Bronchiectasis

Question 1

Definition of bronchiectasis

Answer 1

• Chronic coughs and sputum production
• In presence of abnormal thickening and dilation of the bronchial wall that is
• Visible on lung imaging as:
  Bronchial dilatation –
  1) Bronchoarterial ratio >1
  2) Lack of tapering
  3) Airway visibility within 1cm of pleural surface or touching mediastinal pleura

Indirect signs:
1) Bronchial wall thickening
2) Mucus impaction
3) Mosaicism

Question 2

Causes of bronchiectasis & Ix required

Answer 2

3A, 2I, 1C, 1O

A: ABPA
A: Autoimmune (RA, Sjogren, Scleroderma, IBD)
A: Aspiration (GORD, vocal cord dysfunction)

I: Infection (post TB, foreign body)
I: Immunodef (HIV, CVID)

C: congenital (CF, Kartegerner’s, primary ciliary dyskinesia, congenital trachoebronchial abnorm)

O: obstructive (asthma, COPD)

Ix required:
A) Bloods:
FBC: raised eosinophil (ABPA)
Immunoglobulin IgE & Aspergillus spefic IgE (ABPA)
Serum immunoglobulin (IgA, IgG, IgM)
HIV
CTD screening: RF, anti-CCP, ANA, ANCA, ENA
CF: sweat chloride testing , CFTR gene test
- if suggestive features – early onset, male infertility, malabsorption, pancreatitis
Primary Ciliary Dyskinesia (PCD): ciliary function test, nasal nitric oxide
- with suggestive features – early onset, rhinosinusitis, infertility, recurrent otitis media
α1AT deficiency: alpha-1 antitrypsin level & phenotype
- suggestive features – basal panacinar emphysema

B) Sputum/ BAL:
C&S
MTB C&S

C) Others:
Spirometry
HRCT
Echo - to Ax for complication of pulm HTN

Question 3

Long term Mx of bronchiectasis (12)

Answer 3

1) Treat underlying cause
2) Chest physio
3) Pulm rehab
4) Mucolytics
5) Bronchodilator
6) Vax
7) Abx during infective exac
8) Long term macrolides/ inhaled gentamycin
9) Haemotysis Mx - tranexamic acid/ BAE
10) Surgical resection of focal disease
11) Smoking cessation
12) LTOT (indication to follow COPD - hypoxic))/ NIV if needed (hypercapnia)
13) Consider lung transplant in ≤65 & deteriorate despite optimal Rx

Question 4

What’s the minimal duration of Abx therapy in exac of bronchiectasis?

Reference:
Polverino E, Goeminne PC, McDonnell MJ, et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J 2017; 50: 1700629 [https://doi.org/ 10.1183/13993003.00629-2017].

HCTM PP presentation

Answer 4

Mainly 14 days (in ERS 2017 guideline for the Mx of adult bronchiectasis)
Range to be considered:
Mild: 10-21d
Mod-severe: 14-21d

Question 5

What is the benefit of pseudomonas eradication therapy after the first culture positive?

Answer 5

Helps to reduce hosp admission, exacerbation, and prolonged the time for recurrent Pseudomonas infection

Question 6

What is the pseudomonas eradication treatment after the first culture positive

Answer 6

Has 3 options. No clear evidence to support one regimen over another.

1st regimen:
T ciproflox 750mg BD for 2w, then repeat sputum C&S
If still positive, then give iv beta-lactam that has Pseudomonas cover + aminoglycoside for 2w, then repeat sputum C&S
If still still positive, then for inhaled colistin/gentamycin/ tobramycin for 3m

Question 7

What is the support for the use of macrolides in bronchiectasis?

Answer 7

EMBRACE - used Erythromycin 400mg BD

BAT - used Azithromycin 250mg OD

BLESS study - used Azithromycin 500mg EOD

Question 8

Signs of bronchiectasis exacerbations are

Answer 8

≥3 of Sx for ≥48h:
1) Cough
2) Sputum volume
3) Sputum purulence
4) SOB/ exercise tolerance
5) Fatigue
6) Haemoptysis
AND clinicians determine Rx is required

Question 9

Types of bronchiectasis on imaging

Answer 9

1) Cylindrical
2) Varicose
3) Cystic

Question 10

What are the routine Ix that need to be done in bronchiectasis?

Answer 10

Severity - at baseline
CT scan - at baseline
Comorbidities - at baseline
Ix for causes - at baseline
MTB C&S - at baseline
MMRC - 6-12m
Exacerbations - 6-12m
Sputum C&S - 6-12m
SpO2 - 6-12m
Spirometry - annually
BMI - annually

Question 11

How to Ax severity of bronchiectasis?

Answer 11

Can use FACED score or bronchiectasis severity index (BSI) score.

FACED: (mild ≤2, severe ≥5)
FEV1
Age
Colonisation with Pseudomonas
Extension of disease (#of lobes involved)
Dyspnoea (MMRC)

BSI: (mild ≤4, severe >8)
Age
BMI
FEV1
Colonisation
Dyspnoea (MMRC)
Extension
Exac
Admission to hosp

Question 12

What is the definition of frequent exacerbations in bronchiectasis? How does it affect Mx?

Reference: Hill AT, Sullivan AL, Chalmers JD, et al. Thorax 2019;74 (Suppl 1):1–69

Answer 12

Frequent exacerbations = ≥3 exacerbations/ year

Mx in frequent exac:
1) Pseudomonas - long term inhaled anti-pseudomonal Abx (1st line colistin, 2nd line gentamycin);
or if unable to tolerate due to bronchospasm –> for long term microlide (Azithro 500mg 3x/week);
or use combined inhaled and oral Avx if high exac rate

2) Other potentially pathogenic org - long term microlide or long term oral targeted Abx

3) If no pathogen - long term microlide

4) if despite the above, exac ≥5/year - regular IV Abx every 2-3 months

Question 13

What is the Rx of bronchiectasis with recurrent exacerbations?

Answer 13

If exac ≥3/year:
1) Optimise airway clearance & treat underlying cause

2) If there is pseudomonas infection, for long-term inhaled Abx.
- if lack of response or intolerance, swap inhaled Abx to long term macrolide therapy
- if inadequate response, add on oral Abx

3) If there is a non-pseudomonas infection, for long term macrolide Rx.
- if lack of response or intolerance, swap to long term inhaled Abx, or swap to long-term targeted oral Abx, or combine oral & inhaled Abx