bronchiectasis
BRONCHIECTASIS
Bronchiectasis is an irreversible pathologic dilatation or ectasia of the bronchi due to repeated airway infection and inflammation.
It enhances susceptibility to bronchial infection and increases inflammatory reaction which causes further lung damage.
Classic symptoms of of bronchiectasis are cough with chronic sputum production along with recurring infective exacerbations and hemoptysis.

Principles of Therapy

Goals of Treatment
  • Minimize both respiratory & generalized symptoms; maintain good quality of life
  • Reduce frequency & severity of acute exacerbations
  • Limit disease progression
  • Maintain or improve pulmonary function
  • Avoid or minimize adverse effects of treatment
  • Recognizing acute exacerbation generally depends upon symptomatic changes rather than any specific lab result
  • Identification of acute exacerbation may be difficult since the onset may be gradual w/ sputum volume increasing slowly over months

Pharmacotherapy

Treatment of Acute Exacerbations
Antibiotics
  • One of the mainstays of treatment for bronchiectasis used to break the cycle of bacterial infection that leads to inflammation & further lung injury
  • Appropriate therapy for the treatment of exacerbations & chronic active disease
  • Most patients have chronic colonization of organisms which is the basis for empiric choice of antibiotic
  • Amoxicillin
    • First-line treatment for patients without previous bacteriological exams
    • Higher doses may be used for patients w/ H influenzae colonization
  • Quinolones
    • Eg Ciprofloxacin, Levofloxacin
    • May be used in patients w/ suspected Pseudomonas aeruginosa colonization
    • Reasonable therapeutic option for oral administration in ambulatory patients
  • Some authorities may choose empiric antibiotic therapy based on clinical parameters
    • FEV1/FVC of <60% & daily sputum >20 mL are independently associated w/ the presence of P aeruginosa
    • Patients w/ little sputum & good lung function may only require non-pseudomonal antibiotics
    • Suspected non-pseudomonal acute exacerbation: Consider treatment w/ IV cephalosporin, Amoxicillin/clavulanic acid or oral macrolides (Clarithromycin)
    • Suspected pseudomonal acute exacerbation: Consider treatment w/ Ciprofloxacin (1st-line), Ceftazidime, antipseudomonal Penicillins (Piperacillin/Tazobactam, Aztreonam, Meropenem), Colistin, or IV aminoglycoside (Gentamicin, Tobramycin)
    • Methicillin-sensitive Staph aureus exacerbation: Flucloxacillin, Clarithromycin
    • Methicillin-resistant Staph aureus exacerbation: oral Rifampicin + Trimethoprim, oral Rifampicin + Doxycycline, Vancomycin, or Linezolid
  • Duration of therapy is not well defined, but a minimum of 7-10 days has become a frequent practice
    • Antibiotic therapy of 14 days duration is recommended for acute exacerbations, based on the patient’s prior microbiologic test results & severity of exacerbation
  • Sputum culture & sensitivity are indicated in patients who fail to respond to the initial antibiotic to help define antibiotic selection
Bronchodilators
  • Beta2-agonists may be theoretically beneficial in bronchiectasis since they increase ciliary beat frequency in vitro
  • Administration prior to chest physiotherapy may help produce more sputum during clearance, & may help increase expiratory flow rates
Corticosteroids
  • Inhaled
    • Use is controversial & may not be recommended by some authorities
    • May be beneficial since inflammation & neutrophilic mediator release play a major role in bronchiectasis
    • Small short-term studies suggest that regular use of inhaled corticosteroids may improve lung function & indices but not symptoms
    • Beclomethasone: Small short-term study showed that cough & sputum production may be reduced, & improvement in FEV1 & peak flow rates may occur
    • Fluticasone: Pilot study showed reduced sputum neutrophils & inflammatory cytokines
  • Nasal
    • If present, allergic nasal symptoms may be controlled w/ topical corticosteroids
  • Systemic
    • Not generally recommended
    • Systemic steroids can further depress host immunity, promote increased bacterial & fungal colonization, & perpetuate infection
Other Anti-Inflammatory Agents
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
    • Some studies showed that treatment w/ Indometacin help reduce sputum production in bronchiectasis patients
    • Oral Ibuprofen may help reduce airway inflammation in patients w/ CF
  • Statins
    • Not generally recommended
    • Some studies showed that treatment w/ Atorvastatin improved symptoms based on Leicester Cough Questionnaire (LCQ) score in patients w/ mild disease, but adverse effects were seen
    • No improvement of symptoms seen in patients w/ severe disease (P aeruginosa infection) when assessed using LCQ but w/ significant improvements in St. George’s Respiratory Questionnaire
Long-term Therapies
  • Should be considered in patients who experience ≥3 exacerbations in a year 
  • It is thought that some organisms (eg P aeruginosa & H influenzae) stimulate neutrophilic & inflammatory mediator response in the airways
  • Bacterial pathogens are thought to have an active destructive role, therefore suppressive antibiotic therapies have been used in bronchiectasis patients
Macrolides (Azithromycin, Erythromycin)
  • Should be considered in non-Pseudomonas colonized bronchiectasis patients 
    • Long-term administration can reduce the frequency of exacerbations, & the number of patients experiencing at least one exacerbation
  • Consider treatment w/ macrolides if w/ treatment failure after inhaled antibiotics, or as adjunctive therapy to inhaled antibiotics in Pseudomonas-colonized patients w/ increased frequency of exacerbation
  • Inhibits respiratory mucous secretion by decreasing pulmonary macrophage mucous secretagogue production
    • Also reduces P aeruginosa exotoxin production which slows human respiratory ciliary beat in vitro & decreases P aeruginosa adherence to damaged airway in vitro
  • Low-dose macrolide therapy may be continued for 1-2 years before stopping if there is clear clinical improvement, & withdrawn if no improvement after 3-4 months of therapy
Aerosolized Antibiotics
  • Inhaled Colistin is recommended for patients w/ chronic P aeruginosa colonization
  • Gentamicin is a treatment option in patients w/ chronic P aeruginosa colonization if Colistin therapy fails, & as 2nd-line option after macrolide therapy in non-pseudomonas colonized patients
  • May be considered in patients where bronchiectasis is due to CF
  • Tobramycin or Colistin may improve sputum volume, purulence & exacerbation frequency, sputum P aeruginosa & myeloperoxidase levels & general well-being
    •  A significant reduction in bacterial count for P aeruginosa was seen in patients given Colistin therapy
    • A dose of Tobramycin 300 mg inhaled 12 hrly via nebulizer is used in childn >6 yr & adults x 28 days on, followed by 28 days off for CF patients; this dose has been used in bronchiectasis patients
    • Colistin 0.5-1 MIU in children <2 years & 1-2 MIU in adults & children >2 years inhaled 12 hourly is used in CF patients
  • Inhaled Ceftazidime + Tobramycin given over 12 months improve hospitalization frequency & duration but not lung function or antibiotic usage
  • Increased time to next exacerbation was seen in patients given inhaled Gentamicin & Ciprofloxacin
Tetracyclines
  • Consider treatment w/ Doxycycline if non-Pseudomonas colonized patient is intolerant or unresponsive to combined macrolide & inhaled antibiotic therapy
Intermittent IV Antibiotics
  • Some authorities recommend intermittent IV antibiotics for patients who have copious sputum production (>60 mL/day) when at steady state &/or who have frequent exacerbations (>6 episodes/yr)
  • May also be considered in preparation for major surgery including resection of a bronchiectatic region of lung & other procedures when pulmonary function may be compromised
  • Patients are hospitalized for 2-3 wk of antibiotics every 2-3 mth
    • IV Ceftazidime, Amoxicillin/clavulanic acid & quinolones have been used
    • Chest physiotherapy is administered & rest is encouraged
  • Some authorities have suggested that the intermittent IV antibiotics may decrease the cumulative hospitalizations frequency & the need for antibiotic therapy in the medium term
Bronchodilators
  • May be considered in bronchiectasis patients w/ breathlessness or w/ co-existing COPD or asthma, & use should follow the recommendations of the corresponding disease

Non-Pharmacological Therapy

Chest Physiotherapy
  • Effective airway physiotherapy can improve mucus drainage, reduce inflammation & susceptibility to infections, & improve quality of life 
  • Referral to a chest physiotherapist for a more tailored technique is recommended
Airway Clearance Therapy
  • Patients should learn different airway clearance techniques (ACT) & exercises
    • ACT should include active cycle of breathing techniques, manual techniques (percussion, shaking, vibrations, over pressure), postural drainage, positive expiratory pressure, oscillating PEP, autogenic drainage, inspiratory muscle training (IMT), high frequency chest wall oscillation, intrapulmonary percussive ventilation, & intermittent positive pressure breathing
    • ACT should be performed 1-2x/week
  • Inhalation exercises [eg IMT, expiration w/ the glottis open in the lateral posture (ELTGOL)] when done together w/ pulmonary rehabilitation, increases the beneficial effects of these programs
Pulmonary Rehabilitation
  • Recommended for patients w/ limited exercise capacity
  • Studies showed significant improvements (increased exercise capacity, improved quality of life, longer interval to next exacerbation) in patients
Bronchopulmonary Hygiene
  • One of the mainstays in the treatment of bronchiectasis; w/ unknown long-term effectiveness
  • Aid in removing secretions from the lower airway in patients w/ mucus hypersecretion & problems w/ expectoration problems
  • Systemic hydration enhanced by nebulization w/ saline remains a necessity in patients w/ mucous plugging & viscous secretions
  • Mucolytics (eg Acetylcysteine) alters characteristic of sputum & may aid expectoration of secretions
Oxygen Therapy
  • Long-term oxygen therapy may be considered for patients whose respiratory status is unstable w/ impending respiratory failure, especially patients w/ underlying COPD
  • May consider non-invasive ventilation in patients w/ bronchiectasis w/ respiratory failure, hypercapnea & in need of hospitalization intermittently
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