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- Bronchiectasis is an irreversible dilation 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
Incidence has decreased in the US for 2 reasons:
- Widespread childhood vaccination against pertussis
- Effective treatment of childhood respiratory infections with antibiotics
- Prevalence in adult US population estimated to be >110,000 affected individuals
- Internationally, prevalence increases with age from 4.2/100,000 persons aged 18–34 years to 271.8/100,000 among those aged ≥75 years (1).
- 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
- Routine immunizations against pertussis, measles, Haemophilus influenza type B, influenza, and pneumococcal pneumonia
- Genetic counseling if congenital condition is etiology
- Smoking cessation counseling
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.
- CF bronchiectasis: Bronchiectasis due to CF
- Non-CF bronchiectasis:
- Most cases are idiopathic.
- Most commonly associated with non-CF bronchiectasis is childhood infection.
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
- 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