Getnet Alemu, MD
HUCHS
Feb 3, 2012

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DEFINITION

 Bronchiectasis is an abnormal & permanent dilatation of bronchi

 Itmay be either focal, involving airways supplying a limited

region of pulmonary parenchyma, or diffuse, involving airways
in a more widespread distribution

 Itis a disorder that typically affects older individuals;

approximately two-thirds of patients are women

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 PATHOLOGY

 Is associated with destructive & inflammatory changes in the walls

of medium-sized airways

 Airway inflammation is mediated by neutrophils & results in up-

regulation of enzymes … elastase & matrix metalloproteinases

 The structural components of the wall, including cartilage, muscle,

& elastic tissue, are destroyed & replaced by fibrous tissue

 The dilated airways frequently contain pools of thick, purulent

material, and the more peripheral airways are often occluded by
secretions or obliterated and replaced by fibrous tissue

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 PATHOLOGY … contd

 Additional
features include bronchial and peribronchial
inflammation and fibrosis, ulceration of the bronchial wall,
squamous metaplasia, and mucous gland hyperplasia

 The parenchyma normally supplied by the affected airways is

abnormal, containing varying combinations of fibrosis,
emphysema, bronchopneumonia, and atelectasis

 As a result of the inflammation, vascularity of the bronchial wall

increases, with associated enlargement of the bronchial arteries
and anastomoses

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PATHOLOGY … contd

 Three different patterns of bronchiectasis

 In cylindrical bronchiectasis, the involved bronchi appear

uniformly dilated and end abruptly at the point that smaller
airways are obstructed by secretions

 In varicose bronchiectasis, the affected bronchi have an irregular

or beaded pattern of dilatation resembling varicose veins

 In saccular (cystic) bronchiectasis, the bronchi have a ballooned

appearance at the periphery, ending in blind sacs without
recognizable bronchial structures distal to the sacs

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 ETIOLOGY AND PATHOGENESIS

 Bronchiectasis is a consequence of inflammation and destruction

of the structural components of the bronchial wall.

 Infection is the usual cause of the inflammation; microorganisms

such as P.aeruginosa and H.influenzae produce proteases, and
other toxins that injure the respiratory epithelium and impair
mucociliary clearance

 The host inflammatory response induces epithelial injury, largely

as a result of mediators released from neutrophils

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ETIOLOGY AND PATHOGENESIS

 As protection against infection is compromised, the dilated

airways become more susceptible to colonization and growth of
bacteria

 Thus, a reinforcing cycle can result, with inflammation

producing airway damage, impaired clearance of
microorganisms, and further infection, which then completes the
cycle by inciting more inflammation

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Infectious Causes

 Adenovirus and influenza virus are the main viruses that cause
bronchiectasis

 Virulent bacterial infections, especially necrotizing organisms

such as S. aureus, Klebsiella spp., and anaerobes, remain
important causes when antibiotic treatment of a pneumonia is not
given or is significantly delayed

 Infection with Bordetella pertussis has also been classically

associated with chronic suppurative airways disease

 Bronchiectasis has been reported in pts with HIV infection,

perhaps at least partly because of recurrent bacterial infections
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 Infectious Causes … contd

 Tuberculosis is a major cause of bronchiectasis worldwide

 Nontuberculous mycobacteria are frequently cultured from

patients with bronchiectasis, often as secondary infections or
colonizing organisms.

 However, it has also been recognized that these organisms,

especially those of the MAC complex, can serve as primary
pathogens associated with the development or progression of
bronchiectasis

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 ETIOLOGY AND PATHOGENESIS

 Impaired host defense mechanisms are often involved in the

predisposition to recurrent infections.

 The major cause of localized impairment of host defenses is

endobronchial obstruction.

 Bacteria and secretions cannot be cleared adequately from the

obstructed airway, which develops recurrent or chronic infection.

 Slowly growing endobronchial neoplasms such as carcinoid

tumors may be associated with bronchiectasis.

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ETIOLOGY AND PATHOGENESIS

 Foreign-body aspiration is another important cause of

endobronchial obstruction, particularly in children.

 Airway obstruction can also result from

bronchostenosis, from impacted secretions, or from
extrinsic compression by enlarged lymph nodes

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 ETIOLOGY AND PATHOGENESIS

 Impairmentof pulmonary defense mechanisms occurs with

immunoglobulin deficiency, primary ciliary disorders, or CF

 Panhypogammaglobulinemia is associated with recurrent infection

and bronchiectasis, pts often also have a hx of sinus or skin
infections

 Selectivedeficiency of an IgG subclass, especially IgG2, has also

been described in a small number of pts with bronchiectasis

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 ETIOLOGY AND PATHOGENESIS

 Primary ciliary dyskinesia are responsible for 5–10% of cases of

bronchiectasis

 The cilia become dyskinetic; their coordinated, propulsive action is

diminished, and bacterial clearance is impaired

 The clinical effects include recurrent upper and lower RTI, such as
sinusitis, otitis media, and bronchiectasis

 Because normal sperm motility also depends on proper ciliary function,

men are generally infertile

~ half of pts with ciliary dyskinesia fall into the subgroup of Kartagener’s
syndrome, in which situs inversus accompanies bronchiectasis and sinusitis

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 ETIOLOGY AND PATHOGENESIS … contd

 In CF, the tenacious secretions in the bronchi are associated with

impaired bacterial clearance, resulting in colonization and
recurrent infection with a variety of organisms, particularly
mucoid strains of P. aeruginosa but also S. aureus, H. influenzae,
Escherichia coli, and Burkholderia cepacia.

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 Noninfectious Causes

 Some cases of bronchiectasis are associated with exposure to a

toxic substance that incites a severe inflammatory response
… inhalation of a toxic gas such as ammonia or aspiration of acidic
gastric contents

 An immune response in the airway may also trigger inflammation,

destructive changes, and bronchial dilatation
…. This mechanism is important for bronchiectasis with ABPA,
which is caused at least partly by an immune response to
Aspergillus organisms that have colonized the airway

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 Noninfectious Causes

 In α1-antitrypsin deficiency, the usual respiratory complication is

the early development of panacinar emphysema, but affected
individuals may occasionally have bronchiectasis

 In the yellow nail syndrome, which is caused by hypoplastic

lymphatics, the triad of lymphedema, pleural effusion, and yellow
discoloration of the nails is accompanied by bronchiectasis in
approximately 40% of pts

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 CLINICAL MANIFESTATIONS

 Ptstypically present with persistent or recurrent cough and purulent

sputum production

 Repeated, purulent RTIs should raise clinical suspicion for

bronchiectasis

 Hemoptysis occurs in 50–70% of cases and can be caused by bleeding

from friable, inflamed airway mucosa

 More significant, even massive bleeding is often a consequence of

bleeding from hypertrophied bronchial arteries

 Systemic symptoms such as fatigue, weight loss, and myalgias can

also occur
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CLINICAL MANIFESTATIONS … contd

 When a specific infectious episode initiates bronchiectasis, pts

may describe a severe pneumonia followed by chronic cough and
sputum production

 Alternatively, pts without a dramatic initiating event often

describe the insidious onset of symptoms

 In some cases, pts are either asymptomatic or have a

nonproductive cough, often associated with “dry” bronchiectasis
in an upper lobe

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CLINICAL MANIFESTATIONS … contd

 Dyspnea or wheezing generally reflects either wide spread

bronchiectasis or underlying COPD

 With exacerbations of infection, the amount of sputum increases,

and it becomes more purulent and often more bloody; systemic
symptoms, including fever may also be prominent

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 CLINICAL MANIFESTATIONS … contd
 Physicalexamination of the chest overlying an area of
bronchiectasis is quite variable

 Any combination of crackles, rhonchi, & wheezes may be heard,

all of which reflect the damaged airways containing significant
secretions.

 Aswith other types of chronic intrathoracic infection, clubbing

may be present

 Ptswith severe diffuse ds, particularly those with chronic

hypoxemia, may have associated cor pulmonale and RVF

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RADIOGRAPHIC AND LABORATORY FINDINGS

 The chest radiograph findings are often nonspecific

 The radiograph may be normal with mild disease

 Pts with saccular bronchiectasis may have prominent cystic

spaces, either with or without air-liquid levels, corresponding to
the dilated airways

 Otherfindings are caused by dilated airways with thickened walls,

which result from peribronchial inflammation.

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RADIOGRAPHIC FINDINGS
 The dilated airways are often crowded together in parallel

 When seen longitudinally, the airways appear as “tram tracks”;

when seen in cross-section, they produce “ring shadows.”

 High-resolutionCT provides an excellent view of dilated airways

… the standard technique for detecting or confirming the diagnosis
of bronchiectasis

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 LABORATORY FINDINGS
 Examination of sputum reveals an abundance of neutrophils &
colonization or infection with a variety of possible organisms

 Appropriate staining and culturing of sputum often provide a

guide to antibiotic therapy

 When bronchiectasis is focal, fiberoptic bronchoscopy may reveal

an underlying endobronchial obstruction.

 In other cases, upper lobe involvement may be suggestive of

either tuberculosis or ABPA.

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LABORATORY FINDINGS … contd
With more widespread disease:
 Measurement of sweat chloride levels for CF

 Structuralor functional assessment of nasal or bronchial cilia or

sperm for primary ciliary dyskinesia, and

 Quantitative assessment of immunoglobulins may explain

recurrent airway infection.

PFT may demonstrate airflow obstruction as a consequence of

diffuse bronchiectasis or associated COPD

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 LABORATORY FINDINGS … contd
 Bronchial hyperreactivity and some reversibility of the airflow
obstruction with inhaled bronchodilators are relatively common

 Because the ddx for focal versus diffuse bronchiectasis is different,

the radiographic distribution of disease can serve as a starting
point of the diagnostic workup

 The workup should be dictated by a careful assessment of the

clinical scenario

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Diagnostic Approach

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 Treatment
 Therapy has several major goals:

1) Treatment of infection, particularly during acute exacerbations;

2) Improved clearance of tracheobronchial secretions;

3) Reduction of inflammation; and

4) Treatment of an identifiable underlying problem.

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 Treatment … Antibiotics
 Antibiotics are the cornerstone of bronchiectasis management

 Antibiotics are used only during acute episodes

 Choice of an antibiotic should be guided by Gram’s stain & culture of

sputum, empiric coverage (e.g.,amoxicillin, TMP-SMX, or
levofloxacin) is given initially

 When Pseudomonas spp. is present, oral therapy with a quinolone or

parenteral therapy with an aminoglycoside, or third-generation
cephalosporin is appropriate

 There are no firm guidelines for length of therapy, but a 10- to 14-day
course or longer is typically administered
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 Treatment … Bronchial Hygiene

 Numerous approaches to enhance secretion clearance & include:

 Hydration and mucolytic administration

 Aerosolization of bronchodilators and hyperosmolar agents (e.g.,

hypertonic saline)

 Chest physiotherapy (e.g., postural drainage, mechanical chest

percussion)

 The mucolytic dornase (DNase) is recommended in CF-related

bronchiectasis but not in non-CF bronchiectasis, given concerns
about lack of efficacy and potential harm in the non-CF
population
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Treatment … Anti-Inflammatory Therapy

 Control of the inflammatory response may be of benefit

… alleviated dyspnea, decreased need for inhaled agonists, and
reduced sputum production
… no significant differences in lung function or bronchiectasis
exacerbation rates have been observed

 Risks of immunosuppression and adrenal suppression must be

carefully considered in infectious causes

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 Treatment … Anti-Inflammatory Therapy
 Oral/systemic glucocorticoids may be important in treating
bronchiectasis due to certain etiologies, such as ABPA, or
noninfectious bronchiectasis due to underlying autoimmune
conditions (e.g., RA or Sjögren's syndrome)

 Pts with ABPA may also benefit from a prolonged course of

treatment with the oral antifungal agent itraconazole

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 Treatment … Refractory Cases

 Although surgical therapy was common in the past, more effective

antibiotic & supportive Rx has largely replaced surgery

 When bronchiectasis is localized and the morbidity is substantial

despite adequate medical Rx, surgical resection of the involved
region of lung should be considered

 When massive hemoptysis does not resolve with conservative

therapy, including rest and antibiotics, therapeutic options are
either surgical resection or bronchial arterial embolization

 In advanced cases, lung transplantation can be considered.

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 Complications

 Recurrent infections and repeated courses of antibiotics can lead

to microbial resistance to antibiotics

 Recurrent infections can result in injury to superficial mucosal

vessels, with bleeding and, in severe cases, life-threatening
hemoptysis

 Management of massive hemoptysis usually requires intubation to

stabilize the patient, identifying the source of bleeding, and
protecting the nonbleeding lung.

 Controlof bleeding often necessitates bronchial artery

embolization and, in severe cases, surgery.
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Prognosis
 Vary widely with the underlying etiology and may also be
influenced by the frequency of exacerbations and (in infectious
cases) the specific pathogens involved

 Thedecline of lung function in pts with non-CF bronchiectasis

was similar to that in patients with COPD, with FEV1 declining
by 50–55 mL per year as opposed to 20–30 mL per year for
healthy controls

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 Prevention

 Reversal of an underlying immunodeficient state

 Vaccinationwith chronic respiratory conditions (e.g., influenza

and pneumococcal vaccines)

 Pts who smoke should be counseled about smoking cessation

 Use of suppressive antibiotics to minimize the microbial load and

reduce the frequency of exacerbations has been proposed
… there is less consensus with regard to this approach in non-CF-
associated bronchiectasis

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 Prevention … contd

Possible suppressive treatments include

1) Oral antibiotic (e.g., cipro) daily for 1–2 wks / month

2) Rotating oral antibiotics

3) Macrolide antibiotic daily or three times/wk

4) Inhalation of aerosolized antibiotics [e.g., tobramycin ) on a
rotating schedule (e.g., 30 days on, 30 days off)
5) Intermittent IV antibiotics for pts with more severe bronchiectasis
and/or resistant pathogens

 In addition, ongoing, consistent attention to bronchial hygiene can

promote secretion clearance and decrease the microbial load in the
airways.
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Thank You

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