1. Depart. Of pulmonology R4 백승숙

2. Introduction Bronchiectasis Initially described by Laennec in 1819
An abnormal dilatation of bronchi and bronchioles
due to repeated cycles of airway infection and inflammation
Multiple genetic, anatomic, and systemic causes
The purpose of this update
Pathophysiology, prevalence, diagnosis, natural history, etiologies,
infectious complications, and treatment of non-CF bronchiectasis

3. Pathophysiology
Figure 1. This gross lung specimen from a patient with CF

4. Pathophysiology
Anatomic distortion of conducting airways that results in
chronic cough, sputum production, and recurrent infections
Airway infection and inflammation
Further infection Airway damage
Airway and lung
parenchyma destruction

5. Pathophysiology Sputum analyses and bronchial mucosal biopsy specimens
have shown increased chemotactic activity
(eg, elastase, IL-8, TNF-α, prostanoids)
Systemic markers of inflammation are also elevated
 Play important, yet poorly understood roles
Anatomic factors
Chronic infection & inflammation
Host defense

6. Prevalence The worldwide prevalence is also unknown
4.2 per 100,000 persons aged 18 to 34 years
272 per 100,000 persons among those 75 years of age
Weycker et al, United States
1 per 6,000 persons
Tsang et al, Hong Kong & New Zealand
The worldwide prevalence is also unknown
Women > Men (though seen across the age spectrum)
More frequently in middle aged and elderly persons

7. Diagnosis Symptoms Chronic cough productive of mucopurulent sputum
(occasionally, a dry nonproductive cough)
Dyspnea, Hemoptysis, Nonspecific constitutional complaints
Physical findings
Nonspecific
Crackles and wheezes
Clubbing of the digits
Pulmonary function testing
Airflow obstruction ranging from modest to severe

8. Diagnosis HRCT scanning Bronchial dilatation
; an internal bronchial diameter greater than
the diameter of the accompanying bronchial artery
Lack of bronchial tapering on sequential slices
The extent of disease seen on HRCT scans has been
correlated with functional change and clinical outcomes
Signet ring sign

9. Figure 2.
A: Cylindrical bronchiectasis
B: Varicose bronchiectasis with
a loss of normal bronchial tapering
C: Cystic/saccular bronchiectasis
P. aeruginosa infection
Poorer prognosis

10. Natural History
The clinical course of non-CF bronchiectasis is variable
Frequency 1.5 exacerbations per year in patients
O’Donnell AE et al, North America & U.K.
Decline of approximately 50mL/yr in FEV1
King PT et al, Australia
Factors associated with an accelerated rate of decline of lung function
Chronic colonization by Pseudomonas aeruginosa
History of severe exacerbations
Evidence of systemic inflammation

11. Etiologies

12. Etiologies Idiopathic bronchiectasis Poorly understood subtype
Congenital causes of bronchiectasis
Cystic Fibrosis (CF)
Sinusitis, pancreatic insufficiency, or infertility
Sweat chloride testing, Genetic testing
(mutation on the CF transmembrane conductance regulator)
Primary Ciliary Dyskinesia (PCD)
Bronchiectasis, rhinosinusitis, ear infections, and infertility
1/2 ; Situs inversus totalis
1-antitrypsin (AAT) deficiency
Emphysema is the most commonly associated pulmonary abnormality
Bronchiectasis ; 27% of 74 pts. - Parr et al

13. Etiologies Immune deficiencies Primary hypogammaglobulinemia
Defects of neutrophil adhesion, respiratory burst, and chemotaxis
HIV/AIDS
Immune-related diseases
Allergic Bronchopulmonary Aspergillosis (ABPA)
Collagen vascular diseases
Rheumatoid arthritis
Sjögren syndrome
Inflammatory bowel diseases
Ulcerative colitis
Crohn disease

14. Etiologies COPD & Asthma Stable COPD patients mean FEV1 of 0.96L – 50%
More severe COPD exacerbations, lower airway bacterial colonization,
and increased levels of sputum inflammatory markers
Severe persistent asthma patients – 3%
Chronic gastric aspiration
H. pylori might have a pathogenic role in the development of bronchiectasis
NTM infections
May cause nodular bronchiectasis
Association of RML and lingular predominant bronchiectasis
Presence of M. avium intracellulare complex organisms in bronchiectatic areas

15. Microbiology Nonenteric GNB Accelerated decline in lung function
More frequent exacerbations
Presence of CF
Mildest disease

16. Treatment The goals of bronchiectasis treatment
To reduce the number of exacerbations
To improve quality of life

17. Antimicrobial Therapy
The role of the use of maintenance antibiotic therapy is uncertain
There may be a benefit to a maintenance antibiotic regimen
in patients who frequently experience exacerbations
26 patients
6 to 84 months
Cycles of alternating antibiotics
Radiographic stability of disease
in 77% of patients
10 patients
90 days of oral ciprofloxacin
Decreased numbers of exacerbations
Resistance developed in 2 patients
74 patients
4-week trial of inhaled tobramycin
300 mg twice a day
Microbiological benefit
No clinical benefit
41 patients
3 cycles of 2 weeks on/2 weeks off with inhaled tobramycin
300 mg twice a day
Inhaled gentamicin and colistin
Patients numbers was insufficient

18. Antimicrobial Therapy
Mild-to-moderate exacerbations
Oral antibiotics for 2 to 3 weeks
Optimal duration of therapy is unknown
Oral levofloxacin = IV ceftazidime
Tsang et al, Hong Kong
Oral ciprofloxacin = Oral ciprofloxacin + Inhaled tobramycin
Bilton D et al, U.K.
Severe exacerbations
Require IV antibiotic therapy
 Treatment of exacerbations must be individualized to the patient and
to the infecting organism

19. Reduction of Airway Inflammation
Therapy with inhaled corticosteroids and oral macrolides
May reduce airway inflammation in patients with bronchiectasis
Systemic steroids has never been studied
500 μg of inhaled fluticasone twice per day / 12-months
Erythromycin 500 mg twice per day / 6-months
Azithromycin 500 mg twice per week / 6-months

20. Mobilization of Airway Secretions
Pharmacologic agents
Bronchodilator adrenergic and anticholinergic agents
Mucolytic agent, recombinant human DNase I
Inhaled mannitol
Nebulized hypertonic saline solution
Mechanical mobilization of secretions
Chest physical therapy with postural drainage
Active cycle of breathing
Oscillatory positive expiratory pressure devices
High frequency assisted airway clearance
Pulmonary rehabilitation

21. Surgery Resectional surgery Focal disease
Not respond to conventional management
Uncontrolled hemoptysis despite of interventional techniques
Single-lung and double-lung transplants
End-stage bronchiectasis
68% of 1-year survival rate
Beirne et al

22. Approach to the Patient
Patients in whom bronchiectasis has been diagnosed
Should be evaluated for potential underlying causes
Should have a microbiological examination of their sputum
for routine bacterial and NTM organisms
Should be devised for an individualized plan of therapy

23. Conclusions
A tailored clinical evaluation Detection of underlying causes Appropriate multimodality treatment plan