Bronchiectasis. Grant Waterer. Professor of Medicine, University of Western Australia Adjunct Professor of Medicine, Northwestern University, Chicago
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1 Bronchiectasis Grant Waterer MBBS PhD MBA FRACP FCCP Professor of Medicine, University of Western Australia Adjunct Professor of Medicine, Northwestern University, Chicago
2 Conflicts of Interest I have served on advisory panels for Pharmaxis (Bronchitol) Savara Pharmaceutical (aerovanc) AstraZeneca/GSK/Almirall I will discuss off-label indications of some medications
3 What is bronchiectasis?
4 Damaged, enlarged bronchi
5 Bronchiectasis is a disease with a large spectrum of severity Asymptomatic Disease Classic Severe Disease
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11 How common is bronchiectasis?
12 Prevalence of Bronchiectasis in the USA from Medicare Claims Database Seitz et al Chest 2012
13 What causes bronchiectasis?
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15 What causes bronchietasis? Worldwide Tuberculosis is no.1 In Australia don t know is no.1 Atypical mycobacterial infection Childhood pneumonia/severe pneumonia Various immune defects COPD Lung fibrosis (ILD, rheumatoid etc) Many rare causes
16 Approach to a new patient with bronchiectasis White cell count Immunoglobulins including IgG subclasses and IgE ANA + Rheumatoid factor if hx CTD CF genotype Family history, sinusitis, infertility, Pseudomonas, <30 years onset Mannose binding lectin Alpha-1-antitrypsin? HIV? Sputum cultures Bronchoscopy if suspect NTM Exhaled NO if want to screen for cilial disorders
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18 Non tuberculous mycobactera Women 9x men Anterior segments of RML/lingula Nodules +/- bronchiectasis In men COPD/alcohol overwhelming comorbidities Women almost all post menopausal Strong genetic features Environmental exposure also important
19 What is going on in bronchiectasis?
20 Chronic Bacterial Infection Inflammatory Response Remodeling/Fibrosis Suppressed Immune Response
21 So what can we do about it?
22 Treatment options Oral antibiotics Macrolide, tetracycline, beta-lactams Inhaled antibiotics Nebulized aminoglycosides Mucolytics Hypertonic/normal saline, NAC, mannitol Non Medical Therapies Physiotherapy/mucus clearance
23 Treatment pathway PRODUCTIVE COUGH 2/52 Iv AB s ACTIVE CYCLE OF BREATHING (Huff + Puff) + ACAPELLA/FLUTTER Nebulized Gentamicin 80mg bd Or Tobramycin 160mg bd OR DPI Tobramycin Doxycycline 50mg/day Azithromycin 500mg/ 3x week or 250mg/day
24 Airway Clearance Is Critical Reduce the soup of inflammatory material Reduce the food for bacterial growth Improve ventilation by lung recruitment but I don t want to cough
25 Airway clearance is critical Basic techniques Active cycle of breathing, huff and puff, percussion, exercise! Humidification And avoidance of dehydration Mechanical Acapella, flutter, PEP +/- oscillation, cough assist, cough vests etc Chemical Mannitol, Hypertonic saline, N-acetyl saline, Beta-2 agonists
26 Flutter Uses a steel ball to produce oscillations Exhalation through flutter provides a low level of PEP while producing vibrations extending to the chest Advantages: Easy to clean Portable Inexpensive (~$50) Disadvantages: Requires being held horizontally May be heavy for elderly patients
27 Acapella A flow operated oscillatory PEP device Uses a counterweighted plug and magnet Green expiratory flows of >15 L/minute Blue expiratory flows <15L/minute (not available at NMH) Advantages: Able to vary amount of PEP included (resistance dial on tail) May use in any position Lighter then Flutter Inexpensive (~$50) Disadvantages: Harder to clean Larger than flutter
28 Exacerbation rate/year 3 Macrolide RCT s Drug Placebo BLESS BAT EMBRACE
29 Azithromycin responders Han et al Am J Respir Crit Care Med 2014 Sub-analysis of the Albert et al NEJM 2011 Time to first exacerbation Smoking negated a benefit HR 0.65 ex-smokers, HR 0.99 current smokers Chronic bronchitis no impact on effect HR 0.76 ( ) CB+ HR 0.64 ( ) CB-
30 Doxycycline is a simpler treatment to start with if you don t have Pseudomonas Intermittent high dose amoxycillin also has some evidence
31 Nebulised antibiotics if you do have Pseduomonas and lots of exacerbations
32 Drug companies are finally interested in bronchiectasis!
33 Inhaled antibiotics in trials Ciprofloxacin DPI Liposomal ciprofloxacin Levofloxacin DPI Vancomycin DPI + nebulisation Amikacin nebulisation Aztreonam nebulisation Tobramycin DPI + nebulisation Fosfomycin/Tobramycin nebulisation Colistin DPI + nebulisation
34 What the don t tell you in the text books You can t run a bronchiectasis service without a good physiotherapist (respiratory therapist) Urinary incontinence Physiotherapy Uro/Gynae review Codeine for social events Always ask could this be surgically resected
35
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