bronchiectasis exacerbations; differences and management
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Bronchiectasis exacerbations; differences and management
Michael LoebingerRoyal BromptonImperial College
Plan
Bronchiectasis background and burden
Cases and practical management
Exacerbation and Management
Longer term Management
Bronchiectasis
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
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)
•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
•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
Pathophysiology
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
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
14 days of antibiotics (conditional recommendation, very low quality
of evidence).
Microbiology and Treatment
• Treat underlying cause
• Physiotherapy
• Mucolytics/ HTS
Longer term management
• 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
• Treat underlying cause
• Physiotherapy
• Mucolytics/ HTS
• Antibiotics
– Long term
– Nebulised
– Oral
– Cyclical IVs
Management – long term antibiotics
Bacterial load (CFU/ml)
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
• 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
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
• Well as child
• Cough at sputum age 14
• Referred to local hospital at 17 – CT
• LLL and lingula lobectomy
Case 1 RL 20 female
• 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
• Asthma as child
• Cough and sputum late 40s
• Bronchiectasis diagnosed 2009
• Idiopathic
• Pseudomonas
• Relatively stable 1-2 infection/yr
Case 2 VR 63 female
• 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
• 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
• 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
• 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
Adapted from Loebinger et al 2007
Management - practical
Summary
• Assessment
• Optimisation
• Further therapies
• M.loebinger@rbht.nhs.uk
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