Bronchiectasis

Question 1

What are the causes of bronchiectasis?

Answer 1

Focal (Obstructive)

• Luminal Blockage: Foreign body, tumour, broncholith, mucus plug
• Extrinsic: Enlarged LN, Post-lobar resection

Diffuse (Infective VS non-infective)

• Post-infectious (2nd most common): Bacteria (Pseudomonas, Haemophilus, Pertussis), TB, Viral (Adenovirus, measles, influenza)
• Hypersensitivity: Allergic bronchopulmonary Aspergillosis (ABPA)
• Congenital: Cystic fibrosis, Alpha-1 antitrypsin deficiency (also causes COPD), Kartagener’s syndrome (i.e. Immotile ciliary syndrome / Primary ciliary dyskinesia), Hypogammaglobulinemia
• AI: RA, SLE, Sjogren’s
• IBD, Yellow nail syndrome, Young’s syndrome, Idiopathic (common)
• COPD: increases risk of infection + secondary ciliary dyskinesia

Question 2

What are the features of hx that point towards a diagnosis of bronchiectasis?

Answer 2

Age

Smoking history

Family history – A1AT deficiency etc

Sputum with P. aeruginosa (chronic colonization, 3 months)

Unexplained respiratory symptoms

Past Medical History

• Infertility, sinus/nasal disease (signs of ciliary dysfunction)
• History of TB
• Childhood infections
• History of COPD 🡪 Any lung lesion puts you at a risk of increased infection 🡪 hence other lung diseases
• Others (see etiologies above)

Question 3

What are the symptoms of bronchiectasis?

Answer 3

Cough (>90%) 🡪 however 10% will have no cough – dry bronchiectasis, will require HRCT diagnosis!

Sputum (usually productive, but can also be non-productive)

• 75-100% daily
• 12-20% intermittent
• 5-8% non-productive

Sputum colour (mucoid, mucopurulent, purulent)

Volume: teaspoon (5ml), dessert spoon (10ml), table spoon (15ml), tea cup (200ml)

Shortness of breath (72-83%)

Haemoptysis (45-51%): blood stained (27%), 10ml (20%), massive >235ml (4%)

Chest pain: non-pleuritic

Febrile: acute exacerbation

Question 4

What are the signs of bronchiectasis?

Answer 4

Inspection:

• Finger clubbing
• Cyanosis
• Productive cough + possible haemoptysis

Trachea

• Central
• Deviated (towards affected side in severe focal disease)

Lung volume
- Chest expansion reduced on affected side (focal) OR bilaterally (diffuse)

Percussion & vocal resonance: Normal

Auscultation

• Persistent pan-inspiratory coarse crepitation that changes character on coughing
• Wheeze

Question 5

What are the investigations to be conducted for bronchiectasis?

Answer 5

Bloods

• FBC, CRP, ESR
• Sputum Culture & Sensitivity; TB PCR; AFB, +/- Blood Culture
• Lung function tests (spirometry with reversibility test) – usually obstructive pattern
• +/- ABG

Imaging

• CXR features
• Parallel lines (tram track lines)
• Ring shadow (transverse cuts of dilated airways)
• Indistinct vessel margins
• Volume loss (elevated hemidiaphragm)

HRCT features *** 😊 – must do HRCT to Dx Bronchiectasis!

• Visible airways <1cm from surface (Indicating enlarged PERIPHERAL airways)
• Signet ring sign (dilated bronchi larger than adjacent artery)
• Track track sign w/ lack of bronchial tapering (most sensitive)
• Indirect signs: mucus plugging, lobar volume loss, wall thickening

Others (once bronchiectasis is confirmed)

• TB – Sputum culture, AFB, PCR
• Hypogammaglobulinemia – IgG, A, M, G-subclasses levels
• Allergic Broncho-pulmonary aspergillosis – Sp IgEA1
• Infection – Titres to pneumococcal vaccine, pertussis and HiB
• Cystic Fibrosis – Sweat test (2 measurements) & CFTR genetic mutation analysis
• Rheum Conditions – Autoimmune markers: ANA RF, aCCP, SSA, SSB antibodies
• A1ATD – α1-antitrypsin level and phenotype (commonly lower lobe involvement)

In some cases:

• Bronchoscopy (diagnostic/therapeutic)
• Gastrointestinal evaluation (IBD)
• Nasal nitric oxide testing (Primary ciliary dyskinesia – will be low; allergic diseases – will be high)

Question 6

What is the management of bronchiectasis?

Answer 6

Conservatively, I will encourage vaccinations for respiratory infections and smoking cessation. I will prescribe chest physiotherapy to strengthen the muscles of respiration.

Medically, mainstay of chronic Mx is airway clearance achieved by hypertonic saline nebulisation and bronchodilators. I will also provide Abx for infective exacerbations and treat underlying cause such as Tb

Surgically, I can consider resection of diseased / bleeding lobes, or lobes w/ drug resistant Tb

Question 7

What is infective exacerbation of bronchiectasis?

Answer 7

Definition: a change in one or more of the common symptoms of bronchiectasis (increasing sputum volume or purulence, worsening dyspnoea, increased cough, declining lung function, increased fatigue/malaise) OR the appearance of new symptoms (fever, pleurisy, haemoptysis), AND requiring antibiotic treatment

Donnell’s 4/9 symptoms of exacerbations

• Increased dyspnoea
• Increased cough
• Increased sputum production
• Increased wheezing
• Fever
• Lethargy, malaise
• Changes in chest sounds
• Reduced pulmonary function
• Radiographic changes consistent with a new pulmonary process

Question 8

What are the non pharmacological and conservative treatments of bronchiectasis?

Answer 8

Education, counselling

Smoking cessation

Vaccinations (yearly influenza and 3-year pneumococcal)

Airway Clearance

• Encourage chest physio – to strengthen respi muscles 🡪 reduce symptoms hence improve QoL
• Note: Chest Physio =/= Pul Rehab. Pul Rehab is for symptomatic relief, chest physio is to strengthen muscles
• Nebulised Agents: NAC / Hypertonic saline if thick sputum – mainstay of therapy

Bronchodilator therapy such as beta agonists and anticholinergics to treat airflow obstruction

• Reduce airway inflammation
• Improve lung function (FEV1) and reduce sputum volume
• No effect on mortality-
• Mainly given due patients for suspected COPD co-existence, will not be useful in a “pure” bronchiectasis

Chronic respi failure: non-invasive ventilation for ↑ QoL

Question 9

What are the pharmacological management to treat infective exacerbation of bronchiectasis?

Answer 9

Antibiotics
- Sputum samples for culture before starting Abx
- Antibiotics targeting (1-2 weeks): Haem, Pseudomonas, Strep, Moraxella
- Fluoroquinolones
- Others
• MAC – Rifampicin, ethambutol, Azithromycin till culture negative for 1 year
• ABPA – augmentation of corticosteroids and use of itraconazole 200mg BD for 4 weeks then 200mg OM for 4 more weeks
• Aerolised recombinant human DNAse for cystic fibrosis (not for other causes of bronchiectasis)

Question 10

What is the surgical management of bronchiectasis?

Answer 10

Surgical Indications: localised disease, failed medical treatment

Focal: Removal of obstructive tumour or FB

Diffuse:

• Remove segments that are most damaged and contributing to recurrent acute exacerbations
• Remove segments involved with uncontrolled haemorrhage
• Removal of segments suspected of harbouring drug resistant organism such as MDR MTB or MAC

Lung transplant for congenital causes eg: cystic fibrosis bronchiectasis