Bronchiectasis exacerbations; differences and management. Michael Loebinger Royal Brompton Imperial College

Transcription

1 Bronchiectasis exacerbations; differences and management Michael Loebinger Royal Brompton Imperial College

2 Plan Bronchiectasis background and burden Exacerbation and Management Longer term Management Cases and practical management

3 Bronchiectasis

4 What is the prevalence of bronchiectasis in the UK? (x600 for number) 1) 1/ ) 10/ ) 100/ ) 500/ ) 1000/ ) nobody knows 7) I don t know

5 Prevalence What is the prevalence of bronchiectasis in the UK? 1 1/ / / / / nobody knows 7 I don t know 52/ adults in US (Weycker clin pulm med 2005) Clinical Practice Research database 500/ (Quint ERJ 2015)

6 Morbidity and mortality 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)

7 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)

8 Pathophysiology

9 Aetiology P o s t-in fe c tiv e Id io p a th ic C O P D A s th 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 ritis P C D G O R D IB D A lp h a -1 -a n titry p s in d e fic ie n c y o th e rs

10 Exacerbation definition Pulmonary Exacerbation in Adults with Bronchiectasis: A Consensus Definition from the First World Bronchiectasis Conference 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*

11 Microbiology and Treatment 14 days of antibiotics (conditional recommendation, very low quality of evidence).

12 Longer term management Treat underlying cause Physiotherapy Mucolytics/ HTS

13 Management airway clearance Treat underlying cause Physiotherapy Mucolytics/ HTS Mannitol Ph3 (Bilton 2014 Thorax) HTS small studies varied results (Kellett dose, /12; Nicholson 12/ )

14 Management long term antibiotics Treat underlying cause Physiotherapy Mucolytics/ HTS Antibiotics Long term Nebulised Oral Cyclical IVs Bacterial load (CFU/ml)

15 141 patients exacerbation 500mg MWF 6/12 then 6/12 no treatment 83 patients exacerbation 250mg od 12/12, 90/7 run out 117 patients exacerbation 400mg bd erythromycin 11/12, 1/12 wash out

16 Management long term inhaled 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)

17 Management alternative anti-inflammatories 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

18 Case 1 RL 20 female Well as child Cough at sputum age 14 Referred to local hospital at 17 CT LLL and lingula lobectomy

19 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

20 Case 2 VR 63 female Asthma as child Cough and sputum late 40s Bronchiectasis diagnosed 2009 Idiopathic Pseudomonas Relatively stable 1-2 infection/yr

21 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

22 Case 3 EM 78 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

23 Case 4 JW 53 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

24 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

25 Management - practical Adapted from Loebinger et al 2007

26 Summary Assessment Optimisation Further therapies