Bronchiectasis
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Basics
Description
- Bronchiectasis is an irreversible dilatation of ≥1 airways accompanied by recurrent transmural bronchial infection/inflammation and chronic mucopurulent sputum production.
- It may be focal or diffused.
Epidemiology
- Predominant age: most commonly presents in 6th decade of life
- Predominant sex: female > male (1)
Incidence
- Incidence has decreased in the United States for two reasons:
- Widespread childhood vaccination against pertussis
- Effective treatment of childhood respiratory infections with antibiotics
- Among children, incidence may be higher in indigenous or socioeconomically disadvantaged group (2).
Etiology and Pathophysiology
- Frequently idiopathic
- Chronic infections (PTB), autoimmune disease, genetic causes (cystic fibrosis (CF)), chronic obstructive pulmonary disease (COPD), connective tissue disease, allergic bronchopulmonary aspergillosis (ABPA) (4)
- 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.
Risk Factors
- 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
- Immunodeficiency
- Congenital abnormalities
General Prevention
- Routine immunizations against pertussis, measles, Haemophilus influenzae 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)
- COPD
- 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: 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
- Miscellaneous
- Yellow nail syndrome
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
Description
- Bronchiectasis is an irreversible dilatation of ≥1 airways accompanied by recurrent transmural bronchial infection/inflammation and chronic mucopurulent sputum production.
- It may be focal or diffused.
Epidemiology
- Predominant age: most commonly presents in 6th decade of life
- Predominant sex: female > male (1)
Incidence
- Incidence has decreased in the United States for two reasons:
- Widespread childhood vaccination against pertussis
- Effective treatment of childhood respiratory infections with antibiotics
- Among children, incidence may be higher in indigenous or socioeconomically disadvantaged group (2).
Etiology and Pathophysiology
- Frequently idiopathic
- Chronic infections (PTB), autoimmune disease, genetic causes (cystic fibrosis (CF)), chronic obstructive pulmonary disease (COPD), connective tissue disease, allergic bronchopulmonary aspergillosis (ABPA) (4)
- 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.
Risk Factors
- 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
- Immunodeficiency
- Congenital abnormalities
General Prevention
- Routine immunizations against pertussis, measles, Haemophilus influenzae 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)
- COPD
- 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: 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
- Miscellaneous
- Yellow nail syndrome
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