OXFORD MEDICAL PUBLICATIONS Oxford Desk Reference: Respiratory Medicine Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product informa- tion and clinical procedures with the most up-to-date pub- lished product information and data shee ts provided by the manufacturers and the most recent codes of conduct and safety regulation s. The authors and the publishers do not accept responsibility or legal liability for any e rrors in the text or for the misuse or misapplication of material in this work. 2 Except where otherwise stated, drug doses and recom- mendations are for the non-pregnant adult who is not breast-feeding. Oxford Desk Reference Respiratory Medicine Nick Maskell Senior Lecturer and Consultant Physician in Respiratory Medicine North Bristol Lung Center University of Bristol and Ann Millar Professor of Respiratory Medicine North Bristol Lung Center University of Bristo l 1 1 Great Clarendon Street, Oxford OX2 6DP Oxford University Press is a department of the University of Oxford. It furthers the Univer sity \200\231s objective of excellence in research, scholarship, and education by publishin g worldwide in Oxford New York Auckland Cape Town Dar es Salaam Hong Kong Karachi Kuala Lumpur Madrid Melbourne Mexico Cit y Nairobi New Delhi Shanghai Taipei Toronto With of \201 ces in Argentina Austria Brazil Chile Czech Republic France Greece Guatemala Hungary Italy Japan P oland Portugal Singapore South Korea Switzerland Thailand Turkey Ukraine Vietnam Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries Published in the United States by Oxford University Press Inc., New York Oxford University Press 2009 The moral rights of the author have been asserted Database right Oxford University Press (m aker) First published 2009 All rights reserved. No part of this publication may be reproduced, stored in a retrieval s ystem, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, or under terms agreed with t he appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, a t the address above You must not circulate this book in any other binding or cover and you must impose this sam e condition on any acquirer British Library Cataloging in Publication Data Data available Library of Congress Cataloging in Publication Data Data available Typeset by Cepha Imaging Private Ltd., Bangalore, India Printed in Great Britain on acid fr ee paper by CPI Antony Rowe, Chippenham, Wiltshire ISBN 978 \200\2230 \200\22319 \200\223923912 \200\2232 10 9 8 7 6 5 4 3 2 1 v Preface This book aims to act as a rapid reference for busy health professionals and covers the mai n respiratory disorders that would be encountered both in the inpatient and outpatient sett ing. Each section has been written by an expert in a particular \201 eld and is focused o n providing a clear, concise clinical message on how best to investigate the relevant condi tion. In order to make the book as user-friendly as possible we have included a lot of imag es and illustrations to make the information more accessible. We believe it is one of the o nly books in the \201 eld where chest radiology lies alongside clinical information. Each chapter also includes authors \200\231 tips and key messages and is laid out in a format w hich makes the information easy to \201 nd and digest. The book includes many common-sens e approaches and has a guide for further reading in each area. It should be possible to use as a \200\230 \201 rst-line \200\231 reference book either to jog your memory or to read about a condition with which you are not familiar. It is likely that you will also need to consult other texts and data sources. However, this is a very portable book which can be c arried around with you in your bag or left on the ward for quick and easy reference. We hop e that you will enjoy this new approach. vi Acknowledgements In editing this book we are indebted to colleagues and friends who have kindly given up the ir time and expertise to write each of the separate sections of the book. We acknowledge th at many of them are national and international experts in their \201 eld and we know that this has helped to enhance the quality and clarity of the book, Our special thanks to our families for tolerating our endeavor with this book. vii Brief contents Detailed contents ix Abbreviations xi Contributors xv 1 The healthy lung 1 2 Respiratory physiology 9 3 Clinical presentations 35 4 Asthma 59 5 Chronic obstructive pulmonary disease 89 6 Oxygen 123 7 Diffuse parenchymal lung disease 127 8 Infection 165 9 The immunocompromised host 201 10 Bronchiectasis 223 11 Cystic \201 brosis 231 12 Pulmonary vascular problems/issues 259 13 Lung cancer 281 14 Pleural disease 321 15 Sleep 359 BRIEF CONTENTS viii 16 Occupation and environment 383 17 Lung transplantation/ITU 417 18 Orphan lung diseases/BOLD 441 Index 461 ix Detailed contents Abbreviations xi Contributors xv 5.6 Management of an exacerbation of COPD 112 5.7 Surgery for COPD 116 5.8 Alpha-1-antitrypsin de \201 ciency 120 1 The healthy lung 1 1.1 Pulmonary anatomy 2 1.2 Radiology of the healthy chest 4 6 Oxygen 123 6.1 Home oxygen therapy 124 2 Respiratory physiology 9 7 Diffuse parenchymal lung 2.1 Basic physiology 10 2.2 Lung function tests: a guide to disease 127 7.1 7.2 7.3 Usual interstitial pneumonia 128 Non-speci \201 c interstitial pneumonia 132 Respiratory b ronchiolitis-associated interstitial lung disease 136 interpretation 18 2.3 Exercise testing 24 2.4 Interpretation of arterial blood gasses and acid/base balance 28 7.4 Desquamative interstitial pneumonia 138 7.5 Acute interstitial pneumonia 140 7.6 Lympho id interstitial pneumonia 142 7.7 Cryptogenic organizing pneumonia 144 7.8 Extrinsic allerg ic alveolitis 148 7.9 Sarcoidosis 152 7.10 Pulmonary manifestations of connective 2.5 Respiratory muscle function 32 3 Clinical presentations 35 3.1 Chronic cough 36 3.2 Breathlessness 38 3.3 Haemoptysis 40 3.4 Chest pain 42 3.5 Pre-ope rative assessment 44 3.6 Solitary pulmonary nodule 46 3.7 Wheeze 50 3.8 The acute admission with a new unilateral tissue disorders 156 7.11 Pulmonary manifestations of systemic diseases 160 8 Infection 165 pleural effusion 54 8.1 8.2 8.3 Aspiration syndromes 176 8.4 Lung abscess 180 8.5 Nocardia and actinomycosis 18 2 Viral infections of the respiratory 8.6 tract 184 Community-acquired pneumonia 166 Hospital-acquired pneumonia 172 3.9 Unexplained respiratory failure 56 4 Asthma 59 4.1 Pathophysiology 60 4.2 Allergic rhinitis 64 4.3 Pharmacology 68 4.4 Asthma in pregnancy 76 4.5 Acute asthma 78 4.6 Chronic asthma management 82 8.7 Respiratory tuberculosis 188 8.8 Non-respiratory tuberculosis 192 8.9 Opportunist (non-tuberculous) mycobacteria 194 5 Chronic obstructive pulmonary 8.10 Fungal and parasitic lung disease 196 disease 89 5.1 COPD genetics and epidemiology 90 5.2 COPD pathophysiology 92 5.3 Investigat ions in COPD 98 5.4 Pharmacological therapy (including cor 9 The immunocompromised host 201 9.1 Pneumonia in the non-HIV immunocompromised patient 202 Infection in the HIV compromised hos t 210 Non-infectious HIV-related lung disease 218 pulmonale) 100 9.2 5.5 Non-pharmacological management for COPD 106 9.3 DETAILED CONTENTS x 10 Bronchiectasis 223 14.9 Pleural infection 352 14.10 Surgery for pleural diseases 356 10.1 Bronchiectasis (etiology) 224 10.2 Chronic disease management 228 15 Sleep 359 11 Cystic \201 brosis 231 15.1 Obstructive sleep apnoea 360 15.2 Driving 368 15.3 The overlap syndrome 370 15.4 Non-i nvasive ventilatory support in the 11.1 Cystic \201 brosis diagnosis 232 11.2 Managing acute infective exacerbations 236 acute setting 372 11.3 Chronic disease management 242 11.4 Cystic \201 brosis genetics 248 11.5 Extra-pulmo nary manifestations of cystic 15.5 Nocturnal hypoventilation 374 15.6 Cheyne \200\223Stokes respiration associated with l eft ventricular failure 378 15.7 Other causes of sleepiness 380 \201 brosis 252 12 Pulmonary vascular problems/ 16 Occupation and environment 383 16.1 Drugs and toxins 384 16.2 Pneumoconiosis 390 16.3 Di sability assessment 394 16.4 Occupational asthma 398 16.5 The effects of high altitude on t he lung 404 16.6 Diving 408 16.7 Occupational chronic obstructive pulmonary disease 412 issues 259 12.1 Pulmonary embolism 260 12.2 Pulmonary hypertension 264 12.3 Pulmonary vascu litis and hemorrhage 272 13 Lung cancer 281 13.1 Epidemiology of lung cancer 282 13.2 Symptoms and signs (including SVCO) 284 Work-up of patients with a suspected diagnosis of l ung cancer 288 Treatment of non-small cell lung cancer 292 16.8 Asbestos-related diseases (excluding 13.3 mesothelioma) 414 13.4 17 Lung transplantation/ITU 417 17.1 Lung transplantation: considerations for 13.5 Treatment of small cell lung cancer 298 13.6 PET-CT 300 13.7 Lung cancer screening 304 13.8 Carcinoid 306 13.9 Benign lung tumors 310 13.10 Transbronchial \201 ne needle referral and listing 418 17.2 Complications after lung transplantation 422 17.3 The care of lung transplant candidate or recipient 426 17.4 Acute respiratory distress aspiration 316 syndrome 430 13.11 Interventional bronchoscopy 17.5 Severe acute respiratory syndrome 432 17.6 The ventilated patient 434 techniques 318 14 Pleural disease 321 18 Orphan lung diseases/BOLD 441 18.1 Pulmonary alveolar proteinosis 442 18.2 Churg \200 \223Strauss syndrome 446 18.3 Ciliary dyskinesia 448 18.4 Pulmonary Langerhans \200\231 cel l 14.1 Normal physiologic \202 uid volume and cellular contents 322 Assessment and investigation of an undiagnosed pleural effusion (including thoracoscopy) 326 14.3 Pneumothorax 332 14.4 14.2 histiocytosis 450 18.5 Lymphangioleiomyomatosis 452 18.6 Primary tracheal tumors 454 18.7 Pulmonary arteriove nous malformations 456 18.8 Pulmonary amyloidosis 458 Exudative pleural effusions (excluding malignancy) 338 14.5 Transudative pleural effusions 340 14.6 Malignant effusions 342 14.7 Malignant mesothe lioma 346 Pleural effusions in systemic 14.8 disease 350 Index 461 xi Abbreviations 6 \210\235 7 > < A B ARDS ASA AT BAL BALF BAPE BOOP therefore proportional approx greater than less than alpha beta kappa gamma acute respirato ry distress syndrome American Society of Anaesthesiologists antitrypsin bronchoalveolar lav age bronchoalveolar lavage \202 uid benign asbestos pleural effusion bronchiolitis oblite rans organizing pneumonia bronchiolitis obliterans syndrome breathing reserve British Thora cic Society coronary artery bypass graft community-acquired pneumonia congenital bilateral absence of the vas deferens cystic \201 brosis cryprogenic \201 brosing alveolitis cyst ic \201 brosis liver disease cystic \201 brosis-related diabetes mellitus cystic \201 brosis transmembrane conductance regulator chronic heart failure chronic kidney disease ce ntimeter cytomegalovirus or controlled mechanical ventilation central nervous system carbon monoxide carbon dioxide chronic obstructive airways disease cryptogenic organizing pneumon ia chronic obstructive pulmonary disease continuous positive airway pressure cardiopulmonar y exercise testing central pattern generator Chronic Respiratory Questionnaire cardiac resy nchronisation therapy cerebrospinal \202 uid Churg \200\223Strauss syndrome CTD CTEPH connective tissue disorder chronic thromboembolic pulmonary hypertension common variable im munode \201 ciency chorionic villus sampling constant work rate chest X-ray diffuse alveol ar damage dynamic hyperin \202 ation desquamative interstitial pneumonia diffusing capacit y for carbon monoxide dorsal respiratory group extrinsic allergic alveolitis eosinophilic b ronchitis endobronchial ultrasound electrocardiogram end-expiratory lung volume electromyog raphy expiratory positive airway pressure equal pressure point or extrapleural pneumonectom y expiratory reserve volume erythrocyte sedimentation rate endurance shuttle walk test endo trachael tube full blood count forced mid-expiratory \202 ow rate forced expiratory volum e in 1 second \201 ne needle aspiration \201 bre-optic bronchoscopy functional residual capacity forced vital capacity gram Glasgow coma scale gastrointestinal granulocyte monocy te colony stimulating factor gastro-oesophageal re \202 ux gastro-oesophageal re \202 ux disease glucocorticoid receptor graft versus host disease highly active antiretroviral ther apy hospital-acquired pneumonia health-care-associated pneumonia house dust mite High Depen dency Unit human immunode \201 ciency virus human leucocyte antigen CVID CVS CWR CXR DAD DH DIP DLco DRG EAA EB EBUS ECG EELV EMG EPAP EPP BOS BR BTS CABG CAP CBAVD ERV ESR ESWT ETT FBC FEF25 \200\22375 FEV1 FNA FOB FRC FVC g GCS GI GMCSF CF CFA CFLD CFRD CFTR CHF CKD cm CMV CNS CO CO2 COAD COP COPD CPAP CPET CPG CRQ CRT CSF CSS GOR GORD GR GVHD HAART HAP HCAP HDM HDU HIV HLA ABBREVIATIONS PRELIM RUNNING HEAD xii HME HPV HRCT HSCT IC ICU Ig IIP ILD IPAP IPF IRIS heat and moisture exchanger hypoxic pulmonary vasoconstriction High-resolution computed tom ography haematopoietic stem cell transplantation inspiratory capacity Intensive Care Unit i mmunoglobulin idiopathic interstitial pneumonia interstitial lung disease inspiratory posit ive airway pressure idiopathic pulmonary \201 brosis immune reconstitution in \202 ammat ory syndrome immunosuppressive trypsin inspiratory reserve volume incremental shuttle walk test Intensive Therapy Unit intravenous Kilopascal Kaposi sarcoma long-acting beta agonist lymphangioleiomyomatosis Langerhans \200\231 cells Langerhans \200\231 cell histiocytosis l actate dehydrogenase lymphoid interstitial pneumonia low-molecular-weight heparin latent tu berculosis infection long-term oxygen therapy leukotriene receptor antagonist left ventricu lar failure lung volume reduction surgery meter mucosa-associate lymphoid tissue Medical A dmission Unit metered dose inhaler multi-drug resistant maximal expiratory \202 ow maxima l inspiratory \202 ow milliliter motor neuron disease microscopic polyangiitis malignant pleural effusion myeloperoxidase Medical Research Council magnetic resonance imaging multip le sleep latency testing maximum voluntary contraction National Emphysema Treatment Trial n asogastric non-Hodgkin lymphoma non-invasive ventilation nitric oxide NRT NSAID NSIP NYHA O2 OGTT OI OSA OSAH OSAS PA PaCO2 nicotine replacement therapy non-steroidal anti-in \202 ammatory drug non-speci \201 c in terstitial pneumonia New York Heart Association oxygen oral glucose tolerance test opportun istic infections obstructive sleep apnoea obstructive sleep apnoea/hypopnoea obstructive sl eep apnoea syndrome postero-anterior partial pressure of carbon dioxide in arterial blood p artial pressure of oxygen in arterial blood pulmonary arterial hypertension pulmonary alveo lar proteinosis pulmonary arteriovenous malformation primary ciliary dyskinesia Pneumocysti s pneumonia polymerase chain reaction critical collapsing pressure photodynamic therapy pul monary embolism pulmonary endartectomy positive end-expiratory pressure peak expiratory \202 ow positron emisson tomography pulmonary function test phosphoinositide parain \202 u enza protein kinase A pulmonary Langerhans \200\231 cell histiocytosis periodic leg movemen t in sleep primary pulmonary hypertension pontine respiratory group pressure support ventil ation post-transplantation lymphoproliferative disorder rheumatoid arthritis rapidly adapti ng receptor radio-allergo-sorbent test respiratory bronchiolitis-associated interstitial lu ng disease randomized controlled trial rapid eye movement rapid sequence intubation respira tory syncitial virus residual volume second/s arterial oxygen saturation slowly adapting re ceptor severe acute respiratory syndrome short-burst oxygen therapy PaO2 PAH PAP PAVM PCD PCP PCR Pcrit PDT PE PEA PEEP PEF PET PFT PI PIV PKA PLCH PLMS PPH PR G PSV PTLD IRT IRV ISWT ITU IV kPa KS LABA LAM LC LCH LDH LIP LMWH LTBI LTOT LTRA LVF LVRS m MALT MAU MDI MDR MEF MIF ml MND MPA MPE MPO MRC MRI MSLT MVC NETT NG NHL NIV NO RA RAR RAST RBILD RCT REM RSI RSV RV s SaO2 SAR SARS SBOT ABBREVIATIONS xiii SCC SEPCR squamous cell carcinoma European Society of Clinical Respiratory Physiology St George \200 \231s respiratory questionnaire syndrome of Inappropriate antidiuretic hormone systemic lup us erythematosus sniff nasal inspiratory pressure single nucleotide polymorphism solid orga n transplant single-photon emission computed tomography solitary pulmonary nodule systemic sclerosis superior vena cava superior vena cava obstruction tuberculosis transbronchial bio psy transbronchial needle aspiration total lung capacity TMN TNF TOSCA tumor \200\223nodal \200\223metastasis tumor necrosis factor transcutaneous oxygen and carb on dioxide monitoring transthoracic aspiration biopsy tidal volume urea and electrolytes up per airway resistance syndrome usual interstitial pneumonia ventilation/perfusion ventilato r-associated pneumonia video-assisted thoracoscopic surgery vital capacity vocal cord dysfu nction vascular endothelial growth factor ventilator-induced lung injury ventral respirator y group Wegener \200\231s granulomatosis whole-lung lavage SGRQ SIADH TTAB TV U&E UARS UIP V/P VAP VATS VC VCD VEGF VILI VRG WG WLL SLE SNIP SNP SOT SPECT SPN SSc SVC SVCO TB TBB TBNA TLC This page intentionally left blank xv Contributors Dr Anthony Arnold Department of Respiratory Medicine Castle Hill Hospital Hull Dr G Cardillo Department of Surgery Glen \201 eld Hospital Leicester Dr Janice Ash-Miles Department of Radiology University Hospitals Bristol Bristol Dr Jim Catterall Respiratory Department Bristol Royal In \201 rmary Bristol Dr Angela Atalla Department of Respiratory Medicine Derriford Hospital Plymouth Dr Colin Church Scottish Pulmonary Vascular Unit Western In \201 rmary Glasgow Dr Ian Coutts Department of Respiratory Medicine Royal Cornwall Hospitals Cornwall Dr David Baldwin Department of Respiratory Medicine Nottingham University Hospitals Notting ham Dr Phillip Barber North West Lung Center University Hospital of South Manchester Manchester Dr Michael Darby Department of Radiology Southmead Hospital Bristol Professor Peter Barnes Airway Diseases Section National Heart & Lung Center Imperial Colleg e London Dr Chris Davies Department of Respiratory Medicine Royal Berkshire Hospital Reading Dr Helen Davies Oxford Center for Respiratory Medicine Churchill Hospital Oxford Dr Nick Bell Dept of Respiratory Medicine University Hospitals Bristol Bristol Professor Robert Davies Oxford Center for Respiratory Medicine Churchill Hospital Oxford Dr Diane Bilton Adult CF Unit Papworth Hospital Cambridge Professor David Denison Emeritus Professor in Clinical Physiology Hospital Royal Brompton L ondon Dr Stephen Bourke Department of Respiratory Medicine Royal Victoria In \201 rmary Newcastl e-upon-Tyne Dr Paddy Dennison Department of Respiratory Medicine Dorset County Hospital Dorset Professor Sherwood Burge Department of Respiratory Medicine Birmingham Heartlands Hospital Birmingham Dr A Degryse Vanderbilt University Medical Center Nashville Tennessee, USA Professor Peter Calverley School of Clinical Sciences University of Liverpool Liverpool Dr Amber Degryse Fellow in Pulmonary Disease Vanderbilt Medical Center Nashville Dr James Calvert North Bristol Lung Center Southmead Hospital Bristol CONTRIBUTORS PRELIM RUNNING HEAD xvi Dr David Derry Department of Respiratory Medicine Derriford Hospital Plymouth Jane French Nurse Consultant Papworth Hospital Cambridge Dr Lee Dobson Respiratory Department Torbay Hospital Torquay Dr Peter Froeschle Department of Thoracic and Upper GI Surgery Royal Devon and Exeter Hospi tal Exeter Professor Duncan Geddes Royal Brompton Hospital London Dr James Dodd Department of Respiratory Medicine Royal Devon & Exeter NHS Foundation Trust Exeter Dr Fergus Gleeson Department of Radiology The Churchill Hospital Oxford Dr Anne Dunleavy Department of Respiratory Medicine Royal Free Hospital London Dr Mark Glover Hyperbaric Medicine Unit St Richard \200\231s Hospital Chichester Professor Jim Egan Department of Respiratory Medicine Master Misericordiae Hospital Dublin Dr Anna Goodman Wellcome Trust Center for Human Genetics University of Oxford Oxford Dr Ugo Eleowa Cambridge Institute for Medical Research University of Cambridge Cambridge Dr Mark Grover Hyberbanic Medicine Unit St Richard \200\231s Hospital Chichester Dr Rachel Evans Department of Respiratory Medicine University Hospitals of Leicester Leices ter Dr Melissa Hack Chest Clinic Newport Hospital Wales Professor Tim Evans Department of Intensive Care Medicine Royal Brompton Hospital London Dr P. Halder Institute of Lung Heath Glen \201 eld Hospital Leicester Dr Hosnieh Fathi Division of Cardiovascular and Respiratory Studies Castle Hill Hospital Co ttingham Dr Praneb Haldar Institute for Lung Health Glen \201 eld Hospital Leicester Dr Tony Fennerty Department of Respiratory Medicine Harrogate District Foundation Trust Har rogate Dr David Halpin Department of Respiratory Medicine Royal Devon & Exeter NHS Foundation Trus t Exeter Dr Rhian Finn North Bristol Lung Center Southmead Hospital Bristol Dr Kim Harrison Respiratory Unit Morriston Hospital Swansea Dr Andrew Fisher Department of Respiratory Medicine Freeman Hospital Newcastle-upon-Tyne Dr John Harvey North Bristol Lung Center Southmead Hospital Bristol Dr Helen Firth Consultant Clinical Geneticist Addenbrookes Hospital Cambridge Dr Melissa Heightman Department of Thoracic Medicine University College Hospital London CONTRIBUTORS xvii Dr Martin Hetzel Department of Respiratory Medicine University Hospitals Bristol Bristol Dr Adrian Kendrick Consultant Clinical Scientist University Hospitals Bristol Bristol Dr Bernard Higgins Respiratory Medicine Department Freeman Hospital Newcastle-upon-Tyne Professor Keith Kerr Department of Pathology University of Aberdeen Aberdeen Dr Mathew Hind Royal Brompton Hospital London Dr Ayaz Khan North Bristol Lung Center Southmead Hospital Bristol Dr Nik Hirani MRC Center for In \202 ammation Research Queen \200\231s Medical Research In stitute Edinburgh Dr Will Kinnear Department of Respiratory Medicine University Hospital Nottingham Professor Margaret Hodson Department of Cystic Fibrosis Royal Brompton Hospital London Dr Malcolm Kohler Oxford Center for Respiratory Medicine Churchill Hospital Oxford Dr Clare Hooper North Bristol Lung Center Southmead Hospital Bristol Dr Sophie Kravinskas Division of Therapeutics and Molecular Medicine University of Nottingh am Nottingham Dr John Hurst Academic Unit of Respiratory Medicine Royal Free Hospital Medical School Lond on Dr Gabriel Laszlo Department of Respiratory Medicine Bristol Royal In \201 rmary Bristol Professor Richard Hubbard Division of Epidemiology and Public Health University of Nottingh am Nottingham Professor Y C Gary Lee University of Western Australia Sir Charles Gairduer Hospital Perth Dr Phil Hughes Chest Clinic Derriford Hospital Plymouth Professor Richard Light Vanderbilt University Medical Center Nashville Dr Jane Hurst Consultant in Clinical Genetics Oxford Radcliffe Hospitals NHS Trust Oxford Dr Lim Wei Shen Department of Respiratory Medicine Nottingham University Hospitals Nottingh am Dr Nabil Jarad Department of Respiratory Medicine University Hospitals Bristol Bristol Dr Marc Lipman Consultant in Respiratory and HIV Medicine Royal Free Hospital London Dr Simon Johnson Division of Therapeutics and Molecular Medicine University of Nottingham N ottingham Professor David Lomas Cambridge Institute of Medical Research Cambridge University Cambridg e Dr Andrew Jones Adult Cyctic Fibrosis Center University Hospitals NHS Foundation Trust Wyth enshawe Manchester Dr Toby Maher Royal Brompton Hospital London Dr Adam Malin Respiratory Unit Royal United Hospital Bath CONTRIBUTORS PRELIM RUNNING HEAD xviii Dr William Man Royal Brompton Hospital London Dr Mitzi Nisbet Host Defense Unit Royal Brompton Hospital London Dr Nick Maskell North Bristol Lung Center University of Bristol Bristol Professor Marc Noppen International Endoscopy Clinic University Hospital Brussels Dr Matthew Masoli North Bristol Lung Center University of Bristol Bristol Professor Peter Ormerod Department of Respiratory Medicine Royal Blackburn Hospital Blackbu rn Dr Andrew R L Medford Department of Respiratory Medicine Derriford Hospital Plymouth Professor Paulo Palange Department of Clinical Medicine University of Rome Rome Professor Ann Millar North Bristol Lung Center University of Bristol Bristol Dr Timothy Palfreman Adult Intensive Care Unit Royal Brompton Hospital London Professor Rob Miller Center for Sexual Health & HIV Research University College Hospital Lo ndon Dr Timothy Palfreyman Department of Intensive Care Medicine Royal Brompton Hospital London Dr Robert Milroy Department of Respiratory Medicine Stobhill Hospital Glasgow Dr Manish Pareek Infectious Disease Unit Leicester Royal In \201 rmary Leicester Dr John Moore-Gillon Department of Respiratory Medicine Barts and the London NHS Trust Lond on Dr S Parker Department of Respiratory Medicine Freeman Hospital Newcastle-upon-Tyne Professor Alyn Morice Division of Cardiovascular and Respiratory Studies Castle Hill Hospit al Cottingham Dr Bipen Patel Department of Respiratory Medicine Royal Devon & Exeter NHS Foundation Trust Exeter Dr Cliff Morgan Department of Critical Care & Anesthesia Royal Brompton Hospital London Dr Sam Patel Dept of Respiratory Medicine University Hospitals Bristol Bristol Professor Mike Morgan Department of Respiratory Medicine University Hospitals of Leicester Leicester Professor Ian Pavord Institute of Lung Heath Glen \201 eld Hospital Leicester Professor Nick Morrell Division of Respiratory Medicine Department of Medicine University o f Cambridge Professor Andrew Peacock Scottish Pulmonary Vascular Unit Western In \201 rmary Glasgow Dr Suranjan Mukhersee Directorate of Respiratory Medicine University Hospitals of North Sta ffordshire Stoke-on-Trent Dr Mike Peake Dept of Respiratory Medicine University Hospitals of Leicester Leicester Dr John T Murchison Department of Radiology Royal In \201 rmary of Edinburgh Edinburgh Dr Justin Pepperell Department of Respiratory Medicine Taunton and Somerset Hospital Taunto n CONTRIBUTORS xix Dr Gerrard Phillips Department of Respiratory Medicine Dorset County Hospital Dorset Dr Nicholas Simmonds Department of Cystic Fibrosis Royal Brompton Hospital London Dr Martin Plummeridge North Bristol Lung Center Southmead Hospital Bristol Dr Pasupathy Sivasothy Department of Respiratory Medicine Addenbrooke \200\231s Hospital Ca mbridge Professor Jose Porcel Department of Internal Medicine Lleida, Spain Dr David Smith North Bristol Lung Center Southmead Hospital Bristol Dr Susan Poutanen University Health Network & Mount Sinai Hospital Department of Microbiolo gy Toronto, Canada Professor Monica Spiteri Department of Respiratory Medicine University Hospital of North St affordshire Stoke-on-Trent Dr R Ragendram Department of General Medicine John Redcliffe Hospital Oxford Professor Stephen Spiro Department of Thoracic Medicine University College Hospital London Dr Kasper F Remund Department of Respiratory Medicine Mater Misericordiae Hospital Dublin Dr Iain Stephenson Infectious Diseases Unit Leicester Royal In \201 rmary Leicester Dr Gerrit Van Rensburg Respiratory Unit Royal United Hospital Bath Dr John Strading Oxford Center for Respiratory Medicine Churchill Hospital Oxford Professor Douglas Robinson Laboratories Leti, Madrid and Imperial College, London Dr Jay Suntharalingam Respiratory Unit Royal United Hospital Bath Dr Grace Robinson Department of Respiratory Medicine Royal Berkshire Hospital Reading Dr Richard Teoh Department of Respiratory Medicine Castle Hill Hospital Hull Dr Francisco Rodriguez-Panadero MD El Mirador Tomares (Sevilla) Dr Matthew Thornber Department of Acute Medicine North Bristol NHS Trust Bristol Dr Robin Rudd Consultant Physician London Dr Joseph Unsworth Department of Immunology Southmead Hospital Bristol Dr Pallav Shah Royal Brompton Hospital London Mr David Waller Department of Surgery Glen \201 eld Hospital Leicester Dr Clare Shovlin National Heart & Lung Institute Imperial College London Dr Neil Ward Department of Respiratory Medicine Royal Devon and Exeter Hospital Exeter Dr Anita Simonds Sleep and Ventilation Unit Royal Brompton & Hare \201 eld NHS Trust Londo n CONTRIBUTORS xx Professor Kevin Webb Adult Cystic Fibrosis Center University Hospitals NHS Foundation Unit Manchester Dr R Wilson Host Defense Unit Royal Brompton Hospital London Professor Jadwiga Wedzicha Academic Unit of Respiratory Medicine Royal Free Hospital Medica l School London Dr Robert Winter Addenbrooke \200\231s Hospital Cambridge University Hospitals NHS Foundati on Cambridge Professor Athol Wells Royal Brompton & Hare \201 eld NHS London Dr Nick Withers Department of Respiratory Medicine Royal Devon and Exeter Hospital Exeter Dr Adam Whittle Department of Respiratory Medicine University Hospitals Bristol Bristol 1 Chapter 1 The healthy lung Chapter contents 1.1 Pulmonary anatomy 2 1.2 Radiology of the healthy chest 4 M THORNBER 2 1.1 Pulmonary anatomy Lobes and \201 ssures Each lung is divided into lobes by the presence of \201 ssures; t he left lung by the oblique \201 ssure into an upper and lower lobe, whilst the right is split into an upper, middle, and lower lobe by the oblique and transverse fissures (Fig. 1. 1.1). Accessory \201 ssures can occur, of which the one formed if the azygos vein arches laterally to the mediasti- num instead of medially, giving rise to the \200\230azygos lobe \200\231 is the most common (up to 1%). Pulmonary veins Pulmonary veins Bronchiole Bronchiole Visceral pleura Visceral pleura Interlobular septum Interlobular septum Pulmonary artery Pulmonary artery AUTHOR \200\231S TIPS \200 The visceral pleura is continued on to the major \201 ssures, its visibility as a horizon tal hairline is a normal \201 nding in almost half of all chest X-rays. The horizontal \201 ssure is often incomplete medially, allowing collateral ventilation between lobes. Lymphatics Lymphatics Fig. 1.1.2 Secondary lobule. Nerve supply to the lung Sympathetic supply is from thoracic segments 3 to 5 via the sympat hetic chain which supplies the bronchial airway and pulmonary artery muscle. Parasympatheti c supply is from the vagus nerve which con- stricts bronchial muscle and has secretomotor a ction to the mucous glands. Sensory supply is stretch sensation to the lung and visceral pl eura and pain to the parietal pleura. The diaphragmatic portion is via the phrenic nerve wh ilst the costal portion is from intercostals nerves. Airways The trachea bifurcates into the right and left main bronchi at the level of the man ubrio-sternal joint. The right is typi- cally wider, shorter (3cm) and less steeply angled than the longer (5cm) left. The main bronchi divide into lobar and segmental branches which continue until they reach 1mm in diameter, when they lose their cartilage and become bronc hioles. Both lungs have 10 wedge-shaped bronchopulmonary segments, each with its own air an d blood supply. Blood supply The lung receives both a pulmonary and a bronchial artery supply. The pulmonar y arterial circulation follows the branching of the bronchi, the bronchial arterial circula tion supplies the airways, visceral pleura and lymphoid tissue. Larynx Trachea Lymphatic drainage There are no lymphatic vessels in the alveoli. The lymphatic vessels fro m the alveolar duct and bronchioles follow the bronchial tree back to the hilum and then th e mediastinum. Lymph nodes may occur along their intrapulmonary course. Beneath the viscera l pleura a plexus of lymphatics are present, they drain into the peribronchial lymphatics, through vessels that run in septae through the acini and segments. It is distension of thes e horizontally placed septae which causes Kerley B lines. Left upper lobe Right upper lobe Horizontal fissure Oblique fissure Oblique fissure Middle lobe Left lower lobe Right lower lobe Fig. 1.1.1 Lobes and \201 ssures. AUTHOR \200\231S TIP Since there is communication between the pulmonary and bronchial circu lation in the parenchyma, the bronchial arteries may contribute to gas exchange in pulmonar y vascular disorders. Parenchyma Terminal bronchioles (0.5mm diameter) are the last airway before the alveolar li ned respiratory bronchioles start. There are 20,000 \200\22330,000 terminal bronchioles, ea ch ending in an acinus (primary bronchiole). Respiratory bronchioles within an acinus will branch several times until they reach the further divided alveolar ducts which lead to the alveo- lar sacs and their alveoli. The secondary lobule is the smallest section of lung whi ch can be seen on high resolution computed tomography (HRCT); it contains 5 or 6 acini, who se interlobular septum consists of pulmonary lymphatics, veins and a discrete layer of conn ective tissue (Fig. 1.1.2). CHAPTER 1.1 Pulmonary anatomy 3 Pulmonary veins Trachea Right main bronchus Left main bronchus 1 2 1 3 2 4 Carina 3 4 5 5 10 6 6 9 8 7 10 7 8 9 RIGHT LEFT 1 Apical (RUL) 1 Apical (LUL) 2 Posterior (RUL) 2 Posterior (LUL) 3 Anterior (RUL) 3 Anterior (LUL) 4 Superior Lingular 4 Lateral (RUL) 5 Inferior Lingular 5 Medial (RUL) 6 Lateral Basal 6 Lateral Basal 7 Posterior Basal 7 Posterior Basal 8 Anterior Basal 8 Anterior Basal 9 Medial Basal 9 Medial Basal 10 Apical Lower 10 Apical Lower Fig. 1.1.3 Diagram of the bronchial tree with typical segmental bronchi. Nomenclature is th at used most commonly in the UK. J ASH-MILES & M DARBY 4 1.2 Radiology of the healthy chest The plain chest X-ray (CXR) Technical factors PA (postero-anterior) Full inspiration (mid-d iaphragm crossed by 5th \200\2237th ante- rior ribs) necessary to assess heart size and med iastinal contours. Less = reduced lung volume, obesity or poor patient cooperation. More = asthma, emphysema/chronic obstructive airways disease (COAD) or \201 t healthy young adul t. Heart size <50% of max. internal chest diameter. \200 Aortic knuckle Left pulmonary artery Left hilar point Right hilar point Horizontal fissure Emphysema/COAD, \200\230normal \200\231 heart size may be signi \201 - cantly less, due t o over expansion of rib cage \200\223 changes from previous may be more useful. In elderly /osteoporosis, chest diameter may be relatively less, and so \200\230normal \200\231 heart size could be up to 2/3rds chest diameter. Rotation \200\223 spinous processes over mid t rachea; clavicles and ribs symmetrical. If not, can cause apparent lucency/ increased densi ty of one lung. \200\230Hidden lung \200\231 Beware the \200\230hidden \200\231 zones \200\223 nearly 50% of lung area may be partiall y obscured on PA view by mediastinum and dia- phragm (anterior and posterior costophrenic r ecesses). These areas are even less well seen on portable \201 lms. Aortic knuckle Sternum AP (antero-posterior) supine Magni \201 cation of mediastinum makes sizes inaccurate, but gives useful information on gross lung pathologies and position of lines, drains and tubes. Left main pulmonary artery Ribs Irregular lower border smooth upper border Lateral Allows visualization of \200\230hidden \200\231 areas and localizes to a lobe a le sion seen on PA view. Carina Normal appearances (Fig 1.2.1) Mediastinum Left heart border made up of 4 \200\230moguls \200\231 = aortic knuckle (indents trachea), pulmonary artery, left atrial appendage and le ft ventricle. Right heart border made up from ascending aorta and right atrium. Hilar point s formed by the crossing of upper and lower zone broncho-vascular bundles. Left lies 1 \200 \2231.5cm higher than right. Bronchus intermedius Right and left main bronchi Fig. 1.2.1 Normal PA CXR. Assessing the CXR Systematic approach Mediastinum, lungs, bones and soft tissues. Lung parenchyma Branching pattern of bronchovascular bundles which taper towards periphery. Arteries accompany airways, but latter not discernable except above each hilum when seen e nd-on as rings. Absence of discernable structures in outer 1/3 of lungs. Interstitium only visible when pathological. Fissures may undulate and frequently incomplete (NB cause of col lateral air drift between lobes). Horizontal \201 ssure joins right hilum. Obliques pass from few centimeters behind anterior chest wall to 6th thoracic vertebra. Mediastinum Aorto-pulmonary window is concave (if not, is there nodal disease?). Additional , \200\230double \200\231 contours to heart border signify abnormal pathology. Shift of Hi lar points indicates either traction due to col- lapse/ \201 brosis or pressure from space -occupying lesions. Left heart border obscuration indicates lingular disease. Right heart b order obscuration indicates middle lobe disease. Superior mediastinal borders may widen in elderly due to ectasia of vessels, or by obesity. Diaphragms Right up to 2cm higher than left. If not \200\230dome \200\231 shape, suggests hyperin \202 ation. Localized bulge \200\223 \200\230eventration \200\231 due to muscle de \201 ciency, usually antero-medial portion. CHAPTER 1.2 Radiology of the healthy chest 5 Beware \200 Lungs Overall picture \200\223 lung volumes, symmetry of density and size. Variations with in a lung. Refer to \200\230zones \200\231, not lobes unless obvious or have a lateral \201 lm. \200\230Hidden \200\231 areas \200\223 behind heart, lung apices (partially obscured by o verlying bones), through diaphragm in anterior and posterior costophrenic recesses. Bones \200\223 lower borders of posterior ribs often indistinct, upper borders smooth and clear m argins. Fractures, metastases. Soft tissues \200\223 beware extra thoracic soft tissue lin es mimicking pathology (e.g. pneumothorax). Mastectomy causes unilateral lucency of a lung. Lateral CXR (Fig. 1.2.2) Retrosternal and retrocardiac areas should be more \200\230black \200\231. Gradual transition from whiter to blacker lung over spine as pass cranio-caudally . R Computed tomography (CT) Techniques Helical = spiral = multidetector CT Constant acquisitio n of images as patient passes through scanner. Modern scanners can acquire 64 (or more) ima ges per rotation of X-ray tube. A volume of information is acquired which can be manipulate d to give reformations in sagittal, coronal or oblique planes. IV contrast allows improved visualization of vascular structures. Protocol varies depending on clinical question: \200 Horizontal fissure Retrosternal space Oblique fissures Retro- cardiac space PE scans need high volume at high \202 ow rates, to prefer- entially visualize pulmonary arteries. Staging scans may be in 2 phases; \201 rst to show mediasti- num and second del ayed to show liver in portal venous phase. Pleural disease is better shown at delayed phase to improve soft tissue enhancement. Right Diaphragm Left Posterior Costophrenic spaces Fig. 1.2.2 Normal lateral CXR. AUTHOR \200\231S TIP Clinical information vital to ensure correct protocol followed.