Bronchiectasis

Question 1

Bronchiectasis history?

Answer 1

P: dx date, symptoms

R: congenital (CF, Kartagener’s, congenital hypo-gamma), acquired (childhood infection [TB, Measles, Whooping = have you had childhood MMR], recurrent aspiration, FB, ABPA, RA, Sjogren’s)

I: Most recent CT scan, PFT, sweat test, ciliary function test

C: haemoptysis, recurrent PNA, colonisers (LT ABx?)

M: chest PT, postural drainage, pulmonary rehab, vaccinations, chronic ABx, bronchodilators

C: admissions last 12 months, ET, how well coping, FU

P: prognosis

Question 2

Causes of bronchiectasis?

Answer 2

Congenital: CF, Kartagener’s, congenital hypogamma, yellow-nail

Acquired:

childhood infections (measles, whooping)

localised (tumour, bronchial adenoma FB, TB)

ABPA, RA, Sjogren’s

Recurrent aspiration

ILD

Idiopathic (50%)

Question 3

What are the complications of bronchiectasis? (7)

Answer 3

Recurrent infection

Empyema

Lung abscess

Pleurisy

Cor-pulmonale

Brain abscess

Amyloid (rare, but important for exam)

Question 4

What is your approach to investigating bronchiectasis patient p/w SOB?

Answer 4

T: CXR, HRCT to confirm diagnosis

E:

Exclude exacerbating factors: infection (inflammatory markers & septic work-up + sputum MCS), PE (CTPA), ECG (ischaemia), FBC (anaemia), GORD

Exclude secondary causes: IGs, sweat test, ciliary function test (young), blood film for eoninophilia, IgE, precipitant (ABPA)

S: ABG (hypoxia, acidosis, hypercapnoea), PFT (FEV1 - if <40% severe disease, DLCO)

Screen for complications - TTE (cor-pulmonale)

Question 5

What are the CXR features of bronchiectasis? (3)

Answer 5

Dilated bronchi & bronchioles - tram-tracking sign (parallel line opacities)

Signet-ring (dilated airway in transverse plane adj to pulmonary artery giving appearance of ring)

Cystic changes (cluster of thin walled cystic spaces)

Question 6

What are the CT features of Bronchiectasis?

Answer 6

Same as CXR. Typical finding is airway dilatation, defined as diameter ≥ accompanying branch of pulmonary artery.

Signet-ring

Tram-tracking

Cystic changes

Question 7

What is your approach in managing this patient’s bronchiectasis?

Answer 7

Goals

• Minimize symptoms, improve function, prevent complications

Confirm diagnosis

• HRCT/CXR - airway dilatation, tram-tracking
• Spirometry - usually reduced FEV1 and low FFR
• Sputum (colonising micro-organisms)

Association / Causative / Exacerbating

• Exclude exacerbating factors: infection (inflammatory markers & septic work-up + sputum MCS + BAL + AFB), PE (CTPA), ECG (ischaemia), FBC (anaemia), GORD
• Exclude secondary causes IF NOT DONE already: IGs, sweat test (elevated Cl^-), ciliary function test (young), eosinophilia, Aspergillus specific & serum total IgE, skin prick test (ABPA)

Non-pharm

• Difficult Mx problem requires continuous support and MDT approach
• Educate: the importance of sputum clearance, chest PT and infection prevention
• Sputum clearance: PT, postural drainage, PEP, flutter valves, hypertonic saline nebs
• Link with Pulmonary rehabilitation (improves ET, QOL)
• Direct dietician - HPHE diet (improves strength/physical function in pul rehab patients)
• Infection prevention, vaccinations

Pharm

• Acute exacerbations: guided by sensitivity. For Pseudomonas colonisers, cipro or tazocin.
  
  • ABPA - 0.5-1mg/kg Prednisolone for 2 weeks - taper over 3-6 months. Consider 4 month course of Itraconazole or Voriconazole.
• Preventing exacerbations
  
  • Bronchodilators, if evidence of airway obstruction. Improves SOB, cough, QOL.
  • ICS - controversial: theoretical benefit in reducing inflammation (mechanism for bronchiectasis) but increased risk of Pseudomonas (OR=1.8) + side FX (thrush, HPA, infection, small but sig BMD) - individualized.
  • Prophylactic ABx (macrolide,exclude MAC) - if ≥2 exacerbations/yr. If macrolide intolerant - amoxicillin 500mg BD or Doxycycline 100mg BD (not if Pseudomonas colonisers)
  • Irradicate Pseudomonas - inhaled tobramycin (1st line): nebulised, BD for 28 days. Other options are aztreonam, colistin, gentamycin, ciprofloxacin.
  • IVIG (if deficient), inhaled NAC or DNAse (lacks data), CTA / embolisation (massive haemoptysis >250mls) lung resection for localised disease
  • Statins: anti-inflammatory - improves cough, check Chol/LDL and start if not in target.
  • PPI: if symptoms of reflux (~60% has reflux, study showed and had more severe disease)

F/U

• Regular sputum MCS - the aim is early detection of new buts - clear pseudomonas with neb-tobramycin
• HRCT, PFT…etc
• Cardiovascular exam & TTE (Cor-pulmonale)
• If CF: psychosocial support, counseling, caseworkers, nutrition, compliance, fertility, assess the need for transplant