Bronchiectasis

Question 1

What is bronchiectasis?

Answer 1

Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation.
This is due to irreversible damage to the elastic and muscular components of the bronchial wall.

Question 2

What are the causes of Bronchiectasis?

Answer 2

1. post-infective: tuberculosis, measles, pertussis, pneumonia
2. cystic fibrosis
3. bronchial obstruction e.g. lung cancer/foreign body
4. immune deficiency: selective IgA, hypogammaglobulinaemia
5. allergic bronchopulmonary aspergillosis (ABPA)
6. ciliary dyskinetic syndromes: Kartagener’s syndrome, Young’s syndrome
7. yellow nail syndrome

Question 3

Who can bronchiectasis effect?

Answer 3

Both children and adults

Question 4

is brochiectasis focal or diffuse?

Answer 4

It can be both
Bronchiectasis can be limited to one area of a lung (focal) or it can be widespread (diffuse).

Question 5

What is the most common cause of bronchiectasis?

Answer 5

previous severe lower respiratory tract infection
(such as pneumonia, pertussis, pulmonary tuberculosis, mycoplasma, influenza, or other viral infection.)

Question 6

What is the most common pathogen found in bronchiectasis?

Answer 6

Haemophilus influenzae

Question 7

What make the prognosis in patients with bronchiectasis worse?

Answer 7

With extensive disease.
Who have frequent severe exacerbations — more severe exacerbations are associated with worse quality of life, daily symptoms, lung function decline, and reduced life expectancy.
With breathlessness — this is one of the strongest predictors of mortality.
With a primary antibody deficiency disorder.
Colonized with Pseudomonas – this is associated with a three-fold increase in mortality risk.
With comorbidities.
Who smoke.

Question 8

What are the complications of bronchiectasis?

Answer 8

Infective exacerbations and chronic bacterial colonization.

Haemoptysis — this can involve blood volumes of more than 250 mL and can be life-threatening.

Pneumothorax.

Respiratory failure.

Cor pulmonale.

Chest pain.

Coronary heart disease, ischaemic stroke.

Anxiety and depression.

Urinary incontinence.

Fatigue and reduced exercise tolerance.

Nutritional deficiency.

Reduced quality of life — this is equivalent to the impact of severe COPD.

Question 9

What are the clinical features of bronchiectasis?

Answer 9

Daily expectoration of large volumes of purulent sputum (75% of people).
Dyspnoea (60% of people).
Fever.
Fatigue, reduced exercise tolerance.
Haemoptysis that can be frank (up to 10 mL) or massive (more than 235 mL) (26–51.2%).
Rhinosinusitis.
Weight loss.
Chest pain that is present between exacerbations and is usually non-pleuritic
Sputum colonization with P. aeruginosa.
Young age at presentation.
History of symptoms over many years.
Absence of smoking history.

Question 10

What may be found on examination of a patient with suspected bronchiectasis?

Answer 10

Coarse crackles, especially in the lower lung zones. ( present on inspiration and expiration)

Wheeze.

High-pitched inspiratory squeaks.

Large airway rhonchi (low pitched snore-like sounds).

Palpable chest secretions on coughing or forced expiratory manoeuvre, persisting over time.

Finger clubbing (uncommon).

Question 11

What does the widening of alveoli and bronchioles in bronchiectasis cause issues?

Answer 11

The problem then is that mucus, which we all have in our lungs to some degree, can pool and collect in the airways. And because the airways aren’t meant to be that wide in the first place, they produce more mucus than usual.

Question 12

What is the pathophysiology as to why the airways become wider.

Answer 12

An initial insult to the bronchi (e.g. infection) results in immune cells being recruited to the bronchi. These immune cells secrete cytokines and proteases, leading to inflammation in the bronchi.

This inflammation damages the muscle and elastin found in the bronchial walls, leading to bronchial dilation.
Dilated bronchi are predisposed to persistent microbial colonisation, as mucus traps in the dilated bronchi

Question 13

What percent of bronchiectasis is idiopathic?

Answer 13

40%

Question 14

What are 3 risk factors for bronchiectasis?

Answer 14

1. Age (>70 years)
2. Female gender
3. Smoking history

Question 15

What are three important areas to cover in a bronchiectasis history?

Answer 15

1. History of childhood lower respiratory tract infections: you may forget to ask this if the patient is elderly
2. Family history: ask about congenital conditions (such as cystic fibrosis) and autoimmune conditions (such as rheumatoid arthritis)
3. Smoking history: quantify in pack-years (1 pack-year = smoking 20 cigarettes a day for a year)

Question 16

What features of COPD differentiate from bronchiectasis?

Answer 16

Reduced breath sounds

Absence of high-pitched inspiratory squeaks

CT chest may be normal

Question 17

What features of asthma differentiate from bronchiectasis?

Answer 17

Diurnal variation in symptoms and peak flow

History of atopy

Lung function tests: bronchodilator reversibility

Question 18

What features of pneumonia differentiate from bronchiectasis?

Answer 18

More acute presentation (over days, rather than months or years)

Chest X-ray: consolidation

Question 19

What features of chronic sinusitis differentiate from bronchiectasis?

Answer 19

Vesicular breath sounds

Radiological investigations: normal

Question 20

What bedside investigations can be done to investigate bronchiectasis?

Answer 20

1. Pulse oximetry - target saturations may be reduced in advanced disease
2. Sputum culture - Pseudomonas is particularly common.
3. Lung function tests - typically show an obstructive pattern
4. ECG - may impair ventricular function and lead to pulmonary hypertension.

Question 21

What imaging can be used to investigate bronchiectasis?

Answer 21

1. Chest X-ray: required to exclude other pathologies but may be normal in mild bronchiectasis - severe disease shows tram lines and ring shadows
2. High-resolution C: Shows bronchial dilation with or without airway thickening
3. Bronchoscopy - used in patients with localised bronchiectasis as may be caused by a foreign body or endobronchial lesion

Question 22

What is the medical management of patients with bronchiectasis?

Answer 22

1. Mucoactive agents (e.g. nebulised saline and carbocisteine): aid the clearance of sputum, for patients who have difficulty expectorating sputum (such as frail, elderly patients)
2. Long-term antibiotics (e.g. azithromycin three times a week): may be used in patients who have three or more exacerbations per year, after consultation with a respiratory specialiist
   3.Bronchodilators: offer a long-acting bronchodilator (e.g. formoterol) in patients with activity-limiting dyspnoea
3. Long-term oxygen therapy: if saturations on room air are <88% or PaO2 on room air is <7.3kPa

Question 23

What are surgical management options for bronchiectasis?

Answer 23

Lung resection: for localised bronchiectasis, not controlled by optimum medical management

Lung transplant for patients younger than 65, with rapid deterioration despite optimum medical management

Question 24

What are 3 disease-related complications of bronchiectasis?

Answer 24

1. Respiratory failure: due to failure of gas exchange in the lungs
2. Massive haemoptysis (>250ml per day): often due to rupture of a bronchial artery into a bronchus
3. Anxiety and depression: due to impaired quality-of-life

Question 25

What are 2 treatment-related complications of bronchiectasis?

Answer 25

Macrolides: long QT syndrome, tinnitus and hearing loss

Lung transplant: immediate complications (e.g. blood loss), early complications (e.g. transplant rejection) and late complications (e.g. post-transplantation lymphoproliferative disorder)