This document provides an overview and management guidelines for bronchiectasis. It begins with definitions and descriptions of bronchiectasis, including its clinical presentation and radiological appearance. It then discusses current understanding of bronchiectasis as a systemic disease associated with increased cardiovascular risk. Guidelines for diagnosis and assessment of severity from the British Thoracic Society are summarized. The document presents two clinical cases and reviews recommendations for management of stable disease and exacerbations, including airway clearance techniques, antibiotics, pulmonary rehabilitation, and emerging therapies.

1. Bronchiectasis (non-CF):
A pulmonary and systemic disease
ROSMADI ISMAIL, MD
SEPT 2, 2022

2. OUTLINE
 overview
 Clinical vignette
 Management of stable cases
 Management of exacerbation
 Future direction

3. Bronchiectasis
 Irreversible dilation of the bronchial tree
 Inflamed, dilated, easily collapsible bronchi
 Airflow obstruction
 Impaired clearance of secretions

4. Bronchiectasis = Ectasia of the Bronchus
By National Heart Lung and Blood Institute - National Heart Lung and Blood Institute, Public Domain,
https://commons.wikimedia.org/w/index.php?curid=29583169
Ectasia : is dilation or distention of a tubular structure

6. Radiological appearance
- Cylindrical
- Varicose
- Cystic
+ additional features
Clinical syndrome
- Cough
- Sputum production, hemoptysis
- Recurrent infections
- Chronic bacterial colonisation
Bronchiectasis as a chronic respiratory disease

7. AETIOLOGY

8. Bronchiectasis: systemic disease?
 cardiovascular (CV) disease accounted for up to 25% of all bronchiectasis deaths1.
 Taiwan database: risk of an acute coronary syndrome was 40% higher in those with
bronchiectasis than in those who did not have this diagnosis2.
 UK cohort: increasing severity of bronchiectasis - independently associated with the
development of all forms of CV disease. Associated with 42% increase in the rate of first
episode of coronary events3.
1) Chalmers et al. Am. J. Respir. Crit. Care Med. 2014; 189: 576–85
2) Hung et al . Respirology 2018; 23: 828–34
3) Navaratnam et al. Thorax 2017; 72: 161–6.
Bronchiectasis Systemic inflammation Endothelial dysfunction atherosclerosis

9. • Increasing awareness of the disease
• Increasing incidence and prevalence
• Increasing hospitalizations
• No licensed therapies (but increasing
clinical trials)
• First international guidelines published
in 2017
Bronchiectasis overview
Quint JK. Eur Respir J. 2016;47(1):186–93
Ringshausen FC. PLoS ONE 8(8): e71109
Polverino E. Eur respir J 2017

10. ERS Guideline.Eur Respir J 2017; 50: 1700629

11. European Respiratory Society guidelines on
bronchiectasis found no strong clinical evidence in
support of pharmacotherapy

12. Case 1: Diagnosis
 SN L, 35-year-old lady, Para 3
 Referred for newly diagnosed bronchiectasis ( Based on Hx and HRCT)
 Coughing x4 yrs, copious sputum
 mMRC : 1
 No previous hospitalization ( except for child birth)
 Non-smoker
 Work as SN
 No previous exacerbation

13. Hill et al. Thorax2019;74 (Suppl 1):1–69
British Thoracic Society Guideline 2019

14. BTS Guideline 2018/9
Hill et al. Thorax2019;74 (Suppl 1):1–69
Whom to investigate?
What radiology?
What tests?
Airways clearance
Stepwise Management
The Deteriorating Patient / exacerbation
Who should be followed up in secondary care
Monitoring

15. Recommendation on diagnostic testing
Diagnosis
 Baseline chest X-ray
 Thin section CT of the chest
investigations for causes of bronchiectasis
 FBC
 Serum total IgE and sensitization ( specific IgE or skin test) (ABPA)
 Serum IgG, IgA,IgM (Antibody deficiency)
 Sputum Culture : routine and mycobacterial
 If primary ciliary dyskinesia (PCD) suspected : speed video microscopy
analysis (HSVMA) or nasal nitric oxide (nNO) measurement.
Hill et al. Thorax2019;74 (Suppl 1):1–69

16. Prognosis and severity scoring
predict future exacerbations and hospitalizations, health status, and death over 4 years
Bronchiectasis Severity Index (BSI) or FACED score
Hill et al. Thorax2019;74 (Suppl 1):1–69

17. Bronchiectasis Severity Index (BSI)
BSI: Prognosis
Mortality % Hospitalization %
MILD
(0-4)
1 year 0-2.8 0 -3.4
4 year 0-5.3 0-9.2
MODERATE
(5-8)
1 year 0.8-4.8 1.0 - 7.2
4 year 4-11.3 9.9 - 19.4
SEVERE
≥ 9
1 year 7.6–10.5 16.7 – 52.6
4 year 9.9 –29.2 41.2 – 80.4

18. Case 1: SN L
BMI: 23. FBC: TWC; 6.2 (eosinophil : 9.9%[0.6]), Hb: 11, PLT: 318
RP/ LFT/ FBS: normal
Sputum MTB x2 : negative
Sputum Culture: no growth
RF/ ANCAs/ ANA: -ve
IgG, IgM, IgA: normal level. Serum Galactomannan: -ve
Spirometry 24/5/19:
FEV1: 2.31L (70% pred)
FVC: 2.87L (72% pred)
FEV1/FVC: 80%
BSI: 1 (MILD)
Complaint of copious sputum, through out the day, interfering her job as nurse.

19. Management for stable bronchiectasis

20. Hill AT, et al. Thorax 2019;74(Suppl 1)
Stepwise management of bronchiectasis

22. Pulmonary Rehab
Mucus Problems Exercise
Intolerance
Respiratory Muscle
Weakness
Expiratory
Flow
Modification
Slow
Expiration
Forced
Expiration
Instrumental
Techniques
PEP Oscillatory
PEP
Inspiratory
Muscle
Training (IMT)
Aerobic
Training
Strength
Training
AD
ELTGOL
ACBT
Cough
Huffing
Flutter
Acapella
HFCWO
PEP mask
T-PEP
Positioning
Postural
Drainage
Physical
Activity
Counselling
ELTGOL (L'Expiration Lente Totale Glotte Ouverte en décubitus Latéral)
ACBT: The Active Cycle of Breathing Techniques HFCWO: High-frequency chest wall oscillation
T-PEP: Temporary positive airway pressure

23. Pulmonary Rehabilitation: Systematic Review
Lee, AL et al. Arch Phys Med Rehab 2017 Apr;98(4):774-782
ISWT
SGRQ
LCQ
Leicester Cough Questionnaire
St George’s Respiratory
Questionnaire
incremental shuttle walk Test

24. Pulmonary Rehab
 Offer pulmonary rehabilitation to Patient with mMRC ≥1
 Tailored to the patient’s symptoms, physical capability, disease
characteristics and HRCT scan
 Subscribed to all aspect of pulmonary rehab program.
Hill AT, et al. Thorax 2019;74(Suppl 1)

25. Hill AT, et al. Thorax 2019;74(Suppl 1)
Airway/
mucus
clearance

26. Mucus clearance devices

27. High frequency chest wall oscillation (HFCWO)

28. mucolytic?
Hill AT, et al. Thorax 2019;74(Suppl 1)
 Do not routinely use nebulized recombinant human DNase
and mannitol.
 Consider the use of humidification with sterile water or
normal saline to facilitate airway clearance .
 Oral mucolytic can improve sputum expectoration.
 no data on Carbocysteine ( or our all time favourite :
Bisolvon). Reassess benefit after 6/12 of usage

29. Hill AT, et al. Thorax 2019;74(Suppl 1)
Stepwise management of bronchiectasis

30. Self
management
plan / Action
plan
https://services.nhslothian.scot/Respiratory/Pages/Bronchiectasis-Guidance-Documents.aspx

31. Case 2: Exacerbator
 54 year old man, ex-smoker
 Bronchiectasis and COPD
 On LTOT
 > 5 exacerbation over the past 1 year ( hospital stay : 340
days over the past 1 year)
 mMRC : 4

32. Management for frequent exacerbator

33. Bronchiectasis
exacerbation

34. Management of acute exacerbation
 No RCT evaluating the efficacy of antibiotics in exacerbations on
adults.
 Choices of antibiotic should be guided national antibiotic
guideline / hospital or community antibiogram.
 Prompt treatment of exacerbations.
 Use previous sputum bacteriology results.
 May start empirical antibiotics while waiting culture result.
Hill AT, et al. Thorax 2019;74(Suppl 1)

35. Hill AT, et al. Thorax 2019;74(Suppl 1)
Stepwise management of bronchiectasis

36. BTS 2019. Long term antibiotic ( ≥ 3 exacerbation per year)
Pseudomonas
• inhaled colistin
• inhaled gentamicin
(2nd line)
• Macrolides
• Macrolides + inhaled
antibiotics
Non-Pseudomonas
•Macrolides
•Inhaled gentamycin
•Doxycycline
Hill AT, et al. Thorax 2019;74(Suppl 1)

37. P. aeruginosa in bronchiectasis, why it matters?
 3-fold increased in mortality
 Up to 7-fold increased in hospital admission rate
 More severe exacerbation
 More iv Antibiotic
 Poorer QoL
 Greater lung function decline
ERS Guideline. Eur Respir J 2017; 50: 1700629
Respir Med.2015 Jun;109(6):716-26
Ann Am Thorac Soc. 2015 Nov;12(11):1602-11

38. P. aeruginosa colonisation infection
 Colonization: presence without direct/ indirect sign of
infection.
 Definition highly heterogeneous.
 Most frequent definition:
- x2 isolates ≥ 2 month apart.
Ann Am Thorac Soc. 2015 Nov;12(11):1602-11

39. P. aeruginosa and bronchiectasis
 poor clinical outcomes associated with chronic P. aeruginosa
infection
 P. aeruginosa eradication positively influence important clinical
outcomes including exacerbation frequency
 eradication of organisms other than P. aeruginosa not proven
beneficial
ERS Guideline.Eur Respir J 2017; 50: 1700629

40. Pseudomonas eradication
ERS Guideline.Eur Respir J 2017; 50: 1700629

41. Kocurek et al. Curr Opin Pulm Med 2019, 25:192–200c
Inhaled
anti-pseudomonal
agents

42. Hill AT, et al. Thorax 2019;74(Suppl 1)
Stepwise management of bronchiectasis

43. Long term antibiotic treatment (>=3 months)
Study/
year
Population
No. of
exacerbation
Drug Dose/ Frequency Duration
Key
outcomes
Wong C et al N= 71 ≥1 Azithromycin 500mg x3/week 6/12 Decreased exacerbations
Altenburg J et al N= 43 ≥3 Azithromycin 250mg daily 12/12 Decreased exacerbations
Increase side effect
Increase resistance
Serisier DJ et al N= 117 ≥2 erythromycin 400mg BD 12/12 Decreased exacerbations
Reduce sputum
Increase resistance
Wong C et al Lancet 2012;380:660–7
Altenburg J et al JAMA 2013;309:1251–9
Serisier DJ et al JAMA 2013;309:1260–7
Consider long term antibiotics in patients with bronchiectasis who experience ≥ 3
exacerbations per year (1,2) use inhaled gentamycin or doxycycline as alternative
1. Hill AT, et al. Thorax 2019;74(Suppl 1)
2. ERS Guideline.Eur Respir J 2017; 50: 1700629
Macrolide ( for non-pseudomonas coloniser)

44. Recommendation for anti-inflammatory usage in Bronchiectasis
 Do not offer long-term oral corticosteroids or ICS for
patients with bronchiectasis without other indications
 Do not routinely offer PDE4 inhibitors (sildenafil),
methylxanthines (neulin) or leukotriene receptor
antagonists (montelukast)
 Do not routinely offer CXCR2 antagonists, neutrophil
elastase inhibitors or statins.
BTS guidelines .Hill AT, et al. Thorax 2019;74(Suppl 1)

45. Other BTS recommendation
 Offer a trial of long-acting bronchodilator therapy in patients with symptoms of significant
breathlessness (after reversibility testing) .
 Consider lung resection in localized disease and symptomatic despite optimized medical
therapy.
 Consider transplant referral in bronchiectasis patients aged 65 years or less if the FEV1 is
<30% with significant clinical instability
 Offer annual influenza immunization and polysaccharide pneumococcal vaccination to all
patients with bronchiectasis. (COVID 19 vaccine as well)
 Offer LTOT ( same recommendation for COPD)
 Do not routinely recommend alternative treatments (cough suppression, nutritional
supplement, complementary therapy/homeopathy, supplemental treatments)
Hill AT, et al. Thorax 2019;74(Suppl 1)

46. Emerging therapies in bronchiectasis
Calmers. Lancet Respir Med 2018. February 22, 2018
Brensocatib
Phase 2 trial