Bronchiectasis

Question 1

What is it?

Answer 1

Irreversible and abnormal chronic dilatation of one or more bronchi causing poor mucus clearance

there is a predisposition to recurrent or chronic bacterial infection

Question 2

What are the main causes of bronchiectasis?

Answer 2

• Immunodeficiency
• Post infective
• Mucociliary clearance genetic defects
• Bronchial obstruction- - lung tumour, foreign body, extrinsic lymph node
• Toxic insult- gastric aspiration, toxic chemicals /gases inhaled
• Allergic bronchopulmonary aspergillosis (ABPA)
• RA

Question 3

What infections predispose to bronchiectasis?

Answer 3

whooping cough, TB, pneumonia

Eg. typical story is recurrent childhood pulmonary infections

Question 4

Which immunodeficiencies predispose to bronchiectasis?

Answer 4

• Primary:
  
  • Panhypogammaglobulinaemia
  • IgA deficiency
  • IgG deficiency
• Secondary:
  
  • HIV
  • Malignancy

Question 5

Which Mucociliary clearance genetic defects
predispose to bronchiectasis?

Answer 5

• cystic fibrosis
• primary ciliary dyskinesia (PCD)
• Young’s syndrome
• Kartagener syndrome

Question 6

What is youngs syndrome?

Answer 6

bronchiectasis, sinusitis, reduced fertility (associated with mercury exposure)

Question 7

What is Kartagener syndrome?

Answer 7

Kartagener syndrome- bronchiectasis, sinusitis and situs inversus (PCD + situs inversus)

Question 8

What does bronchiectasis have associations with

Answer 8

IBD, Yellow nail syndrome

Question 9

What are common organisms causing Bronchiectasis exacerbation?

Answer 9

Haemophillus influenzae

Pseudonomas aeruginosa

Moraxella Catarrhalis
Stenotrophomonas maltophilia

Fungi- Aspergillus, Candida

Non- tuberculosis mycobacteria

Less common- Staph aureus (CF)

Question 10

How to diagnose a acute exacerbation of disease?

Answer 10

A person with bronchiectasis with a deterioration in 3 or more key symptoms for at least 48 hours:
- Cough
- Sputum volume/purulence
- Sputum colour/ consistancy
- SOB and exercise tolerance
- fatigue
- haemoptysis

Question 11

Differential diagnosis?

Answer 11

asthma, COPD and malignancies

Question 12

Symptoms in brnchiectasis

Answer 12

• Chronic cough
• Persistent large sputum production (Eggcups)
• SOB
• Haemoptysis
• Weight loss/ fatigue/ exercise limitation

Question 13

Signs of examination suggesting bronchiectasis

Answer 13

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

Question 14

How to diagnose bronchiectasis? What results will you get?

Answer 14

High-resolution CT (GOLD STANDARD)- Tram-tracks and signet ring signs

CXR- Tram-track airways (bronchial wall thickening) and ring shadows. Can be normal CXR

Question 15

Blood tests to find cause of newly diagnosed bronchiectasis?

Answer 15

• Immunoglobulin levels- immunodeficiency causes
• Cystic fibrosis genotype
• Aspergillus specific IgE/IgG and serum total IgE
• HIV test
• Rheumatoid factor- RA
• Autoantibodies - (might be rheumatic related)- ANA, anti-CCP, RF
• Alpha-1-antitrypsin level

FBC
U&E

Question 16

What other investigations can be done?

Answer 16

Sputum sample- M, C and S and for nontuberculous mycobacteria- for all patients and during acute exacerbation

Pt with localised disease on imaging - bronchoscopy - check from bronchial obstruction

Question 17

how to check for primary ciliary dyskinesia

Answer 17

features of primary ciliary dyskinesia:
early-onset of symptoms (in childhood/ teenage years), otitis media, rhinosinusitis and male infertility

Question 18

Management overview?

Answer 18

Educate the patient on their condition
Help with smoking cessation, diet and exercise
Immunisations: yearly flu vaccine

Treat underlying cause
Eg. Bronchodilators in patients with coexisting asthma/COPD or in those with significant breathlessness.

Reduce the number of exacerbations
Physiotherapy- mucus/airway clearance
Mucoactives/lytics

Treat acute exacerbations with 10-14 day antibiotics

Question 19

Main organisms causing acute exacerbation and antibiotic treatment?
and for how long?
Side effects if any?

Answer 19

Antibiotics for 10-14 days - use sputum cultures/sensitivities for acute exacerbations

• Haemophilus influenzae- Oral Amoxicillin/Doxy (Ax)
• Pseudomonas Aerug- Oral Ciprofloxacin- may get achilles tendonitis

Question 20

In severe acute exacerbation infections give

Answer 20

IV antibiotics

Question 21

Long-term (prophylactic) antibiotics used in :

Answer 21

for pts with recurrent infective exacerbations(> 3 per year)

despite education, treatment of underlying causes, physiotherapy (+/- mucoactives) should be considered for long term antibiotics.

Question 22

Pulmonary rehabilitation is advised if :

Answer 22

MRC dyspnoea score >= 3

Question 23

Complications that can occur

Answer 23

• Recurrent infections
• Infective exacerbations
• Chronic respiratory failure
• Haemoptysis(may be massive and life-threatening)
• Cor pulmonale
• Pneumothorax
• Chest pain

Question 24

Allergic Bronchopulmonary Aspergillosis (ABPA) is caused by:

Answer 24

Caused by aspergillus fumigatus exposure
Aspergillus is a common fungus found indoors and outdoors

Question 25

ABPA Pathophysiology

Answer 25

ABPA is a combination of types 1 and 3 hypersensitivity reactions following inhalation of fungal spores i.e. it is not a fungal infection Repeated damage from these immunological reactions leads to bronchiectasis (often upper lobe)

Question 26

Who is ABPA seen in?

Answer 26

ABPA is seen more in patients with Asthma, Bronchiectasis and Cystic Fibrosis

Question 27

How is ABPA diagnosed ?

Answer 27

Combination of symptoms (often dry cough and wheeze) Positive blood tests (raised Aspergillus IgE level as well as a high Total IgE – these are often accompanied by a high eosinophil level too)

Question 28

Treatment of ABPA?

Answer 28

Steroids may be required if ongoing symptoms and high Total IgE level