BRONCHIECTASIS

EPIDEMIOLOGY
• The prevalence of bronchiectasis is not accurately
know and data varies considerably throughout the
world with a very high incidence in the Indigenous
populations of New Zealand and Australia (Chang et al
2010). Females and the elderly are also more
frequently affected (Seitz at al 2012, Quint et al 2016).
• It has been estimated that there are at least 110,000
adults in the United States with this condition
• Indonesia has not yet reported a number of definite
figures regarding this disease.
 ETIOLOGY
• Bronchiectasis requires the combination of
two important processes taking place in the
bronchi:
– local infection or inflammation and
– inadequate clearance of secretions, airway
obstructions, or impaired host defense
• Pulmonary infections (i.e., bacterial, viral, fungal), especially severe
or chronic infections
• Disorders of secretion clearance or mucous plugging
– Cystic fibrosis (CF)
– Primary ciliary dyskinesia (PCD)
– Allergic bronchopulmonary aspergillosis (ABPA)
• Bronchial narrowing or other forms of obstruction
– COPD
– Aspiration
– Tumors
• Immunodeficiency (e.g., common variable
immunodeficiency, hypogammaglobulinemia, HIV)
• Chronic inflammatory diseases (e.g., rheumatoid arthritis, Sjögren
syndrome, Crohn's disease)
 CLASSIFICATION
Reid, 1950
• Tubular by smooth dilation of the bronchi;
• Varicose in which the bronchi are dilated
with multiple indentations; and
• Cystic in which dilated bronchi terminate in
blind ending sacs
A) Tubular/Cylindrical B) Varicose C) Saccular/cystic seen on HRCT
Pathophysiology
• The first stage in the development of bronchiectasis is
an initial infective insult to the airways, which triggers a
mucociliary response.
• Micro-organisms trigger the release of toxins and an
inflammatory response within the airways.
• This inflammatory response includes the release of
neutrophils, lymphocytes and macrophages within the
bronchial lumen.
• Neutrophils also alter the function of the cilial
epithelium, leading to changes in cilial beat frequency
and mucous gland hypersecretion.
• Both processes compromise mucociliary clearance.
• This loss of mucociliary transport renders the airways
susceptible to microbial colonization.
• Neutrophils play a central role in the tissue damage in
bronchiectasis, by releasing mediators (including
inflammatory cytokines, elastases and matrix
metalloproteinases) which destroy the bronchial
elastin and other supporting lung structure, leading to
permanent dilatation of the bronchi
• . Airway walls become thickened with normal mucosal
and muscular layers substituted by oedema, ulceration
or fibrosis.
• In proximal airways, the structural cartilage
may be diminished, provoking a reduction in
supportive structure.
• These changes in airway structure further
contribute to pooling of mucus, and the self-
perpetuating cycle of infection and
inflammation, which promotes progressive
airway damage and recurrent infections
 CLINICAL MANIFESTATIONS
• Chronic productive cough (lasting months to years) with copious
mucopurulent sputum ; the following may be heard on auscultation:
– Crackles and rhonchi
– Wheezing (due to obstruction from secretions, airway collapsibility, or a
concomitant condition)
– Bronchophony
• Rhinosinusitis
• Dyspnea
• Hemoptysis: usually mild/self-limiting but severe hemorrhage that
requires embolization may occur.
• Nonspecific symptoms (i.e., fatigue, weight loss, pallor due to anemia)
• Clubbing of nails (uncommon)
• Exacerbations of bronchiectasis
– Increased production of mucous above baseline
– Low-grade fever
 DIAGNOSTIC
Imaging
• Chest x-ray (best initial test)
– Inflammation and fibrosis of bronchial walls lead to the
appearance of parallel “tram track” lines
– Thin-walled cysts (i.e., dilated bronchi forming sacs), possibly
with air-fluid levels
– Late-stage bronchiectasis: honeycombing
• High-resolution computer tomography
(HRCT): confirmatory test
– Dilated bronchi with thickened walls; possible signet-
ring appearance and tram track lines
– Cysts, especially at bronchial ends in lower lobes,
and honeycombing
Other tests
• Sputum culture and smear: to determine infectious etiology (i.e.
bacteria, mycobacteria, and fungi)
• Blood tests
– CBC with differential: may show anemia of chronic
disease and ↑ WBCs, ↑ neutrophils
– HIV testing, genetic testing for CF, immunoglobulin quantitation to
determine other possible etiologies
• Pulmonary function tests: findings consistent with obstructive
pulmonary disease (i.e. ↓ FEV1/FVC ratio)
• Bronchoscopy: to visualize tumors, foreign bodies, or other lesions;
may also be used in combination with bronchoalveolar lavage (BAL)
to obtain specimens for staining and culture
 TREATMENT
• Bronchiectasis is a permanent anatomical malformation and
therefore cannot be cured. However, symptoms and
advancement of the disease can be controlled. The treatment
of any underlying cause is also very important.
Conservative
• Bronchopulmonary hygiene and
chest physiotherapy: “cupping and clapping” and postural
drainage, hydration, directed cough
• Antibiotic therapy of exacerbations
• Smoking cessation
• Vaccinations (i.e.seasonal influenza vaccine, pneumococcal
vaccine)
• Bronchodilators, corticosteroids, and nebulized hypertonic
saline are not routinely used but may be considered,
especially in patients with notable obstructive symptoms.
Invasive procedures
• Surgical resection of bronchiectatic lung or
lobectomy : indicated in pulmonary hemorrhage,
inviability of bronchus, and substantial sputum
production in unilateral bronchiectasis
• Pulmonary artery embolization: indicated in
pulmonary hemorrhage
• Lung transplantation should be considered in
severe disease
 COMPLICATIONS
• Recurrent bronchopulmonary infections →
obstructive ventilation disorder → respiratory
failure and cor pulmonale
• Pulmonary hemorrhage (massive hemoptysis)
• Pleural empyema
• Lung abscess