FEV1/VC Caucasian FEMALES courtesy of Vitalograph Prediction Nomogram B T P S FEMALES Age (yrs) FVC (liter) FEV1 (liter) Height (cm) (inches) 20 180 70 69 68 4 67 30 170 66 3 65 64 40 160 63 62 3 61 60 150 50 59 2 58 57 56 2 60 140 70 Index to emergency topics Alveolar hemorrhage 27 Anaphylaxis 641 Asthma 130 Carbon monoxide poisoning 607 COPD exacer bation 177 Cricothyroidotomy 764 Cystic \201 brosis 216 Haemoptysis (massive) 43 Hypercal caemia 304 Inappropriate ADH secretion 306 Paraquat poisoning 600 Pulmonary embolus 412 Sic kle cell acute chest syndrome 565 Spinal cord compression 309 Superior vena cava obstructio n 302 Tension pneumothorax 382 Toxic inhaled substances 602 Upper airways obstruction 641 OXFORD MEDICAL PUBLICATIONS Oxford Handbook of Respiratory Medicine Published and upcoming Oxford Handbooks Oxford Handbook of Clinical Medicine 6/e (also available for PDAs and in a Mini Edition) Ox ford Handbook of Clinical Specialties 7/e Oxford Handbook of Acute Medicine 2/e Oxford Hand book of Anesthesia 2/e Oxford Handbook of Applied Dental Sciences Oxford Handbook of Cardio logy Oxford Handbook of Clinical Dentistry 4/e Oxford Handbook of Clinical and Laboratory I nvestigation 2/e Oxford Handbook of Clinical Diagnosis Oxford Handbook of Clinical Hematolo gy 2/e Oxford Handbook of Clinical Immunology and Allergy 2/e Oxford Handbook of Clinical D rugstore Oxford Handbook of Clinical Surgery 2/e Oxford Handbook of Critical Care 2/e Oxfor d Handbook of Dental Patient Care 2/e Oxford Handbook of Dialysis 2/e Oxford Handbook of Em ergency Medicine 3/e Oxford Handbook of Endocrinology and Diabetes Oxford Handbook of ENT a nd Head and Neck Surgery Oxford Handbook for the Foundation Program Oxford Handbook of Gast roenterology and Hepatology Oxford Handbook of General Practice 2/e Oxford Handbook of Geni tourinary Medicine, HIV and AIDS Oxford Handbook of Geriatric Medicine Oxford Handbook of M edical Sciences Oxford Handbook of Nephrology and Hypertension Oxford Handbook of Neurology Oxford Handbook of Obstetrics and Gynecology Oxford Handbook of Oncology 2/e Oxford Handbo ok of Ophthalmology Oxford Handbook of Palliative Care Oxford Handbook of Practical Drug Th erapy Oxford Handbook of Psychiatry Oxford Handbook of Public Health Practice 2/e Oxford Ha ndbook of Rehabilitation Medicine Oxford Handbook of Respiratory Medicine Oxford Handbook o f Rheumatology 2/e Oxford Handbook of Tropical Medicine 2/e Oxford Handbook of Urology Oxford Handbook of Respiratory Medicine SECOND EDITION Stephen Chapman Clinical Lecturer in Respiratory Medicine, University of Oxford Grace Robinson Consultant in Respiratory Medicine, Royal Berkshire Hospital, Reading John Stradling Professor of Respiratory Medicine, University of Oxford Sophie West Consultant in Respiratory Medicine, Oxford 1 1 Great Clarendon Street, Oxford OX2 6DP Oxford University Press is a department of the University of Oxford. It furthers the Univer sity s objective of excellence in research, scholarship, and education by publishing worldw ide in Oxford New York Athens Auckland Bangkok Bogot Buenos Aires Cape-Town Chennai Dar-e s-Salaam Delhi Florence Hong-Kong Istanbul Karachi Kolkata Kuala-Lumpur Madrid Melbourne Me xico-City Mumbai Nairobi Paris S o-Paulo Shanghai Singapore Taipei Tokyo Toronto Warsaw wi th associated companies in Berlin Ibadan 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 (maker) First edition published 2005 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 Typeset by Cepha Imaging Private Ltd., Bangalore, India Printed in Italy on acid-free paper by LegoPrint S.p.A. ISBN 978-0-19-954516-2 10 9 8 7 6 5 4 3 2 1 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 information and c linical procedures with the most up to date published product information and data sheets p rovided by the manufacturers and the most recent codes of conduct and safety regulations. T he authors and the publishers do not accept responsibility or legal liability for any error s in the text or for the misuse or misapplication of material in this work. 2 Except where otherwise stated, drug doses and recommendations are for the non-pregnant ad ult who is not breast-feeding. v Preface This Handbook of Respiratory Medicine has been written largely by specialist registrars, fo r specialist registrars. Three of the four authors, Stephen Chapman, Grace Robinson, and So phie West, are specialist registrars on the Oxford rotation and John Stradling is Professor of Respiratory Medicine in Oxford. It is in a format that the authors would like to have h ad when they started their specialist registrar training. However, we hope that any health worker or student with an interest in respiratory medicine will \201 nd this text a rapid and useful reference source. The layout of the book tries to ful \201 l the requirement to be able to look up a topic q uickly when the clinical need arises, but also to provide a bit more insight into the more dif \201 cult areas. Therefore the chapters are of necessity different in style and re \202 ect the authors \200\231 own views on how to best approach and understand an area. The handbook is divided into \201 ve sections: clinical presentations and approaches to s ymptoms and problems; the clinical conditions themselves; supportive information; procedure s; and useful appendices (also on the inside covers), containing more technical and referen ce information. We hope you \201 nd it helpful. Feedback on errors and omissions would be much appreciate d. Please post your comments via the OUP website: www.oup.com/uk/medicine/handbooks. Stephen Chapman Grace Robinson John Stradling Sophie West June 2005 The second edition of this book has allowed us to make several improve- ments in response t o readers \200\231 suggestions. We have changed the order of chapters within each section t o alphabetical, and improved the index, to make the contents more rapidly accessible. We ha ve added new content such as more detailed radiology, pandemic in \202 uenza, and pulmonar y com- plications of sickle cell disease. In addition, we have updated and enhanced topics where there have been new guidelines and relevant publications. The overall aim of the book , to provide a rapid and comprehensive resource for all those involved in respiratory medic ine, has remained paramount. October 2008 vi Acknowledgements We would like to offer our grateful thanks to the following friends and colleagues for thei r reviewing and advice on various sections of this book. First edition Dr Nicholas Bates, Dr Lesley Bennett, Dr Rachel Bennett, Dr Malcolm Benson, Dr Penny Bradbu ry, Mrs Debbie Buttar, Dr James Calvert, Dr Jane Collier, Dr Graham Collins, Dr Chris Conlo n, Dr Chris Davies, Dr Helen Davies, Dr Rob Davies, Dr Thearina de Beer, Mrs Joan Douglass, Dr Rachael Evans, Dr Fergus Gleeson, Dr Maxine Hardinge, Dr Ling Pei Ho, Dr Andrew Jeffrey s, Dr Nick Maskell, Dr Phil Mason, Dr Kim McAnaulty, Dr Fiona McCann, Dr Sarah Menzies, Dr Annabel Nickol, Dr Jayne Norcliffe, Dr Jeremy Parr, Mrs Lisa Priestley, Dr Naj Rahman, Dr C atherine Richardson, Mrs Jo Riley, Dr Peter Sebire, Mrs Gerry Slade, Dr Mark Slade, Dr S Ro lf Smith, Dr Catherine Swales, Dr Denis Talbot, Dr David Taylor, Dr Catherine Thomas, Dr Es t e T r k, Dr David Waine, Dr Chris Wathen, Dr John Wiggins, and Dr Eleanor Wood. Second edition: additional acknowledgements Dr Luke Howard, Dr Clare Jeffries, Dr Stuart Mucklow, Dr Andrew Stanton, and Mrs Jan Turner -Wilson. Dr Fergus Gleeson and Dr Rachel Benamore provided considerable help with the radio logy section. vii Contents Abbreviations xi Part 1 Clinical presentations \200\224approaches to problems 1 Breathlessness 2 Chest pain 3 Chronic cough and a normal CXR 4 Critically ill patient wit h respiratory disease 5 Diffuse alveolar hemorrhage 6 Diffuse lung disease 7 Haemoptysis 8 Pleural effusion 9 Post-operative breathlessness 10 Pregnancy and breathlessness 11 Pre-ope rative assessment 12 Pulmonary disease in the immunocompromised 3 7 11 21 25 29 39 45 57 61 65 (non-HIV) 67 13 Pulmonary disease in the immunocompromised (HIV) 79 85 91 14 Sleep and ventilation 15 Unexplained ventilatory failure Part 2 Clinical conditions 16 Acute respiratory distress syndrome 17 Asbestos and the lung 18 Asthma 19 Bronchiectasis 101 107 121 149 CONTENTS 20 Bronchiolitis 159 21 Chronic obstructive pulmonary disease (COPD) 165 22 Connective tiss ue disease and the lung 185 23 Cor pulmonale 201 24 Cystic \201 brosis 205 25 Eosinophili c lung disease 227 26 Extreme environments \200\224 \202 ying, altitude, diving 235 27 Gas trointestinal disease and the lung 243 28 Hypersensitivity pneumonitis 249 29 Hyperventilat ion syndrome 255 30 Idiopathic interstitial pneumonias 259 31 Lung cancer 279 32 Lung trans plantation 317 33 Mediastinal abnormalities 331 34 Pediatric lung disorders pertinent to ad ult patients 339 35 Pleural effusion 345 36 Pneumoconioses 363 37 Pneumothorax 373 38 Pulmo nary hypertension 385 39 Pulmonary thromboembolic disease 401 40 Respiratory infection \200 \224bacterial 419 41 Respiratory infection \200\224fungal 465 42 Respiratory infection \200 \224mycobacterial 487 43 Respiratory infection \200\224parasitic 523 44 Respiratory infecti on \200\224viral 529 45 Sarcoidosis 547 46 Sickle cell disease \200\224pulmonary complicati ons 561 ix CONTENTS 47 Sleep apnoea and hypoventilation 48 Toxic agents 49 Unusual conditions (BOLD) 50 Upper a irway diseases 51 Vasculitis and the lung 567 593 609 639 649 Part 3 Supportive care 52 Ethical issues 53 Financial entitlements 54 Immunosuppressive drugs 55 Inhalers and nebu lizers 56 Intensive therapy unit (ITU) \200\224when to involve 57 Non-invasive ventilation 58 Oxygen therapy 59 Palliative care 60 Pulmonary rehabilitation 61 Smoking cessation 62 Tr acheostomy 665 671 675 683 689 693 703 713 723 729 735 Part 4 Practical procedures 63 Airway management 64 Bronchoscopy 65 Chest drains 66 Cricothyroidotomy 67 Miscellaneous diagnostic tests 68 Pleural biopsy 69 Pleurodesis 741 745 757 763 767 771 775 CONTENTS x 70 Pneumothorax aspiration 71 Thoracentesis 72 Thoracoscopy (medical) 779 781 785 Appendices 1 Blood gasses and acid \200\223base balance 791 2 BMI calculator; height and weight conver ter 805 3 CT anatomy of the thorax 807 4 CT patterns of lung disease 815 5 Lung function te sting 827 6 Plain radiograph and lobar collapses 837 7 Radiological investigations and radi ation exposure 841 8 Useful websites 845 Index 847 FEV1 nomogram \200\224males inside cover FEV1 nomogram \200\224females inside cov er xi Abbreviations A \200\223a ABG ABPA ACE ADH AFB AFP AIA AIDS AIP ANA ANCA ANP APTT ARDS ATRA ATS AVM A&E B AL BCG bd BHR BIPAP BMI BNP BOOP BP BPD BSAC BTS CABG CAP CCB CCF CF alveolar to arterial gradient arterial blood gas allergic bronchopulmonary aspergillosis an giotensin-converting enzyme antidiuretic hormone acid-fast bacillus alpha-fetoprotein aspir in-induced asthma acquired immune de \201 ciency syndrome acute interstitial pneumonia ant inuclear antibody antinuclear cytoplasmic antibody atrial natriuretic peptide activated par tial thromboplastin time acute respiratory distress syndrome all-trans retinoic acid Americ an Thoracic Society arteriovenous malformation accident and emergency bronchoalveolar lavag e bacille Calmette \200\223Gu rin twice a day bronchial hyperreactivity or hyperresponsive ness bi-level positive airways pressure body mass index (kg/m2) B-type natriuretic peptide bronchiolitis obliterans organizing pneumonia blood pressure bronchopulmonary dysplasia Bri tish Subaqua Club British Thoracic Society coronary artery bypass graft community-acquired pneumonia calcium-channel blocker congestive cardiac failure cystic \201 brosis xii ABBREVIATIONS CFA CFT CFTR CHART CI CLL CMV CNS CO CO2 COHb COP COPD CPAP CRP CSA CSF CT CTPA CVA CVD CVP CXR DIC DIP dsDNA DVLA DVT EBUS ECG Echo ECOG EEG EGFR EIA ELCAP ELISA EMG ENT EOG cryptogenic \201 brosing alveolitis complement \201 xation test cystic \201 brosis tr ansmembrane conductance regulator continuous hyperfractionated accelerated radiotherapy con traindication chronic lymphocytic leukemia cytomegalovirus central nervous system carbon mo noxide carbon dioxide carboxyhaemoglobin cryptogenic organizing pneumonia chronic obstructi ve pulmonary disease continuous positive airway pressure C-reactive protein central sleep a pnoea cerebrospinal \202 uid computed tomography computed tomographic pulmonary angiogram cardiovascular accident cardiovascular disease central venous pressure chest radiograph di sseminated intravascular coagulation desquamative interstitial pneumonitis double-stranded DNA Department of Vehicle Licensing Authority deep vein thrombosis endoscopic bronchial ult rasound electrocardiogram echocardiogram Eastern Cooperative Oncology Group electroencephal ogram epidermal growth factor receptor enzyme immunoassay Early Lung Cancer Action Project enzyme-linked immunosorbent assay electromyogram ear, nose, and throat electro-oculogram ABBREVIATIONS xiii EPAP ERS ESR ESS EUS-FNA FBC FEV1 FiO2 FNA FOB FPAH FRC FVC g GBM GCS GI GORD GP GU GVHD h H2 HAART HACE HAPE Hb HCG HCO \200\223 3 HHT HIV HLA HOOF HP HRCT HSCT HSV ICU IDDM IFA expiratory positive airways pressure European Respiratory Society erythrocyte sedimentation rate Epworth sleepiness scale/score endoscopic ultrasound \201 ne-needle aspiration full blood count forced expiratory volume in 1 s fractional inspired oxygen \201 ne-needle as pirate \201 bre optic bronchoscopy familial pulmonary arterial hypertension functional re sidual capacity forced vital capacity gram glomerular basement membrane Glasgow coma scale gastrointestinal gastro-oesophageal re \202 ux disease general practitioner genitourinary graft-versus-host disease hour histamine receptors, type 2 highly active antiretroviral the rapy high altitude cerebral oedema high altitude pulmonary oedema hemoglobin human chorioni c gonadotrophin bicarbonate hereditary haemorrhagic telangiectasia human immunode \201 cie ncy virus human leucocyte antigen home oxygen order form hypersensitivity pneumonitis high resolution computed tomography haematopoietic stem cell transplant herpes simplex virus int ensive care unit insulin-dependent diabetes mellitus indirect immuno \202 uorescence assay ABBREVIATIONS xiv IgE IgG IgM IIP ILD IM INR IPAH IPAP IPF IRIS ITU IV IVC JVP kCO l LAM LCH LDH LFT LIP LMWH LOS LTOT LVRS M, C & S MDI MDR-TB MDT mg MGUS MI min MND MRI MRSA MTB ng NIPPV immunoglobulin E immunoglobulin G immunoglobulin M idiopathic interstitial pneumonia inters titial lung disease intramuscular international normalized ratio idiopathic pulmonary arter ial hypertension inspiratory positive airways pressure idiopathic pulmonary \201 brosis i mmune reconstitution in \202 ammatory syndrome intensive therapy unit intravenous inferior vena cava jugular venous pressure carbon monoxide transfer factor liter lymphangioleiomyom atosis Langerhans cell histiocytosis lactate dehydrogenase liver function test lymphoid int erstitial pneumonia low molecular weight heparin lower oesophageal sphincter long-term oxyg en therapy lung volume reduction surgery microscopy, culture, and sensitivity metered dose inhaler multidrug-resistant TB multidisciplinary team milligram monoclonal gammopathy of un certain signi \201 cance myocardial infarction minute motor neuron disease magnetic resona nce imaging methicillin (or multiply) resistant Staphylococcus aureus mycobacterium tubercu losis nanogram non-invasive positive pressure ventilation ABBREVIATIONS xv NIV NNRTI NO NO2 non-REM NSAID NSCLC NSIP NTM NYHA OCP od OSA OSAHS OSAS PaCO2 PAF PaO2 PAP PAS PAVM PC20 non-invasive ventilation non-nucleoside reverse transcription inhibitor nitric oxide nitrog en dioxide non-rapid eye movement sleep non-steroidal anti-in \202 ammatory drug non-small cell lung cancer non-speci \201 c interstitial pneumonia non-tuberculous mycobacteria New York Heart Association oral contraceptive pill once a day obstructive sleep apnoea obstruc tive sleep apnoea/hypopnoea syndrome obstructive sleep apnoea syndrome arterial carbon diox ide tension platelet-activating factor arterial oxygen tension pulmonary artery pressure pa ra-aminosalicylic acid pulmonary arteriovenous malformations provocative concentration (of histamine or methacholine) causing a 20% fall in FEV1 primary ciliary dyskenesia carbon dio xide tension Pneumocystis carinii (now jiroveci) pneumonia polymerase chain reaction primar y care trust pulmonary embolus positive end expiratory pressure peak expiratory \202 ow r ate percutaneous endoscopic gastrostomy position emission tomography pulmonary function tes t Public Health Laboratory Service pulmonary hypertension orally/by mouth oxygen tension pr imary pulmonary hypertension as required prostate-speci \201 c antigen PCD PCO2 PCP PCR PCT PE PEEP PEFR PEG PET PFT PHLS PHT PO PO2 PPH prn PSA ABBREVIATIONS xvi PSB PSG PT PTH qds RADS RAST RBBB RB-ILD protected specimen brush polysomnography prothrombin time parathyroid hormone four times a day reactive airways dysfunction syndrome radioallergosorbent test right bundle branch bloc k respiratory bronchiolitis-associated interstitial lung disease Royal College of Physician s randomized controlled trial rapid eye movement radiofrequency ablation ribonuclear protei n respiratory syncytial virus reverse transcriptase polymerase chain reaction residual volu me right ventricular hypertrophy arterial oxygen saturation severe acquired respiratory syn drome subacute bacterial endocarditis subcutaneous scleroderma antibody (to topoisomerase 1 ) small cell lung cancer self-contained underwater breathing apparatus short burst oxygen t herapy side-effect systemic lupus erythematosus sulfur dioxide shortness of breath superior vena cava superior vena caval obstruction tuberculosis transbronchial biopsy three times a day T-helper 2 cell transient ischaemic attack total lung capacity total lung carbon monox ide transfer factor tumor necrosis factor RCP RCT REM RFA RNP RSV RT-PCR RV RVH SaO2 SARS SBE SC SCL-70 SCLC SCUBA SBOT SE SLE SO2 SO B SVC SVCO TB TBB tds Th2 TIA TLC TLCO TNF ABBREVIATIONS xvii TPMT TRALI TSH U&E UIP UK URT URTI USS V/Q VAP VATS VC VTE WBC WCC WHO ZN A1-AT \2372 \237HCG g thiopurine methyltransferase transfusion-related acute lung injury thyroid-stimulating horm one urea and electrolytes usual interstitial pneumonia United Kingdom upper respiratory tra ct upper respiratory tract infection ultrasound scan ventilation/perfusion ratio ventilator -acquired pneumonia video-assisted thoracoscopic surgery vital capacity venous thromboembol ism white blood cell count white cell count World Health Organization Ziehl \200\223Neelsen A1-antitrypsin beta-2 adrenergic receptor beta human chorionic gonadotrophin micrograms This page intentionally left blank Part 00 Part 1 Clinical presentations \200\224 approaches to problems 1 Breathlessness 2 Chest pain 3 Chronic cough and a normal CXR 4 Critically ill patient wit h respiratory disease 5 Diffuse alveolar hemorrhage 6 Diffuse lung disease 7 Haemoptysis 8 Pleural effusion 9 Post-operative breathlessness 10 Pregnancy and breathlessness 11 Pre-ope rative assessment 12 Pulmonary disease in the 3 7 11 21 25 29 39 45 57 61 65 67 immunocompromised (non-HIV) 13 Pulmonary disease in the 79 immunocompromised (HIV) 14 Sleep and ventilation 15 Unexplained ventilatory failure 85 91 This page intentionally left blank 3 Chapter 1 Breathlessness Clinical assessment and causes 4 Speci \201 c situations 6 CHAPTER 1 Breathlessness 4 Clinical assessment and causes Physiological mechanisms of breathlessness Dyspnoea refers t o the abnormal and uncomfortable awareness of breathing. Its physiological mechanisms are p oorly understood; pos- sible afferent sources for the sensation include receptors in respir atory muscles, juxtacapillary (J) receptors (sense interstitial \202 uid), and chemore- c eptors (sensing iCO2 and dO2). Clinical assessment All patients need a full history and exa mination. Key points in the assess- ment are: \200 Duration and onset of breathlessness. The box opposite groups the causes of breathlessness by speed of onset, although in practice some variability and overl ap exists. Patients often underestimate the duration of symptoms \200\224enquiring about ex ercise tolerance over a period of time is a useful way of assessing duration and progressio n Severity of breathlessness. Assess the level of handicap and disability by asking about effects on lifestyle, work, and daily activities Exacerbating factors. Ask about rest and exertion, nocturnal symptoms, and body position. T he timing of nocturnal breathlessness may provide clues to the likely cause: left ventricul ar failure causes breathlessness after a few hours of sleep, and resolves after about 45 mi n; asthma tends to occur later in the night; laryngeal inspiratory stridor causes noisy bre athlessness of very short duration (<1 min); and Cheyne \200\223 Stokes apnoeas result in b reathlessness that is recurrent and clears each time in less than 30 s. Orthopnoea is sugge stive of left ventricular failure or diaphragm paralysis, although it is also common in man y chronic lung diseases. Breathlessness during swimming is characteristic of bilateral diap hragm paralysis. Trepopnoea refers to breathlessness when lying on one side as a result of ipsilateral pulmonary disease - Associated symptoms, such as cough, haemoptysis, chest pain , wheeze, stridor, fever, loss of appetite and weight, ankle swelling, and voice change. Wh eeze may occur with pulmonary oedema, pulmonary embolism, bronchiolitis, and anaphylaxis, i n addition to asthma and COPD Personal and family history of chest disease - Lifetime employment, hobbies, pets, travel, smoking, illicit drug use, medications Examination of the cardiovascular and respiratory systems. Observe the pattern and rate of breathing. Assess for signs of respiratory dis- tress. Look for paradoxical abdominal movem ent if the history suggests diaphragmatic paralysis. A useful bedside test is to exercise t he patient (e.g. by stepping on and off a 15 \200\22320-cm block) until their breathlessnes s occurs, and then measure oximetry immediately on stopping when the \201 nger is still; a fall in oxygen saturation is expected with organic causes of dyspnoea. CLINICAL ASSESSMENT AND CAUSES 5 Investigations Initial investigations typically include resting oximetry, peak \202 ow an d spirometry, CXR, and ECG. Further tests depend on clinical suspicion; options include ful l PFTs with measurement of lung volumes lying and standing, gas transfer and \202 ow-volu me loop, bronchial hyperresponsive- ness or reversibility testing, maximal mouth or inspira tory sniff pressures, ABGs (with measurement of A \200\223a gradient, see p 793), exercise oximetry, ventilation perfusion scanning and CTPA, HRCT, blood tests (full blood count and TSH), echo, exercise ECG, and cardiac catheterization. Causes of breathlessness grouped by speed of onset Instantaneous - Pneumothorax - Pulmonary embolism. Acute (minutes \200\223hours) - Airways disease (asthma, exacerbation of COPD, upper airways obstruction) Parenchymal disease (pneumonia, pulmonary oedema, pulmonary hemorrhage, acute hypersensitivity pneumonitis) - Pulmonary vascular disease (pulmonary emb olism) - Cardiac disease (e.g. acute myocardial infarction, arrhythmia, valvular disease, tamponade, aortic dissection) Metabolic acidosis - Hyperventilation syndrome. Subacute (days) - Many of the above, plus: \200 Pleural effusion \200 Lobar collapse \200 Acute interstitial pneumonia \200 Su perior vena cava obstruction \200 Pulmonary vasculitis. Chronic (months \200\223years) - Some of the above, plus: Obstructive airways disease (COPD, asthma) \200 Diffuse parenchymal disease (including id iopathic pulmonary \201 brosis, sarcoidosis, bronchiectasis, lymphangitis carcinomatosis) Pulmonary vascular disease (chronic thromboembolic disease, idiopathic pulmonary hypertensi on, veno-occlusive disease) Hypoventilation (chest wall deformity, neuromuscular weakness, obesity) \200 Anemia \200 Thyrotoxicosis. CHAPTER 1 Breathlessness 6 Speci \201 c situations Causes of breathlessness with a normal CXR - Airways disease (asth ma, upper airways obstruction, bronchiolitis) - Pulmonary vascular disease (pulmonary embol ism, idiopathic pulmo- nary hypertension, intrapulmonary shunt) Early parenchymal disease (e.g. sarcoid, interstitial pneumonias, infec- tion \200\224viral, PCP) Cardiac disease (e.g. angina, arrhythmia, valvular disease, intracardiac shunt) Neuromuscular weakness - Metabolic acidosis - Anemia - Thyrotoxicosis - Hyperventilation sy ndrome (p 255). Causes of episodic/intermittent breathlessness - Asthma - Pulmonary oedema - Angina - Pulmo nary embolism - Hypersensitivity pneumonitis - Vasculitis - Hyperventilation syndrome. Distinguishing cardiac and respiratory causes of breathlessness This can be dif \201 cult. Many of the clinical features of left heart failure are non-speci \201 c and easily confu sed with respiratory disease (e.g. orthop- noea, wheeze). In chronic cardiac failure, crack les on auscultation and radiological features of pulmonary oedema may be absent even when t he pulmonary capillary wedge pressure is signi \201 cantly raised (due to adap- tive chang es from vascular remodeling). The presence of emphysema may also render crackles inaudible and lead to atypical CXR appearances of pulmonary oedema. Chronic left heart failure common ly leads to a restrictive ventilatory defect and reduced gas transfer on PFTs, and may also result in pulmonary hypertension. HRCT features of left heart failure include septal and p eribronchovascular interstitial thickening, ground-glass shadowing, pleural effusions, and cardiomegaly. Resting ECG is useful \200\224in practice, a cardiac cause of breathlessness is unlikely in the setting of a completely normal ECG. Exercise ECG, echo, and cardiac cath eterization may be required. Measurement of B-type natriuretic peptide (BNP) is a recent de velopment that may be of value in the diagnosis of cardiac failure; in patients presenting as an emergency with breathlessness, a serum BNP level <50 ng/L makes cardiac failure very unlikely. Cardiac and respiratory diseases can, of course, coexist. Further information Maisel AS, Krishnaswamy P et al. Rapid measurement of B-type natriureti c peptide in the emer- gency diagnosis of heart failure. N Engl J Med 2002; 347: 161 \200 \2237 7 Chapter 2 Chest pain Acute pleuritic chest pain 8 Chronic chest pain 9 CHAPTER 2 Chest pain 8 The majority of patients with chest pain referred to the respiratory team have either acute pleuritic pain or persistent, well-localized pain. Cardiac pain rarely presents in this ma nner, although it should be considered in exer- tional pain or in the presence of risk fact ors for ischaemic heart disease. Within the respiratory system, pain may arise from the par ietal pleura, major airways, chest wall, diaphragm, and mediastinum; the lung parenchyma an d visceral pleura are insensitive to pain. Processes involving the upper parietal pleura ca use a pain localized to that part of the chest. The lower parietal pleura and outer region of diaphragmatic pleura are innervated by the lower six intercostal nerves, and pain here m ay be referred to the abdomen. The central region of the diaphragm is supplied by the phren ic nerve (C3, 4, and 5), and pain may be referred to the ipsilateral shoulder tip. Tracheob ronchitis tends to be associated with retrosternal pain. Acute pleuritic chest pain - Pleuritic pain is sharp, well-localized, worse on coughing and inspiration, and the subsequent limitation of inspiration often leads to a degree of breat hlessness Causes of acute pleuritic chest pain include: Pulmonary infarction (following embolism) \200 Pneumonia \200 Pneumothorax \200 Peric arditis \200 Pleural infection (empyema, tuberculous) \200 Autoimmune disease (e.g. sys temic lupus erythematosus, rheumatoid arthritis) Musculoskeletal \200 Fractured rib In addition, consider atypical presentations of serious conditions such as myocardial infar ction, aortic dissection, oesophageal rupture, and pancreatitis. Consider angioinvasive fun gi, such as aspergillus, as a cause of chest pain in the immunocompromised Diagnosis is typically based on \200\230pattern recognition \200\231 of clinical features followed by selected investigations. Initial investigations typically include CXR, ECG, ABG s, serum in \202 ammatory markers, and D-dimers. Further investigations may include V/Q sc anning or CT pulmonary angiography, pleural aspiration, and measurement of serum autoantibo dies Pulmonary embolism (p 401) commonly presents with pleuritic pain and exclusion of this diag nosis is the usual reason for referral. Assess risk factors for thromboembolic disease. Nor mal oxygen saturations and PaO2 in the \200\230normal \200\231 range do not exclude the di agnosis; calculate the A \200\223a gradient (p 793). The presence of a pleural rub is a non - speci \201 c sign that occurs with pleural in \202 ammation of any cause - In young adu lts, pneumococcal pneumonia may present with acute onset pleuritic chest pain, although systemic symptoms such as fever usually predate the pa in by hours CHRONIC CHEST PAIN 9 The pain from pericarditis is pleuritic, but central, and relieved on leaning forward; there may also be a pericardial rub, characteristic ECG features, and a sm all pericardial effusion on echo Musculoskeletal pain may occur as a result of cervical disc disease, arthritis of the shoul der or spine, a fractured rib, or costochondritis (Tietze \200\231s syndrome), which often follows a viral infection The presence of chest wall tenderness does not invariably indicate a benign, musculoskeleta l cause; tenderness may be seen in malignant chest wall in \201 ltration and sometimes fol lowing pulmonary infarction - Other features besides pleurisy that may suggest a diagnosis of systemic lupus erythematosus include rash, photosensitivity, oral ulcers, arthritis, per icarditis, renal or neurological disease, cytopenia, positive ANA, and dsDNA. Chronic chest pain - Persistent chest pain that is well localized is typically caused by ch est wall or pleural disease. Causes include: \200 Malignant pleural disease or chest wall in \201 ltration \200 Benign musculoskeletal pain \200 Pleural infection (empyema, tuberc ulous) \200 Benign asbestos pleural disease \200 Autoimmune disease (e.g. systemic lupu s erythematosus, rheumatoid arthritis) Recurrent pulmonary infarction (emboli, vasculitis) Pain from malignant chest wall in \201 ltration is often \200\230boring \200\231 in chara cter, and may disturb sleep; it is frequently not related to respiration. Causes include pr imary lung cancer, secondary pleural malignancy, mesothe- lioma, and rib or sternal involve ment from malignancy (including myeloma and leukemia) Chronic thromboembolic disease tends to present with breathlessness; when chest pain occurs , it is usually episodic, rather than persistent - As with acute pleuritic pain, investigat ions are directed by initial clinical suspicion. Consider CT chest, bone scan, serum autoan tibodies, full blood count and \201 lm, serum electrophoresis. CXR may appear normal in m alignant chest wall disease. This page intentionally left blank 11 Chapter 3 Chronic cough and normal CXR Etiology and clinical assessment 12 Treatment 14 Causes of cough (with or without CXR abnor mality) 15 Causes of chronic cough: asthma, GORD 16 Causes of chronic cough: rhinitis, post -infectious, ACE inhibitors, idiopathic 18 CHAPTER 3 Chronic cough and normal CXR 12 Etiology and clinical assessment Cough is a frequent symptom of many respiratory diseases a nd is often associated with underlying lung pathology and an abnormal CXR. Cough can occur in otherwise healthy people and is often a self-limiting symptom. Persistent coughing can b e a socially disabling and distressing symptom, for which help is often sought. Cough synco pe is loss of consciousness following violent coughing, a Valsalva type maneuver, which imp airs venous return to the heart and provokes bradycardia and vasodilatation (similar to an ordinary faint). Important as car drivers must cease driving until liability to cough synco pe has ceased, con \201 rmed by medical opinion; commercial drivers must cease driving and have no cough syncope or pre- syncope for 5 years if they have a chronic respiratory condi tion, including smoking. If they have asystole due to cough, driving can be considered afte r pacemaker insertion. \200 Acute cough = cough lasting less than 3 weeks, usually due to viral upper respiratory tract infection. May linger for 3 \200\2238 weeks as a \200\230post-viral cough \200\231 Chroni c cough = cough lasting more than 8 weeks Patients with a normal CXR and persistent cough a re often grouped under the heading \200\230chronic cough \200\231 It can sometimes be dif \201 cult to determine the underlying cause Susceptible individuals have a heightened coug h re \202 ex Investigation is warranted, but successful response to therapeutic trials may aid determination of the underlying cause. Centers vary in their approach to this Speciali st cough clinics suggest they achieve diagnosis and effective treatment in over 80% of pati ents referred with chronic cough. \200 \200 \200 Etiology In practice, over 90% of cases of chronic cough with a normal CXR are caused by on e or more of: \200 \200 \200 Cough variant asthma or eosinophilic bronchitis Gastro-oesophageal re \202 ux disease Post -nasal drip, due to perennial or allergic rhinitis, vasomotor rhinitis, or chronic sinusiti s. In clinic: full history can be unhelpful. Although cough is most commonly due to asthma, re \202 ux, or post-nasal drip, there may be no speci \201 c symptoms to suggest these diag noses. \200 \200 Duration of cough When it tends to occur \200\224night or early morning, after exertion, on exposure to dust, pollen, or cold air (asthma), after meals or on sitting or bending over (GORD), nocturnal (post-nasal drip and asthma) Non-productive or productive and, if so, how much sputum and color. Signi \201 cant amounts of sputum usually indicate a primary lung pathology Haemoptysis Fever \200 etiology AND CLINICAL ASSESSMENT 13 Associated symptoms: \200 \200 \200 \200 \200 Previous respiratory disease, such as childhood asthma, eczema, or hay fever History of sinus disease or perennial rhinitis Hi story of previous severe respiratory infections, such as whooping cough, that may have caus ed bronchiectasis Known cardiac disease or valvular heart disease Drug history ?ACE inhibit or Occupation ?Workplace irritants Pets/birds Smoker Use of recreational drugs. Shortness of breath or wheeze Throat clearing or sensation of post-nasal drip Chest pain An kle swelling/orthopnoea/paroxysmal nocturnal dyspnoea Dyspepsia \200 \200 \200 \200 \200 \200 Examination can also be unhelpful, as it is usually normal. Look for signs of underlying lu ng disease or other medical conditions, such as heart failure, neurological disease (partic ularly bulbar involvement). Investigations Initially \200 \200 Ensure CXR is normal Spirometry may indicate restrictive or obstructive defect. Performance of spirometry may provoke cough and bronchospasm Methacholine provocation test (p 769) pro vides the best positive predictive value for cough due to asthma. A lack of response sugges ts cough variant asthma is extremely unlikely. PC20 is normal in eosinophilic bronchitis Se rial peak \202 ow recordings twice daily for 2 weeks. Greater than 20% diurnal variation suggests asthma. Can be normal in cough variant asthma Induced sputum examination, if avail able, for eosinophil count, to suggest either asthma or eosinophilic bronchitis. Usually a research tool. Later \200 Consider chest HRCT if any features suggestive of lung cancer or interstitial lung disease, as a small proportion may present with a normal CXR (central tumor) Consider ENT examinati on if predominantly upper respiratory tract disease, resistant to treatment. Consider sinus CT Consider bronchoscopy if foreign body possible, or history suggestive of malignancy, sm all carcinoid, endobronchial disease. Perform after CT to help guide bronchoscopist Conside r oesophageal pH monitoring. CHAPTER 3 Chronic cough and normal CXR 14 Treatment The initial treatment of patients with a chronic cough is determined by what the most likely underlying cause is, based on the history and investi- gations. The key is to g ive any drug treatment at a high enough dose, and for a long enough time (such as 3 months) , to be effective. Symptomatic treatment for cough Over the counter medicines may provide relief, although the re is little evidence of a speci \201 c pharmacological effect. Below is a list of possibl e treatments: \200 \200 Honey and lemon \200\224home remedies Dextromethorphan \200\224a non-sedating opiate. Compo nent of many over the counter cough remedies. Dose response, with maximum cough re \202 ex suppression at 60 mg. (Benylin preparations, Actifed preparations, Vicks Vaposyrup p reparations, Sudafed Linctus , Night Nurse ) Menthol \200\224short lived cough suppressan t (Benylin preparations, Vicks Vaposyrup preparations) Sedative antihistamines \200\224 suppress cough, but cause drowsiness. Good for nocturnal cough Codeine or pholcodine \200 \224opiate antitussives \200\224codeine requires prescription. No greater ef \201 cacy tha n dextromethorphan and greater side-effect pro \201 le Opiates \200\224prescription. Low d ose morphine sulfate 5 \200\22310 mg showed signi \201 cant improvement in patients with i ntractable cough in RCT (Morice AH et al Am J Crit Care Med 2007). Side-effect pro \201 le of opiates, so should be used with caution. CAUSES OF COUGH (WITH OR WITHOUT CXR ABNORMALITY) 15 Causes of cough (with or without CXR abnormality) Respiratory \200 Infection: viral upper and lower respiratory tract infection, bacterial pneumonia, TB, pert ussis Chronic bronchitis Obstructive airways disease: COPD, asthma Cough variant asthma Eos inophilic bronchitis Obstructive sleep apnoea (nocturnal only) Lung cancer Bronchiectasis, cystic \201 brosis Interstitial lung disease Airway irritants: smoking, dusts and fumes, acute smoke inhalation Airway foreign body. \200 \200 \200 \200 \200 \200 \200 \200 \200 Mediastinal \200 