Bronchiectasis

Question 1

Definition bronchiectasis

Answer 1

A persistent or progressive chronic debilitating disease characterised by permanent dilation of the bronchi due to irreversible damage to the elastic and muscular components of the bronchial wall.

Symptoms can vary from intermittent expectoration and infection to persistent daily expectoration of large volumes of sputum

Question 2

Classification bronchiectsis (based on location)

Answer 2

Can be limited to one area of a lung (focal), or can be widespread (diffuse)

Question 3

Causes of bronchectasis

Answer 3

Caused by inflammatory damage to the airways, most commonly due to severe LRTIs (pneumonia, pertussis, pulmonary TB, influenza). Most commonly H.influenza is found in bronchiectasis

Can also occur after an aspiration or inhalation injury, asthma, cystic fibrosis, IBD

Some congenital defects are associated with bronchiectasis:
* Williams-Campbell syndrome
* Marfan’s syndrome
* Mounier-Kuhn syndrome
* Young’s syndrome

40% of adults and children with bronchiectasis have no identifiable cause

Question 4

What are some complications of bonchiectasis

Answer 4

Complications include: Infective exacerbations and chronic bacterial colonization, pneumothorax, respiratory failure, nutritional deficiency, reduced quality of life

Patients with mild bronchiectasis can have a normal life expectancy, but prognosis is worse in people with extensive disease, breathlessness, comorbidities and who have frequent severe exacerbations.

Question 5

Presentation of bronchiectasis. What can be found O/E

Answer 5

Consider a diagnosis in children:
* With a chronic moist or productive cough unresponsive to 4 weeks of antibiotics, especially between viral colds, or with +ve sputum cultures for Staphylococcus aureus, H. influenzae, P.aeruginosa
* A recurrent or persistent wet cough (over 6 weeks duration)
* Asthma that does not respond to treatment
* An episode of severe pneumonia or recurrent pneumonia
* Exertional breathlessness and unexplained haemoptysis

On examination, there might be:
* Finger clubbing, cyanosis, chest deformity and hyperinflation, persistent inspiratory crackles and wheeze

Question 6

Investigations for bronchiectasis

Answer 6

• Sputum culture- to identify colonising pathogens
• CXR- to exclude other pathology- may not see changes unless severe disease
• Spirometry- to assess the severity of airflow obstruction and identify any co-existent diagnoses
• O2 sats, FBC
• High-resolution computed tomography (HRCT) (thin section scanning) is the most frequently used imaging test to establish the diagnosis- Will see bronchial dilation
• Investigations to identify the underlying cause

Question 7

What investigations should be considered to identify an underlying cause

Answer 7

• Cystic fibrosis testing (sweat chloride or gene testing)
• Screening for gross antibody deficiency
• Specific antibody levels against capsular polysaccharides of Streptococcus pneumonia.
• Test for primary ciliary dyskinesia — for children where no other cause for bronchiectasis is identified

Question 8

When should children with bronchiectasis be reviewed, what does review involve

Answer 8

All children should be reviewed in secondary care at least annually, assessment should include:
* Exacerbation frequency (should refer children with three or more exacerbations a year to a specialist), BMI, sputum sample for bacterial culture, spirometry and O2 saturation, compliance with treatment, assessment of symptoms

Question 9

How can we calculate a baseline severity score

Answer 9

In secondary care using the Bronchiectasis Severity Index

Question 10

Management of bronchiectasis

Answer 10

• The cause of bronchiectasis should be treated (e.g cystic fibrosis)
• Vaccination against streptococcus pneumonia and seasonal influenza should be offered
• Should be taught airway clearance techniques by a physiotherapist

Antibiotic treatment of non-cystic fibrosis bronchiectasis acute exacerbations:
* Can be started empirically, but should be guided by sputum culture sensitivities:
* For patients between 1 month and 11 yo= Amoxicillin/ clarithromycin 1st line, co-amoxiclav 2nd line
* For patients between 12 and 17 yo= Doxycycline 1st line, co-amoxiclav 2nd line
* Empirical IV antibiotics can be used if the child is severely unwell or unable to take oral Abx:
* Co-amoxiclav for children between 1 month and 17 yo (could also give ciprofloxacin following specialist advice)

Question 11

How should acute exacerbations be managed

Answer 11

• An acute exacerbation should be managed in hospital if the child has any features of severe illness, including: cyanosis, confusion, marked breathlessness or laboured breathing, peripheral oedema, at temperature of 38 °C or higher.
• People with bronchiectasis who have three or more exacerbations per year may be offered antibiotic prophylaxis (oral or inhaled) by a respiratory specialist to reduce further exacerbations