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- Bronchiectasis is an irreversible dilatation of ≥1 airways accompanied by recurrent transmural bronchial infection/inflammation and chronic mucopurulent sputum production.
- Generally classified into cystic fibrosis (CF) and noncystic fibrosis (non-CF) bronchiectasis
- Predominant age: most commonly presents in 6th decade of life
- Predominant sex: female > male (1)
Incidence has decreased in the United States for two reasons:
- Widespread childhood vaccination against pertussis
- Effective treatment of childhood respiratory infections with antibiotics
- In the United States, prevalence estimated to be 52.3/100,000.
- Prevalence increased substantially with age from 4.2/100,000 persons aged 18 to 34 years to 271.8/100,000 among those aged 75 years and older (1).
Etiology and Pathophysiology
- CF bronchiectasis: bronchiectasis due to CF
- Non-CF bronchiectasis
- Most cases are idiopathic.
- Most commonly associated with non-CF bronchiectasis is childhood infection.
- Vicious circle hypothesis: Transmural infection, generally by bacterial organisms, causes inflammation and obstruction of airways. Damaged airways and dysfunctional cilia foster bacterial colonization, which leads to further inflammation and obstruction.
- Nontuberculous mycobacterial infection is both a cause and a complication of non-CF bronchiectasis.
- Severe respiratory infection in childhood (measles, adenovirus, influenza, pertussis, or bronchiolitis)
- Systemic diseases (e.g., rheumatoid arthritis and inflammatory bowel disease)
- Chronic rhinosinusitis
- Recurrent pneumonia
- Aspirated foreign body
- Congenital abnormalities
- Routine immunizations against pertussis, measles, Haemophilus influenza type B, influenza, and pneumococcal pneumonia
- Genetic counseling if congenital condition is etiology
- Smoking cessation
Commonly Associated Conditions
- Mucociliary clearance defects
- Primary ciliary dyskinesia
- Young syndrome (secondary ciliary dyskinesia)
- Kartagener syndrome
- Other congenital conditions
- α1-Antitrypsin deficiency
- Marfan syndrome
- Cartilage deficiency (Williams-Campbell syndrome)
- Chronic obstructive pulmonary disease
- Pulmonary fibrosis, causing traction bronchiectasis
- Postinfectious conditions
- Bacteria (H. influenzae and Pseudomonas aeruginosa)
- Mycobacterial infections (tuberculosis [TB] and Mycobacterium avium complex [MAC])
- Whooping cough
- Aspergillus species
- Viral (HIV, adenovirus, measles, influenza virus)
- Immunodeficient conditions
- Primary: hypogammaglobulinemia
- Secondary: allergic bronchopulmonary aspergillosis (ABPA), posttransplantation
- Sequelae of toxic inhalation or aspiration (e.g., chlorine, luminal foreign body)
- Rheumatic/chronic inflammatory conditions
- Rheumatoid arthritis
- Sjögren syndrome
- Systemic lupus erythematosus
- Inflammatory bowel disease
- Yellow nail syndrome