Bronchiectasis

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DESCRIPTION

Bronchiectasis is an irreversible syndrome with symptoms of chronic productive cough and recurrent exacerbations and with characteristic findings on cross-sectional imaging of bronchial wall dilation and thickening.

EPIDEMIOLOGY

Prevalence

  • Overall estimated prevalence of bronchiectasis in the United States is 701 per 100,000 (1).
  • Prevalence is higher among women than men and increases with age.

ETIOLOGY AND PATHOPHYSIOLOGY

  • Bronchiectasis often arises as a complication of inherited or acquired disease states but may arise as an isolated diagnosis.
  • Vicious cycle hypothesis (1):
    • An initial pulmonary insult causes airway inflammation, dysfunction, and structural disease.
    • Dysfunctional airways are further impaired in their ability to clear infections.
    • A pattern of lung damage/inflammation and progressive airway dysfunction is established, leading to clinical decline.
  • Neutrophil extracellular traps (NETs) levels and neutrophil elastase activity correlate with disease activity and may serve as potential therapeutic targets (1).

GENERAL PREVENTION

  • Routine immunization against respiratory infections (pertussis, measles, Haemophilus influenzae type B [HIB], influenza, and Streptococcus pneumonia).
  • Early recognition and treatment of respiratory disease, inflammatory disease, and other predisposing conditions
  • Genetic counseling for patients with inheritable conditions which predispose to bronchiectasis who wish to conceive
  • Encourage and support smoking cessation in all patients who smoke

COMMONLY ASSOCIATED CONDITIONS

  • Many cases are idiopathic, and bronchiectasis may be an isolated pulmonary diagnosis.
  • Acquired conditions associated with bronchiectasis:
    • Pneumonia
    • GERD
    • Asthma/chronic obstructive pulmonary disease (COPD)
    • Rheumatologic conditions (rheumatoid arthritis, IBD)
    • Tuberculosis (TB)
    • Allergic bronchopulmonary aspergillosis (ABPA)
    • Chronic rhinosinusitis
    • Focal airway obstruction
  • Inherited conditions associated with bronchiectasis:
    • Cystic fibrosis
    • Primary ciliary dyskinesia
    • Congenital abnormalities of the airways (e.g., tracheobronchomalacia, Mounier-Kuhn syndrome)
    • α1-Antitrypsin deficiency

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