Bronchiectasis

Question 1

Definition of bronchiectasis?

Answer 1

Persistent or recurrent bronchial sepsis related to irreversibly damaged and dilated bronchi.

Question 2

Pathological definition?

Answer 2

Abnormal dilation of proximal and medium-sized bronchi (>2 mm) due to destruction of muscular and elastic bronchial wall components.

Question 3

Key pathological changes?

Answer 3

• Transmural inflammation
• Oedema
• Scarring
• Ulceration

Question 4

Was non-CF bronchiectasis previously considered rare?

Answer 4

Yes – termed an “orphan disease” <20 years ago.

Question 5

Is bronchiectasis now recognised as common?

Answer 5

Yes – increasingly recognised in developed countries.

Question 6

Classic symptom of bronchiectasis?

Answer 6

Chronic productive cough with large volumes of sputum.

Question 7

Other key feature?

Answer 7

Recurrent chest infections.

Question 8

Role of chest X-ray?

Answer 8

Low sensitivity; baseline assessment only.

Question 9

Gold standard diagnostic test?

Answer 9

High-resolution CT (HRCT).

Question 10

Broncho-arterial ratio diagnostic threshold?

Answer 10

> 1.

Question 11

Other CT features?

Answer 11

• Lack of tapering
• Airway visible within 1 cm of pleura
• Bronchial wall thickening
• Mucus plugging
• Mosaic perfusion (air trapping)

Question 12

Three morphological types?

Answer 12

Cylindrical, varicose, cystic.

Question 13

Why investigate underlying cause?

Answer 13

Changes management in 7–57% of cases.

Question 14

Examples of management changes?

Answer 14

• IVIG for CVID
• Antibiotics for NTM
• Steroids/antifungals for ABPA

Question 15

COPD patients with what features should be investigated?

Answer 15

≥2 exacerbations/year + previous P. aeruginosa culture.

Question 16

Other high-risk groups?

Answer 16

• Rheumatoid arthritis
• IBD
• Severe asthma
• Immunosuppressed
• Chronic rhinosinusitis
• Persistent cough >8 weeks

Question 17

Prevalence of bronchiectasis in COPD?

Answer 17

~54% (range 26–69%).

Question 18

Associated with worse outcomes?

Answer 18

Yes – more exacerbations, worse lung function, more P. aeruginosa.

Question 19

% of bronchiectasis in UK due to Allergic Bronchopulmonary Aspergillosis?

Answer 19

4%

Question 20

Diagnostic features of Allergic Bronchopulmonary Aspergillosis?

Answer 20

• Clinical deterioration
• Total IgE >500 IU/mL
• Aspergillus-specific IgE
• Radiological infiltrates/proximal bronchiectasis
• Eosinophilia

Question 21

% of bronchiectasis due to immune deficiency?

Answer 21

~5%.

Question 22

Most common immune defect in adults?

Answer 22

Antibody production defects.

Question 23

Why is CVID important to recognise?

Answer 23

IVIG reduces exacerbations.

Question 24

Cardinal feature of CVID?

Answer 24

Recurrent sino-pulmonary infections.

Question 25

Diagnostic criteria for CVID?

Answer 25

* Age >4 years * Low IgG ± IgA/IgM * Impaired vaccine response * Exclusion of other causes

Question 26

Classic triad of Kartagener’s syndrome?

Answer 26

Bronchiectasis + chronic sinusitis + dextrocardia.

Question 27

Primary Ciliary Dyskinesia (PCD) features?

Answer 27

* Recurrent otitis media * Infertility * Situs inversus (~55%)

Question 28

Tests for PCD?

Answer 28

* Nasal nitric oxide * High-speed video microscopy * Electron microscopy

Question 29

Should CF be considered in adults with bronchiectasis?

Answer 29

Yes, especially if <40 years.

Question 30

Clinical clues for adult CF?

Answer 30

* Upper lobe bronchiectasis * Sinus disease * Persistent S. aureus * Male infertility * Malabsorption

Question 31

Diagnostic tests for CF?

Answer 31

Sweat chloride ×2 + CFTR mutation analysis.

Question 32

RA prevalence in bronchiectasis referrals?

Answer 32

2–5%.

Question 33

Goals of bronchiectasis treatment?

Answer 33

* Improve symptoms * Reduce exacerbations * Improve QoL * Preserve lung function * Improve survival

Question 34

Are airway clearance techniques recommended?

Answer 34

Yes – for all patients.

Question 35

Most common airway clearance techniques?

Answer 35

* Active cycle breathing * Autogenic drainage * PEP devices

Question 36

Is DNase recommended in non-CF bronchiectasis?

Answer 36

No – increases exacerbations.

Question 37

Does hypertonic saline help?

Answer 37

Yes – improves QoL.

Question 38

Are ICS routinely recommended?

Answer 38

No (unless coexistent asthma).

Question 39

Indication for long-term macrolides?

Answer 39

≥3 exacerbations/year despite optimal treatment.

Question 40

Example regimen?

Answer 40

Azithromycin 250 mg three times weekly.

Question 41

What must be excluded before starting macrolides?

Answer 41

Active NTM infection.

Question 42

Why is Pseudomonas aeruginosa important?

Answer 42

* More severe disease * Faster FEV1 decline * Increased mortality

Question 43

Independent predictors of mortality?

Answer 43

* Age >70 * FEV1 <30% * BMI <18.5 * Prior hospitalisation * Recurrent exacerbations

Question 44

Broncho-arterial ratio >1 = ?

Answer 44

Bronchiectasis.

Question 45

Chronic productive cough + dextrocardia = ?

Answer 45

Kartagener’s syndrome.

Question 46

Bronchiectasis + high IgE + eosinophilia = ?

Answer 46

ABPA.

Question 47

Bronchiectasis + recurrent sinopulmonary infections + low IgG = ?

Answer 47

CVID.