Bronchiectasis

Question 1

Define bronchiectasis

Answer 1

Bronchiectasis = lung disease characterised by abnormally chronic bronchial dilation – featuring impaired mucociliary clearance and frequent bacterial infections

Question 2

Explain the aetiology / risk factors of bronchiectasis

Answer 2

Often secondary to severe/recurrent infections
Severe inflammation in lung -> fibrosis + bronchial wall damage + cilia loss -> bronchial dilation –> pooling of mucus -> further cycles of infection, damage and fibrosis

Many different causes - Idiopathic (50%)

Impaired drainage (genetic) - CF, Alpha 1 anti-trypsin def

Post-infectious - Severe bacterial pneumonia, TB, Aspergillus fumigatus, ABPA
Childhood – whooping cough, measles, influenza, pertussis

Host defence defects/Immunocompromised - Immunoglobulin deficiency, HIV, CF = most common identifiable cause, Kartagener’s syndrome (Primary ciliary diskinesia)

Obstruction - Inside lumen – foreign body
Within wall – connective tissue disorder (e.g. RA)
Outside wall – tumour, lymph nodes

GI - IBD, GORD

Question 3

Summarise the epidemiology of bronchiectasis

Answer 3

Often initially arises in childhood

Incidence has decreased
o Use of Abs
o Approximately 1 in 1000 per year

Question 4

Recognise the presenting symptoms of bronchiectasis

Answer 4

Productive cough with purulent sputum or haemoptysis – chronic (+ worsened during exacerbation)
o Sputum green or rust
o Worse at night + lying down

SOB

Chest pain

Malaise, fever, fatigue, weight loss (acute respiratory illness) - FLAWS

Symptoms usually begin after an acute respiratory illness -> INCREASED sputum production, change in colour and WORSENING cough

Question 5

Recognise the signs of bronchiectasis on physical examination

Answer 5

Clubbing

Auscultation
o	Crackles = coarse crepitations, Usually at bases
Shift with coughing
o	High pitched inspiratory squeaks
o	Rhonchi
o	Wheeze

Underlying cause
o Connective tissue disorder/rheumatoid arthritis
o FLAWS – tumour
o Mouth sores, rashes, pallor – immunosuppression

Question 6

Identify appropriate investigations for bronchiectasis

Answer 6

CXR – 1st line
o May be normal
o Tram lines = parallel lines radiating from hilum to the diaphragm
o Fibrosis – consolidation (impacted mucus) – large lung volumes – thin walled ring shadows

High res CT – GOLD STANDARD
o Thickened dilated airways with/without air fluid levels
o Signet ring/ string of beads sign

Bloods
o	FBC: eosinophilia (in aspergillosis), neutrophilia (infection)
Serum HIV 
o	Serum immunoglobulins
o	Serum rheumatoid factor
o	Serum alpha 1 anti-tryspin
Sputum MC+S

Common organisms
Gram negative = pseudomonas aeruginosa, haemophilus influenza, Klebsiella pneumoniae
Gram positive = Staphylococcus aureus, Streptococcus pneumoniae,
Mycobacteria

Ask for a CF bacterial culture
•	Sweat chloride test – CF
o	>60mmol/L = CF likely
Skin prick test – aspergillus sensitivity
o	Elevated IgE
Sinus X-ray
Mucociliary clearance study

Question 7

Generate a management plan for bronchiectasis

Answer 7

Acute
o 2 IV Abs with pseudomonas efficacy
normal: amoxicillin, clarithromycin, trimethoprim
pseudomonas: fluoroquinolone (ciprofloxacin) or aminoglycosides (gentamicin)

Chronic
o Exercise and nutrition - Pulmonary rehabilitation, Hydration
o Inhaled bronchodilators - Salbutamol
o Physiotherapy - Airway clearance therapy
o Nebulised hyperosmolar agent - Hypertonic saline
o ICS
o Prophylaxis Abs - CF, Frequent infections
o Surgical - Localised resection, Lung transplantation

Question 8

Identify the possible complications of bronchiectasis

Answer 8

• Massive haemoptysis
• Persistent infections
• Empyema
• Resp Failure
• Cor-pulmonale (Right sided heart failure)
• Multi-organ abscess

Question 9

Summarise the prognosis for patients with bronchiectasis

Answer 9

Irreversible condition
o Typical disease consists of periods of symptom control with exacerbations
o Most are still symptomatic after 10 years

Dyspnoea and sputum production have greatest impact on quality of life