Bronchiectasis

Question 1

What is Bronchiectasis?

Answer 1

Irreversible and abnormal dilatation of the airways

Question 2

What are the general categories associated with the cause of bronchiectasis?

Answer 2

1. Infective insults plus defective mucociliary clearance
2. Airway obstruction
3. immunodeficiency - Impaired host immunity

Question 3

How do immunodeficiency’s lead to bronchiectasis’s?

Answer 3

Immunodeficiencies predispose patients to infection. Recurrent infective insults, combined with a poor host immune response, increase the risk of developing bronchiectasis.

Question 4

Which immunodeficiency’s are associated with bronchiectasis? (primary and secondary)

Answer 4

Primary -
Panhypogammaglobulinaemia
IgA deficiency
IgG deficiency

Secondary
HIV
Malignancy

Question 5

Which airway obstructions are associated with bronchiectasis?

Answer 5

Foreign bodies (particularly in children)
mucus plugging
stenosis
tumours 
lymph nodes

Question 6

How does Mucociliary dysfunctions lead to bronchiectasis?

Answer 6

Dysfunction leads to mucus accumulation and predisposes to recurrent infection

Question 7

Which Mucociliary dysfunctions are associated with bronchiectasis?

Answer 7

Primary ciliary dyskinesia

• autosomal recessive pattern
• immotile cilia often resulting in recurrent infections and bronchiectasis
• associated with early-onset of symptoms (in childhood/ teenage years), otitis media, rhinosinusitis and male infertility.

Young syndrome -

• characterised by male infertility (obstructive azoospermia), sinusitis and bronchiectasis
• pathogenesis is poorly understood but may feature impaired mucociliary clearance.

Question 8

What is Kartagener syndrome?

Answer 8

primary ciliary dyskinesia combined with situs inversus.

Question 9

Which congenital airway defects are associated with bronchiectasis?

Answer 9

Williams–Campbell syndrome:
- defective cartilage in the airways (fourth to sixth division) resulting in bronchiectasis.

Mounier-Kuhn syndrome:
- characterised by dilatation of the trachea itself as well as the bronchi.

Question 10

Which other conditions are associated with bronchiectasis?

Answer 10

Rheumatic diseases - rheumatic arthritis and Sjogren’s syndrome

Allergic bronchopulmonary aspergillosis - exaggerated immune response to Aspergillus, tends to occur in asthmatics

COPD

Inflammatory bowel disease

Question 11

How does COPD lead to bronchiectasis?

Answer 11

patients with COPD who smoke are at increased risk of repeated infection and this may lead to bronchiectasis.

Question 12

What are the signs and symptoms of bronchiectasis?

Answer 12

Symptoms -

• Persistent sputum production (purulent/mucopurulent sputum)
• Chronic cough
• Dyspnoea
• Haemoptysis
• Weight loss

Signs

• Crackles
• High pitched inspiratory squeaks
• Wheeze
• Clubbing (rare)

Question 13

Describe acute infective exacerbations of bronchiectasis. Which symptoms are associated with this?

Answer 13

When the abnormal and dilated airways have impaired ability to clear sputum and pathogens from the lungs resulting in increased risk of infection.

There is worsening of chronic features (dyspnea, cough and sputum production) as well as those of systemic infection such as fever and malaise.

Question 14

Which imaging tests are used to diagnose Bronchiectasis? Which of theses imaging tests is the main mode of diagnosis?

Answer 14

Chest X ray- Routine first line investigation
- Can be normal or show non specific abnormalities

Thin section CT - Dilated airways with an increased bronchoarterial ratio is seen.
signet ring sign

Bronchoscopy-
locate site of haemoptysis, Exclude obstruction
obtainsamples for culture

Question 15

What causes in the signet ring sign in bronchiectasis?

Answer 15

caused by the cross-section of a dilated bronchus with its accompanying branch of the pulmonary artery.

Question 16

Which tests and blood test are conducted to investigate the cause of bronchiectasis?

Answer 16

Blood tests -

• FBC
• renal function
• Serum immunoglobins - if elevated do serum protein electrophoresis

Cultures
- Sputum cultures -performed on all patients. if p.aeruginosa positive is also highly suspicious for bronchiectasis
Cystic fibrosis sweat test

Blood cultures - in patients presenting with fever or signs of systemic infection.

Aspergillus fumigatus

• Serum total IgE
• Sensitisation assessment (specific IgE or skin prick test)

Those with features of arthritis, vasculitis, or conncetive tissue disease

• Rhumatoid factor
• anti-cyclic citrullinated peptide
• antinuclear antibodies (ANA)
• anti-neutrophil cytoplasmic antibodies (ANCA)

Question 17

How do childhood conditions contribute to the development of bronchiectasis?

Answer 17

Child hood conditions like whopping cough, umps and rubella can damage the lungs and predispose individuals to bronchiectasis

Question 18

When should a Bronchoscopy be considered and why?

Answer 18

should be considered in people with localised disease on imaging to check for a local obstruction - e.g foreign body.

Question 19

What are the goals in the management of bronchiectasis?

Answer 19

educate the patient on their condition,

treat any underlying cause

reduce the number of exacerbations they suffer.

Question 20

Which lifestyle changes should be made by those with bronchiectasis?

Answer 20

smoking cessation, diet and exercise.

Question 21

What are the management options for people with bronchiectasis? (7 treatments)

Answer 21

Airway clearance -
- Respiratory physiotherapist should teach airway clearance techniques e.g. active cycle of breathing techniques

Mucoactives

• agents that help the clearance of mucus from the airways.
• indicated in patients with ongoing exacerbations (e.g. > 3 / year) despite optimal conservative measures.
• Isotonic and hypertonic saline may be used
• Oral carbocisteine may be trialled and continued if it offers a benefit.

Prophylactic antibiotics
- Patients with recurrent exacerbations (> 3 per year) despite education, treatment of underlying causes, physiotherapy (+/- mucoactives) should be considered for long term antibiotics.

Treatment depends whether or not the patient is colonised by P. aeruginosa:

Colonised - inhaled colistin and gentamicin. Oral azithromycin or erythromycin can be considered in those who don’t tolerate inhaled therapy or in addition to inhaled therapy where it is ineffective.

Not colonised - azithromycin or erythromycin may be used. Inhaled gentamicin can be considered second line.

Bronchodilators
- Bronchodilators can be used in patients with coexisting asthma/COPD or in those with significant breathlessness.

Surgery -
Lung resection - localised disease + uncontrolled symptoms

Lung transplant -
patients aged 65 or younger with an FEV1 < 30% and clinical instability or rapidly deteriorating lung function despite medical intervention. Other indications include complications such as massive haemoptysis and pulmonary hypertension.

Question 22

How are Infective exacerbation treated?

Answer 22

Patients require prompt clinical assessment and basic investigations including CXR, blood tests, sputum and blood cultures where indicated.

sepsis should be managed with the sepsis 6 principles

Antibiotic therapy may guided by previously obtained cultures

Question 23

What are the complications of Bronchiectasis? (8)

Answer 23

Infective exacerbation

Chronic respiratory failure

Haemoptysis (may be massive and life-threatening)

Cor pulmonale

Pneumothorax

Pleural effusion

cerebral abscess

amyloidosis.

Question 24

What is the prognosis of Bronchiectasis?

Answer 24

he prognosis of bronchiectasis is closely linked with the underlying severity of disease.

Patients with mild disease may have a life-expectancy that is equal to the general population. Those with more severe disease tend to have a reduced life expectancy.

Question 25

Name the main organisms which cause infection in Bronchiectasis?

Answer 25

H. influenzae; Strep.

pneumoniae; Staph. aureus; Pseudomonas aeruginosa