Bronchiectasis

By: Anteneh A.[R1]

November, 2020
 Definition
 Bronchiectasis is an irreversible airway dilation that
involves the lung in either a focal or diffuse manner.
 Focal bronchiectasis refers to bronchiectatic changes in
a localized area of the lung.
 Diffuse bronchiectasis is characterized by wide-spread
bronchiectatic changes throughout the lung.
 It classically has been categorized as:
Cylindrical or tubular (most common form)
Varicose
Cystic
 Etiology
 It can arise from infectious or noninfectious causes.
 Clues to the underlying etiology are often provided by
the pattern of lung involvement.
 Focal bronchiectasis can be a consequence of
obstruction of the airway either extrinsic or intrinsic.
 Extrinsic causes can be compression by adjacent LAP
or parenchymal tumor mass.
 Intrinsic be due to an airway tumor or aspirated foreign
body, a scarred/stenotic airway, or bronchial atresia.
 Diffuse bronchiectasis often arises from an underlying
systemic or infectious disease process.
 More pronounced involvement of the upper lung fields is
most common in CF & also postradiation fibrosis.
 Predominant involvement of the lower lung fields is
usually due to:
Chronic recurrent aspiration (e.g., due to esophageal
motility disorders like those in scleroderma).
End-stage fibrotic lung disease (e.g., traction
bronchiectasis from idiopathic pulmonary fibrosis).
Recurrent immunodeficiency-associated infections
(e.g., hypogammaglobulinemia)
 Preferentially affecting the mid lung field:
Infection by nontuberculous mycobacteria (NTM),
most commonly MAC.
Congenital causes include dyskinetic/immotile cilia
syndrome.
 Predominant involvement of the central airways:
Allergic bronchopulmonary aspergillosis (ABPA):
immune-mediated reaction to Aspergillus damages
the bronchial wall.
Congenital causes include tracheobronchomegaly
(Mounier- Kuhn syndrome) & Williams-Campbell
syndrome.
 In many cases, the etiology of bronchiectasis is not
determined (25–50%- idiopathic disease).
 Epidemiology
 The epidemiology varies greatly with the underlying
etiology.
 Patients born with CF often develop significant clinical
bronchiectasis in late adolescence or early adulthood.
 But atypical presentations of CF in adults in their thirties and
forties are also possible.
 MAC Bronchiectasis classically affects nonsmoking women
>50 years of age.
 In general, the incidence of bronchiectasis increases with
age.
 Bronchiectasis is more common among women than men.
 In TB prevalent areas, bronchiectasis more frequently occurs
as a sequela of granulomatous infection.
 Malnutrition may predispose to immune dysfunction & dev’t
 Pathogenesis & Pathology
 The most widely cited mechanism of infectious
bronchiectasis is the “vicious cycle hypothesis”.
Susceptibility to infection & poor mucociliary
clearance result in microbial colonization of the
bronchial tree.
P. aeruginosa propensity for colonizing damaged
airways and evading host defense mechanisms.
Impaired mucociliary clearance can result from
inherited conditions (CF or dyskinetic cilia syndrome).
Mediators released directly from bacteria can interfere
with mucociliary clearance.
 Proposed mechanisms for noninfectious bronchiectasis
include:
Immune-mediated reactions that damage the
bronchial wall.
Traction bronchiectasis via parenchymal distortion
as a result of lung fibrosis.
 Clinical Manifestations
 The most common clinical presentation is a persistent
productive cough with ongoing production of thick,
tenacious sputum.
 Physical findings often include crackles & wheezing,
clubbing of the digits (some pts).
 Mild to moderate airflow obstruction is often detected on
PFTs, overlapping with COPD.
 Acute exacerbations are usually characterized by changes
in the nature of sputum production, with increased volume
& purulence.
 However, typical signs & symptoms of lung infection,
such as fever and new infiltrates, may not be present.
 Diagnosis
 The presence of “tram tracks” indicating dilated airways
on chest radiographs is consistent with bronchiectasis.
 But chest radiographs lack sensitivity.
 Chest CT is more specific for bronchiectasis & is the
imaging modality of choice for confirming the diagnosis.
 CT findings include:
 Airway dilation (“tram tracks” or “signet-ring sign”)
 Lack of bronchial tapering
 Bronchial wall thickening in dilated airways
 Inspissated secretions (“tree-in-bud” pattern)
 Cysts emanating from the bronchial wall
 Approach
 The evaluation of a patient with bronchiectasis entails:
Elicitation of a clinical history
Chest imaging
Workup to determine the underlying etiology
 Evaluation of focal bronchiectasis almost always
requires bronchoscopy.
 A workup for diffuse bronchiectasis includes analysis
for the major etiologies, with an initial focus on
excluding CF.
 PFT is an important component of a functional
assessment of the patient.
 Treatment of Bronchiectasis
 Treatment of infectious bronchiectasis:
Control of active infection
Improvements in secretion clearance
Bronchial hygiene so as to decrease the microbial
load within the airway
Minimize the risk of repeated infections
 Con…
Antibiotic Treatment
Antibiotics targeting the causative or presumptive
pathogens (H. influenzae & P. aeruginosa) should
be administered in acute exacerbations.
Usually for a minimum of 7–10 days and perhaps
for as long as 14 days.
Decisions about treatment of NTM infection can be
difficult.
 Diagnostic criteria NTM infection:
 At least two sputum samples positive on culture
 At least one BAL fluid sample positive on culture
 A biopsy sample displaying histopathologic features of
NTM infection (granuloma or a positive stain for AFB)
along with one positive sputum culture
 A pleural fluid or other sample positive on culture.
 The recommended regimen for HIV-negative patients:
 Macrolide combined with rifampin and ethambutol.
 Guidelines recommend macrolide susceptibility
testing for clinically significant MAC isolates.
 Con…
Bronchial Hygiene
 The numerous approaches used to enhance secretion
clearance in bronchiectasis include:
 Hydration & mucolytic administration
 Aerosolization of bronchodilators & hyperosmolar
agents (hypertonic saline)
 Chest physiotherapy
 Pulmonary rehabilitation & regular exercise program
improve exercise capacity & quality of life.
 The mucolytic dornase (DNase) is recommended
routinely in CF-related bronchiectasis.
 Con…
 Anti-inflammatory therapy
 Control of the inflammatory response may be of benefit in
bronchiectasis.
 It alleviated dyspnea, decreased need for inhaled β-
agonists, and reduced sputum production.
 However, no significant differences in lung function or
exacerbation rates have been observed.
 Risks of immunosuppression & adrenal suppression should
be considered.
 Glucocorticoids may be important in ABPA & autoimmune
causes.
 Patients with ABPA may also benefit from a prolonged
course of oral antifungal agent itraconazole.
 Con…
 Refractory Cases
In select cases, surgery can be considered, with
resection of a focal area of suppuration.
In advanced cases, lung transplantation can be
considered.
 Complications
 Recurrent infections
 Life-threatening hemoptysis
 Intubation to stabilize the patient
 Identification of the source of bleeding
 Protection of the nonbleeding lung.
 Bronchial artery embolization &, in severe cases,
surgery.
 Prognosis
 Outcomes depends on:
The underlying etiology & comorbid conditions
The frequency of exacerbations (in infectious cases)
The specific pathogens involved (worse with P.
aeruginosa colonization)
 FEV1 declining by 50–55 mL per year as opposed to
20–30 mL per year for healthy controls (one study).
 Prevention
 Administration of gamma globulin for Ig deficient
patients
 Influenza & pneumococcal vaccines
 Smoking cessation
 Suppressive treatment to prevent recurrence
 Con…
 Possible suppressive treatments for recurrences (> 3/yr):
1. Administration of oral antibiotic (ciprofloxacin)
daily for 1–2 weeks per month.
2. Use of a rotating schedule of oral antibiotics.
3. Administration of a macrolide antibiotic daily or
three times per week.
4. Inhalation of aerosolized antibiotics on a rotating
schedule (30 days on, 30 days off)
5. Intermittent administration of IV antibiotics
 A benefit of long-term macrolides (6–12 months of
azithromcin or erythromycin) in decreasing rates of
bronchiectasis exacerbation.
 References
Harrison’s principle of internal medicine 20 th
ed.