bronchiectasis exacerbations; differences and management
TRANSCRIPT
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Bronchiectasis exacerbations; differences and management
Michael LoebingerRoyal BromptonImperial College
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Plan
Bronchiectasis background and burden
Cases and practical management
Exacerbation and Management
Longer term Management
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Bronchiectasis
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What is the prevalence of bronchiectasis in the UK ? (x600 for number)
1) 1/100000
2) 10/100000
3) 100/100000
4) 500/100000
5) 1000/100000
6) nobody knows
7) I don’t know
0
4
13
75
12
7
1 2 3 45 6 7
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What is the prevalence of bronchiectasis in the UK ?
1 1/100000
2 10/100000
3 100/100000
4 500/100000
5 1000/100000
6 nobody knows
7 I don’t know
Prevalence
52/100000 adults in US (Weycker clin pulm med 2005) Clinical Practice Research database 500/100000 (Quint ERJ 2015)
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•Morbidity
321 clinic attendances from 100pts in 6/12 (Kelly et al E J Int
Med 2003)
greater inpatient stay and annual cost/pt than other chronic diseases (CCF, DM) (Weycker clin pulm med 2005)
Morbidity and mortality
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•Mortality
UK 12 yr survival 68.3% (Loebinger et al ERJ 2009)
UK 4yr survival 89.8% (Chalmers et al ARJCCM 2014)
Spain 5 yr survival 81.2% (Martinez-Garcia et al ERJ 2014)
Turkey 4 yr survival 58% (Onen et al Respir med 2007)
•Increasing mortality (Roberts et al Respir Med 2010)
Morbidity and mortality
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Pathophysiology
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P o s t- in fe c tiv e
Id io p a th ic
C O P D
A sth m a
Im m u n o d e fic ie n c y
A B P A
R h e u m a to id a rth r it is
P C D
G O R D
IB D
A lp h a -1 -a n t it ry p s in d e f ic ie n c y
o th e rs
Aetiology
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Exacerbation definition
A person with bronchiectasis with a deterioration in three or more of the
following key symptoms for at least 48 hours:
1) Cough
2) Sputum volume and / or consistency
3) Sputum purulence
4) Breathlessness and / or exercise tolerance
5) Fatigue and / or malaise
6) Haemoptysis
AND a clinician determines a change in bronchiectasis treatment is
required*
Pulmonary Exacerbation in Adults with Bronchiectasis: A Consensus
Definition from the First World Bronchiectasis Conference
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14 days of antibiotics (conditional recommendation, very low quality
of evidence).
Microbiology and Treatment
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• Treat underlying cause
• Physiotherapy
• Mucolytics/ HTS
Longer term management
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• Treat underlying cause
• Physiotherapy
• Mucolytics/ HTS– Mannitol Ph3 (Bilton 2014 Thorax)
– HTS small studies varied results
(Kellett 2005 – 1 dose, 2011-3/12; Nicholson – 12/12 2012)
Management – airway clearance
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• Treat underlying cause
• Physiotherapy
• Mucolytics/ HTS
• Antibiotics
– Long term
– Nebulised
– Oral
– Cyclical IVs
Management – long term antibiotics
Bacterial load (CFU/ml)
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141 patients 08-09
≥ 1 exacerbation
500mg MWF 6/12 then 6/12 no treatment
83 patients 08-10
≥ 3 exacerbation
250mg od 12/12, 90/7 run out
117 patients 08-11
≥ 2 exacerbation
400mg bd erythromycin 11/12, 1/12 wash
out
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• Colistin - ↓ exacerb in PP (Haworth et al ARJCCM 2014)
• AZLI – no change in QoLB(Barker et al Lancet Resp Med 2014)
• Gentamicin - ↓bacterial, exacerbations,↑QoL(Murray et al 2011 AJRCCM)
Management – long term inhaled
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Oral CSx• No evidence
Inhaled CSx • 6RCTs Cochrane • Some ↓ sputum and i0 markers• No good evidence
Statins• ↓ LCQ
NSAIDs• Inhaled indomethacin 25pt • Some ↓ sputum and SOB• No good evidence
Development• CXCR2 antags / N0 elastase inhibs / PDE4 inhibs
Management – alternative anti-inflammatories
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• Well as child
• Cough at sputum age 14
• Referred to local hospital at 17 – CT
• LLL and lingula lobectomy
Case 1 RL 20 female
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• Well but relapse few months later
• 2/3 pot green sputm
• 4-5 infection/yr
• 2011 repeat CT scan
• Referred to RBH
• IgG <2, A<0.1, M<0.3g/L
• Normal B and T subsets almost absent memory B cells
• Diagnosed with CVID
• Started azithromycin
• IVIG (when trough 7.2 azithro discontinued)
• Case 1 underlying diagnosis
Case 1 RL 20 female
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• Asthma as child
• Cough and sputum late 40s
• Bronchiectasis diagnosed 2009
• Idiopathic
• Pseudomonas
• Relatively stable 1-2 infection/yr
Case 2 VR 63 female
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• Deterioration last couple of years
• More sputum
• More SOB
• More infections
• Limited effect of antibiotics
• Treated with steroids
• Case 2 additional diagnosis
Case 2 VR 63 female
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• Well as child, young adult
• 8 yr history of productive cough
• 6 infections/yr
• Widespread bronchiectasis
• Host defence screen unremarkable
• Some reflux symptoms
• PPI
• Physio review,
Acapella, HTS, positive pressure
• Significant improvement
• 2 infections/yr
• Case 3 - optimisation
Case 3 EM 78 female
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• Primary Ciliary Dyskinesia
• Deterioration age 40
• Multiple infections - Pseudomonas
• PSA eradication unsuccessful
• Colomycin nebulised
• Some stabilisation but increased infections
• Increased physiotherapy
• Addition of azithromycin
Case 4 JW 53 female
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• More recently repeated need for antibiotics
• Needing several admissions for IV therapy per year
• Anxiety and Depression
• All management optimised
• Cyclical intravenous antibiotics
• Case 4 additional therapies
Case 4 JW 53 female
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Adapted from Loebinger et al 2007
Management - practical