January 2019 Volume 74 Supplement 1 AnThorax international journal of RESPIRATORY MEDICINE BRITISH THORACIC SOCIETY GUIDELINE FOR BRONCHIECTASIS IN ADULTS British Thoracic Society Bronchiectasis in Adults Guideline Development Group thorax.bmj.com BTS Bronchiectasis in Adults Guideline Development Group Professor Adam T Hill, Dr Anita L Sullivan, Professor James D Chalmers, Professor Anthony De Soyza, Professor J Stuart Elborn, Professor R Andres Floto, Ms Lizzie Grillo, Dr Kevin Gruffydd-Jones, Ms Alex Harvey, Dr Charles S Haworth, Mr Edwin Hiscocks, Dr John R Hurst, Dr Christopher Johnson, Dr W Peter Kelleher, Dr Pallavi Bedi, Miss Karen Payne, Mr Hesham Saleh, Dr Nicholas J Screaton, Dr Maeve Smith, Professor Michael Tunney, Dr Deborah Whitters, Professor Robert Wilson, Professor Michael R Loebinger On behalf of the British Thoracic Society The BTS Guideline for Bronchiectasis in Adults has been endorsed by: Association for Chartered Physiotherapists in Respiratory Care Association of Respiratory Nurse Specialists Primary Care Respiratory Society UK Royal College of Physicians London Healthcare providers need to use clinical judgement, knowledge and expertise when deciding whether it is appropriate to apply recommendations for the management of patients. The recommendations cited here are a guide and may not be appropriate for use in all situations. The guidance provided does not override the responsibility of healthcare professionals to make decisions appropriate to the circumstances of each patient, in consultation with the patient and/or their guardian or carer. 74 January 2019 Volume 74 Supplement 1 S1 Volume 74 Supplement 1 Volume Contents Volume 74 Supplement 1 | THORAX January 2019 Pages 1–69 Pages ThoraxAn international journal of RESPIRATORY MEDICINE BRITISH THORACIC SOCIETY GUIDELINE FOR BRONCHIECTASIS 1 Summary of recommendations and good 15 Tests for specific disease groups with associated THORAX IN ADULTS British Thoracic Society practice points bronchiectasis Bronchiectasis in Adults Guideline Development Group 15 Allergic bronchopulmonary aspergillosis January 2019 January Section 1 16 Immune dysfunction 10 Introduction 16 Serum immunoglobulins (IgG, IgA, & IgM) and thorax.bmj.com 10 Target audience for the guideline electrophoresis 10 Scope of the guideline Journal of the British Thoracic Society 16 Full blood count (FBC) 10 Areas not covered by the guideline 16 Measurement of baseline specific antibody Impact Factor: 9.655 10 Definition Editors-in-Chief levels against capsular polysaccharides of N Hart (UK) Streptococcus pneumonia G Jenkins (UK) Section 2 Rheumatoid Arthritis AR Smyth (UK) 10 Methodology 17 Deputy Editors Recurrent aspiration 10 Clinical Questions and Literature Search 17 N Kaminski (USA) 17 Cystic fibrosis M Rosenfeld (USA) 10 Appraisal of Literature Ciliary dysfunction Associate Editors 11 Considered Judgement and Grading of Evidence 17 D Baldwin (UK) J-L Pepin (France) 18 Young’s syndrome R Chambers (UK) M Polkey (UK) 11 Drafting the Guideline T Coleman (UK) J Quint (UK) 18 Alpha-1 antitrypsin deficiency GJ Criner (USA) N Rahman (UK) 11 Guideline Group Members and Declarations of J Davies (UK) K Robinson (USA) Interest 18 Congenital Causes A Floto (UK) S Shaheen (UK) 18 Investigations of lung microbiology M Griffiths (UK) R Stevens (USA) 11 Stakeholders L Heaney (UK) A Vonk Noordegraaf N Hopkinson (UK) (The Netherlands) Section 7 S Janes (UK) C Wainwright (Australia) Section 3 B Kampman (UK) T Wilkinson (UK) 19 Prognosis: What is the outlook for these patients? B Mohkelesi (USA) P Wolters (USA) 11 How common is bronchiectasis? Section 8 Statistical Editors Section 4 A Douiri (UK) 19 Severity scoring C Flach (UK) 12 How should the diagnosis of bronchiectasis C Jackson (UK) be determined? S Stanojevic (USA) Section 9 R Szczesniak (USA) 12 Imaging 20 Stable state treatment B Wagner (USA) 20 Airway clearance techniques Y Wang (UK) Section 5 Which patients should be taught airway Journal Club Editor 12 In whom should the diagnosis of bronchiectasis 20 P Murphy (UK) be suspected? clearance techniques? President, British Thoracic Society 21 Adherence Dr M Elliott 13 COPD Which airway clearance techniques should be Editorial Office 13 Alpha-1 antitrypsin deficiency (A1AT) 21 Thorax, BMA House, Tavistock Square, taught? London WC1H 9JR, UK 13 Asthma How often should patients carry out airway T: +44 (0)20 7383 6373 13 Rhinosinusitis 24 E: [email protected] clearance techniques? How long should an airway 13 Chronic systemic infection Twitter: @ThoraxBMJ clearance session last? 13 Rheumatoid arthritis ISSN: 0040-6376 (print) 24 How soon should the patient be reviewed after ISSN: 1468-3296 (online) 13 Other connective tissue diseases the initial assessment? Disclaimer: Thorax is owned and published by the 13 Inflammatory bowel disease British Thoracic Society and BMJ Publishing Group 24 Mucoactives in bronchiectasis Ltd, a wholly owned subsidiary of the British Medical Association. The owners grant editorial freedom to 25 What is the evidence for long term anti-inflammatory the Editor of Thorax. Section 6 Thorax follows guidelines on editorial independence 14 Investigations for causes of bronchiectasis therapies in bronchiectasis? produced by the World Association of Medical Editors and the code on good publication practice 14 Standard laboratory tests of the Committee on Publication Ethics. MORE CONTENTS Thorax is intended for medical professionals and is provided without warranty, express or implied. Statements in the Journal are there sponsibility of their authors and advertisers and not authors’ institutions, the BMJ Publishing Group Ltd, the British Thoracic Society or theBMAunless otherwise This article has been chosen by the Editors to be of special interest specified or determined by law. Acceptance of or importance and is freely available online. advertising does not imply endorsement. To the fullest extent permitted by law, the BMJ This article has been made freely available online under the BMJ Publishing Group Ltd shall not be liable for any loss, injury or damage resulting from the use of Thorax Journals Open Access scheme. or any information in it whether based on contract, See http://authors.bmj.com/open-access/ tort or otherwise. Readers are advised to verify any information they choose to rely on. Copyright: © 2019 BMJ Publishing Group Ltd and This journal is a member of and subscribes to the principles of the the British Thoracic Society. All rights reserved; no COPE Committee on Publication Ethics part of this publication may be reproduced, stored in a retrieval system or transmitted in any form or Member since 2009 http://publicationethics.org/ by any means, electronic, mechanical, photocopying, JM03979 recording or otherwise without the prior permission of Thorax. Thorax is published by BMJ Publishing Group Ltd, typeset by Exeter Premedia Services Private Ltd, Chennai, India and printed in the UK on acid-free paper. Thorax (ISSN No: 0040–6376) is published monthly by BMJ Publishing Group and distributed in the USA by Air Business Ltd. Periodicals postage paid at Jamaica NY 11431. POSTMASTER: send address changes to Thorax, Air Business Ltd, c/o Worldnet Shipping Inc., 156-15, 146th Avenue, 2nd Floor, Jamaica, NY 11434, USA. Contents Volume 74 Supplement 1 | THORAX January 2019 27 What treatments improve outcomes for patients Section 18 with stable bronchiectasis? 46 Specialist vs non-specialist setting 30 Does long term bronchodilator treatment improve outcomes for patients with bronchiectasis? Section 19 31 Pulmonary Rehabilitation 46 What are the complications of bronchiectasis? Inspiratory Muscle Training 32 Section 20 Field Walking Tests 32 47 Summary of research recommendations 33 What is the role of surgery in managing bronchiectasis? Tables 33 Lung transplantation for bronchiectasis Table 1 Quick Summary Guide 34 What is the role of influenza and pneumococcal Table 2 Levels of evidence vaccination in management of bronchiectasis Table 3 Grades of recommendation 35 Treatment of respiratory failure Table 4 Variables involved in calculating the severity 36 Bronchiectasis and other treatments score in the Bronchiectasis Severity Index Table 5 Variables involved in calculating severity in Section 10 the FACED score Do pathogens have an impact on prognosis in 36 Table 6 Common organisms associated with bronchiectasis? acute exacerbation of bronchiectasis and Section 11 suggested antimicrobial agents- adults 37 What is the evidence for the role of viruses/ Table 7 Routine monitoring tests fungal disease in patients with bronchiectasis? Figures Section 12 4 Figure 1 Management of the deteriorating patient 37 Does eradication of potentially pathogenic 5 Figure 2 Stepwise management microorganisms improve outcomes in patients 26 Figure 3 Physiotherapy management-stepwise with stable bronchiectasis? airway clearance 26 Figure 4 Airway clearance – exacerbation Section 13 43 Figure 5 Algorithm for initial assessment and 38 Exacerbations treatment – rhinosinusitis 38 Does antibiotic therapy improve outcomes in patients with an exacerbation of bronchiectasis? Appendices 55 Appendix 1: Guideline group members Section 14 55 Appendix 2: Protocol for test dose of a nebulised 40 Specific treatments antibiotic 40 What treatments improve outcomes in patients 55 Appendix 3: Long term antibiotic regimes with bronchiectasis and ABPA? 56 Appendix 4: Specialist immunology investigations 41 Does immunoglobulin replacement treatment 59 Appendix 5: Summary table of papers for improve outcomes in