Bronchiectasis (RESP)

Question 1

Define bronchiectasis.

Answer 1

Permanent abnormal dilation of bronchi secondary to chronic infection or inflammation, due to the destruction of the elastic and muscular components of the bronchial wall + impaired mucociliary clearance

Question 2

What is bronchiectasis a consequence of?

Answer 2

Recurrent and/or severe infections secondary to an underlying disorder

Question 3

How can you classify bronchiectasis? (2)

Answer 3

• CF bronchiectasis
• non-CF bronchiectasis

Question 4

What are some causes of bronchiectasis? (11)

Answer 4

• idiopathic (50%)
• post-infective/recurrent pulmonary infections (measles, pertussis, pneumonia)
• cystic fibrosis - autosomal recessive disorder causing increased viscosity of secretions due to defect in CFTR
• TB
• COPD
• aspiration
• chronic inflammatory diseases e.g. RA, coeliac disease
• allergic bronchopulmonary aspergillosis (ABPA) - bronchiectasis + bronchoconstriction caused by hypersensitivity response to aspergillus fungus
• primary ciliary dyskinesia (Kartagener’s syndrome) - bronchiectasis + dextrocardia (or complete sinus inversus) + recurrent sinusitis (+ male infertility)
• bronchial obstruction (lung cancer, foreign body)
• immune deficiency (selective IgA, hypogammaglobulinaemia)

Question 5

What are the clinical features of cystic fibrosis (bronchiectasis)? (5)

Answer 5

• recurrent chest infections
• failure to thrive (delayed puberty)
• malabsorption (steatorrhoea)
• short stature
• male infertility

Question 6

How is cystic fibrosis (bronchiectasis) diagnosed?

Answer 6

Via sweat test (abnormally high sweat chloride)

Question 7

How is cystic fibrosis (bronchiectasis) screened?

Answer 7

At birth via heel prick

Question 8

How do you manage cystic fibrosis (bronchiectasis)?

Answer 8

Chest physiotherapy

Question 9

What are the clinical features of allergic bronchopulmonary aspergillosis (bronchiectasis)? (3)

Answer 9

• wheeze
• dry cough
• dyspnoea

Question 10

What would you find on bloods in allergic bronchopulmonary aspergillosis (bronchiectasis)? (2)

Answer 10

• eosinophilia
• raised IgE

Question 11

How do you manage allergic bronchopulmonary aspergillosis (bronchiectasis)?

Answer 11

Oral prednisolone

Question 12

How does obstructive disease cause bronchiectasis?

Answer 12

Mucus plugs form in airways and obstruct airways

Question 13

What are the most common organisms that cause bronchiectasis? (4)

Answer 13

• Haemophilus influenzae (most common)
• Pseudomonas aeruginosa
• Klebsiella spp.
• Streptococcus pneumoniae

Question 14

What are the clinical features of bronchiectasis? (10)

Answer 14

• chronic cough associated with large/copious amounts of purulent sputum (green/rusty colour) - may be worsened lying flat/on one side
• haemoptysis (red-tinged sputum)
• recurrent chest infections
• SOB (especially on exertion)
• chest pain
• malaise
• fever
• weight loss
• symptoms usually begin after acute respiratory illness + worsen during acute exacerbations
• acute exacerbation: fatigue, weight loss, wheezing

Question 15

What might you see on examination in bronchiectasis? (3)

Answer 15

• clubbing
• coarse inspiratory crackles - usually at lung base, shift with coughing, rhonchi, high-pitched inspiratory squeaks
• wheeze (high-pitched inspiratory squeaks)

Question 16

What are the risk factors for bronchiectasis? (6)

Answer 16

• cystic fibrosis
• host immunodeficiency
• previous infections
• congenital disorders of the bronchial airways
• primary ciliary dyskinesia
• tall, thin, white females 60+ –> pulmonary non-TB mycobacteria-related bronchiectasis

Question 17

What are the 1st-line investigations for bronchiectasis? (6 + 6)

Answer 17

• high-resolution chest CT
• CXR
• FBC
• sputum culture + sensitivity
• sweat chloride test
• pulmonary function tests
• (serum alpha-1 antitrypsin phenotype and level)
• (serum immunoglobulins)
• (rheumatoid factor)
• (specific IgE or skin-prick test to Aspergillus fumigatus)
• (serum HIV antibody)
• (nasal NO)

Question 18

What initial investigation do you request for bronchiectasis and what would this show? (7)

Answer 18

CXR

• obscured hemidiaphragm
• thin-walled ring shadows with/without fluid levels
• tubular or ovoid opacities
• dilated bronchi –> tram lines (parallel line shadows from hilum to diaphragm)
• fibrosis
• atelectasis
• pneumonic consolidation

Question 19

What is the gold-standard confirmatory test for bronchiectasis and what would it show? (5)

Answer 19

High resolution chest CT

• thickened, dilated airways
• varicose constrictions along airways
• cysts and/or tree-in-bud pattern
• signet ring sign
• tram-track sign

Question 20

What does spirometry show in bronchiectasis?

Answer 20

Obstructive pattern (<0.7) - assess reversibility

Question 21

Why do we do sputum MC&S for bronchiectasis?

Answer 21

To look for underlying cause - may find H. influenzae (most common) or Pseudomonas aeruginosa (most common in CF patients)

Question 22

What would we see in CF on the cystic fibrosis sweat test?

Answer 22

Abnormally high chloride

Question 23

What are some differential diagnoses for bronchiectasis? (4)

Answer 23

• COPD - diminished breath sounds, no inspiratory squeaks, chest CT normal/emphysema
• asthma - no inspiratory squeaks
• pneumonia - more acute
• chronic sinusitis - no inspiratory squeaks, CXR and CT normal

Question 24

What is included in the management plan for bronchiectasis? (7)

Answer 24

• exercise and improved nutrition
• airway clearance therapy (chest physiotherapy):
  
  • postural drainage
  • percussion
  • vibration
  • maintenance of oral hydration
• inhaled bronchodilator (salbutamol)
• mucoactive agent e.g. nebulised hypertonic saline
• antibiotics for exacerbations (e.g. amoxicillin, vancomycin) + long-term rotating antibiotics in severe cases (refractory to medical treatment)
• immunisations
• surgery (lobectomy)

Question 25

When is surgery done for bronchiectasis?

Answer 25

Lobectomy in uncontrolled haemoptysis or localised disease

Question 26

Why do we do inspiratory muscle training in bronchiectasis?

Answer 26

Good evidence base for patients with non-CF bronchiectasis

Question 27

What long term prophylactic Abx can be used in Pseudomonas aeruginosa bronchiectasis infections?

Answer 27

Oral macrolides or inhaled aminoglycosides

Question 28

What are some complications of bronchiectasis? (4)

Answer 28

- massive haemoptysis - respiratory failure - cor pulmonale - ischaemic stroke

Question 29

Describe the prognosis of bronchiectasis.

Answer 29

Irreversible condition, the typical disease course consists of periods of symptom control interrupted by periods of exacerbations