CROFTON AND DOUGLAS’S RESPIRATORY DISEASES CROFTON AND DOUGLAS’S RESPIRATORY DISEASES EDITED BY ANTHONY SEATON CBE, BA, MD, FRCP, FRCPE, FFOM, FMedSci Professor of Environmental & Occupational Medicine, University of Aberdeen, Scotland Honorary Consultant Physician, Aberdeen Royal Hospital NHS Trust DOUGLAS SEATON MD, FRCP Consultant Physician, Department of Respiratory Medicine, The Ipswich Hospital NHS Trust, Suffolk, England The late A. GORDON LEITCH BSc, MB, PhD, FRCPE, FCCP FIFTH EDITION IN TWO VOLUMES VOLUME 1 © 2000 by The right of the Authors to be distributors Blackwell Science Ltd identified as the Authors of this Work Marston Book Services Ltd Editorial Offices: has been asserted in accordance PO Box 269 Osney Mead, Oxford OX2 0EL with the Copyright, Designs and Abingdon, Oxon OX14 4YN 25 John Street, London WC1N 2BL Patents Act 1988. (Orders: Tel: 01235 465500 23 Ainslie Place, Edinburgh EH3 6AJ Fax: 01235 465555) All rights reserved. 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Respiratory organs —Diseases. registered at the United Kingdom I. Seaton, Anthony. II. Seaton, Trade Marks Registry Douglas. III. Leitch, A. Gordon (Andrew Gordon) IV. Crofton, John, Sir, 1912– Respiratory diseases. V. Title: Respiratory diseases. [DNLM: 1. Respiratory Tract Diseases. WF 140 C9413 2000] RC731.C7 2000 616.2 —dc21 DNLM/DLC for Library of Congress 99-15579 CIP CONTENTS Contributors, vii 13 Pneumonia, 356 Douglas Seaton Preface, ix 14 Empyema, 445 Acknowledgements, x Douglas Seaton Volume 1 15 Lung Abscess, 460 1 Development and Structure, 1 Douglas Seaton Anthony Seaton 16 Tuberculosis: Pathogenesis, Epidemiology and 2 Functions of the Lung, 26 Prevention, 476 A.Gordon Leitch A.Gordon Leitch 3 Epidemiology, 63 17 Pulmonary Tuberculosis: Clinical Features, 507 Anthony Seaton A.Gordon Leitch 4 Lung Defences and Immunology, 83 18 Extra-Pulmonary Tuberculosis, 528 Christopher Haslett R.Andrew Seaton 5 Genetics of Lung Disease, 91 19 Management of Tuberculosis, 544 Julian M.Hopkin A.Gordon Leitch 6 Clinical Aspects, 102 20 Opportunistic Mycobacterial Disease, 565 Anthony Seaton A. Gordon Leitch 7 Diagnostic Imaging, 119 21 Actinomycotic and Fungal Diseases, 573 Arthur J.A.Wightman Anthony Seaton 8 Minimally Invasive Diagnostic Procedures, 148 22 Parasitic Diseases, 604 Douglas Seaton Anthony Seaton 9 Drugs in Lung Disease, 193 23 Chronic Bronchitis and Emphysema, 616 Douglas Seaton William MacNee 10 Smoking, 311 24 Respiratory Failure, 696 Ian A.Campbell William MacNee 11 Air Pollution, 324 25 Pulmonary Embolism, 718 Anthony Seaton Douglas Seaton and Anthony Seaton 12 Acute Upper Respiratory Tract Infection, 335 26 Pulmonary Hypertension, 748 Douglas Seaton Anthony Seaton v vi / CONTENTS 27 Pulmonary Oedema and Adult Respiratory Distress 44 Pneumothorax, 1182 Syndrome, 766 Douglas Seaton Christopher Haslett 45 Chest Wall and Neuromuscular Disorders, 1212 28 Bronchiectasis, 794 Anthony Seaton Douglas Seaton 46 Abnormalities and Diseases of the Diaphragm, 1234 Index Anthony Seaton 47 Sleep Apnoea/Hypopnoea Syndrome, 1250 Volume 2 Neil J. Douglas 29 Bronchiolar Disease, 829 48 Hyperventilation Syndromes, 1264 Anthony Seaton Anthony Seaton 30 Cystic Fibrosis, 839 49 Diseases of the Mediastinum, 1269 Andrew P. Greening Douglas Seaton 31 Pulmonary Fibrosis, 877 50 Developmental Disorders of the Lungs, 1309 Anthony Seaton Douglas Seaton and Anthony Seaton 32 Asthma: Epidemiology, 894 51 Some Less Common Pulmonary Diseases, 1330 Peter G.J.Burney Anthony Seaton 33 Asthma: Cellular and Humoral Mechanisms, 907 52 Respiratory Infection in the Christopher Haslett Immunosuppressed, 1346 R.Andrew Seaton, Julian M.Hopkin and Douglas Seaton 34 Asthma: Clinical Features, 922 Anthony Seaton and Graham Crompton 53 Pulmonary Manifestations of Systemic Disease, 1380 Anthony Seaton 35 Asthma: Management, 973 Graham Crompton 54 Occupational Lung Diseases, 1404 Anthony Seaton 36 Reactive Airways Dysfunction Syndrome, 998 Anthony Seaton 55 Drug-induced Lung Disease, Oxygen Toxicity and Related Syndromes, 1458 37 Hypersensitivity Lung Diseases, 1002 Anthony Seaton Anthony Seaton 56 Some Paediatric Influences on Adult Lung 38 Pulmonary Eosinophilias, 1020 Disease, 1476 A.Gordon Leitch George Russell 39 Sarcoidosis, 1035 57 Diving and the Lung, 1481 A.Gordon Leitch Stephen J.Watt 40 Pulmonary Lymphocytic Angiitis and 58 Assisted Ventilation, 1495 Granulomatosis, 1063 John M.Shneerson Anthony Seaton 59 Lung Transplantation, 1516 41 Lung Cancer, 1077 Timothy W.Higgenbottam Ronald J.Fergusson 60 Terminal Care in Respiratory Disease, 1524 42 Other Pulmonary Neoplasms and Related Douglas Seaton Conditions, 1124 Anthony Seaton 61 Medicolegal Aspects of Lung Disease, 1536 Anthony Seaton 43 Diseases of the Pleura, 1152 Anthony Seaton Index Colour plate section falls between pages 630 and 631, Vol. 1. CONTRIBUTORS PETER G.J . BURNEY MA, MD, FRCP, FFPHM, A. GORDON LEITCH [Deceased] RVCC, Chalmers Department of Public Health Sciences, King’s College London, Hospital, Lauriston Place, Edinburgh EH3 9HA Capital House, 42 Weston Street, London SE1 3QD WILLIAM MACNEE MD, FRCPE, Department of IAN A. CAMPBELL BSc, MD (Lond.), FRCP, Respiratory Medicine, Royal Infirmary of Edinburgh, Lauriston Consultant Chest Physician, Llandough Hospital, Cardiff Place, Edinburgh EH3 9YW CF64 2XX GRAHAM CROMPTON MBCHB, FRCPE, GEORGE RUSSELL MB, FRCP, FRCPE, FRCPCH, Consultant Physician, Department of Respiratory Medicine, Department of Child Health, Royal Aberdeen Children’s Western General Hospital, Crewe Road, Edinburgh EH4 2XU Hospital, University of Aberdeen, Cornhill Road, Aberdeen AB25 2ZD NEIL J. DOUGLAS MD, FRCPE, Department of Respi- ratory Medicine, Royal Infirmary of Edinburgh, Lauriston Place, R. ANDREW SEATON MD, MRCP, DTM & H, Edinburgh EH3 9YW Directorate of Medicine, Tayside University Hospitals NHS Trust, Ninewells Hospital, Dundee DD1 9SY RONALD J . FERGUSSON MD, FRCPE, Consultant Physician, Respiratory Medicine Unit, Western General ANTHONY SEATON CBE, BA, MD, FRCP, FRCPE, Hospital, Edinburgh EH4 2XU FFOM, FMedSci, Professor of Environmental and Occupa- ANDREW P. GREENING FRCPE, Consultant tional Medicine, Aberdeen Royal Infirmary, Foresterhill, Physician and Senior Lecturer Department of Respiratory Aberdeen AB25 2ZD Medicine, Western General Hospital, Crewe Road, Edinburgh EH4 2XU DOUGLAS SEATON MD, FRCP, Consultant Physician, Department of Respiratory Medicine, The Ipswich Hospital CHRISTOPHER HASLETT BSc (Hons), MbchB NHS Trust, Heath Road, Ipswich IP4 5PD (Hons) FRCP, FRCPE, FMedSci, Head of Department of Respi- ratory Medicine Unit, University of Edinburgh, Royal Infirmary JOHN M. SHNEERSON MA, DM, FRCP, Director, Edinburgh, Lauriston Place, Edinburgh EH3 9YW Respiratory Support and Sleep Centre, Papworth Hospital, Papworth Everard, Cambridge CB3 8RE TIMOTHY W. HIGGENBOTTAM BSc, MA, MD, FRCP, Consultant Physician, Section of Respiratory and Molecular Medicine, Department of Medicine and Pharmacol- STEPHEN J . WATT BSc, FRCPEd, AFOM, Department ogy, University of Sheffield, Royal Hallamshire Hospital, of Environmental & Occupational Health, University of Sheffield S10 2JF Aberdeen, Foresterhill, Aberdeen AB25 2ZD JULIAN M. HOPKIN MD, MSc, MA, FRCP, FRCPE, ARTHUR J .A. WIGHTMAN MBBS, DMRD, FRCR, Professor of Experimental Medicine, Experimental Medicine Consultant Radiologist, Royal Infirmary Edinburgh, Lauriston Unit, University of Wales, Swansea SA2 8PP Place, Edinburgh EH3 9YW vii PREFACE A decade has passed since we wrote the previous edition in Britain and the improved outlook for young victims of Crofton and Douglas, in conjunction with our late col- of cystic fibrosis. We have, however, made little impact league Gordon Leitch. Gordon’s untimely death, giving on the prognosis of lung cancer, have not had as much his life while saving those of others, was a very sad blow success as we would have liked in the battle against the not only to his family but also to his many friends and col- amoral tobacco industry, and have watched in dismay as leagues in Scotland and over the world. His work in the poor medical practice in other countries has encouraged control and management of tuberculosis continued in the the development of multi-drug resistant tuberculosis. footsteps of Sir Robert Philip and Sir John Crofton, and he And, in spite of all the research in the subject, we have seen would undoubtedly have made a most important contri- asthma become progressively more prevalent in children. bution to the international battle against the disease had In our day-to-day care of sick patients, we must not take he survived. He was also a splendid all-round physician; our eyes off the public health aspects of our specialty. something of the flavour of this comes across in the chap- The previous edition of this book was well-received, ters he contributed to this book and which he delivered to and its translation into Greek and Italian, together with us just the day before he left on his tragic holiday. its production in a low-cost Asian edition, served as a The first edition of this book was published in 1969, at reminder to us of the need to write for a world-wide read- the end of an era in which medical research had made the ership. In this edition, we have reflected the increase in most notable contributions to the direct care of patients understanding of disease processes that has accrued from and to the prevention of disease, marked by the discovery basic research, but we have also endeavoured to maintain of antibiotics and antituberculous chemotherapy, the the tradition of writing for the practising physician, who demonstration of the harmful effects of smoking, and the sees a multitude of patients with diseases common and elucidation of the structure of DNA. The original volume rare, and who needs guidance on diagnosis and manage- was a relatively slim one, written by our distinguished ment. We are grateful to a number of friends and col- predecessors, John Crofton and Andrew Douglas, who leagues for agreeing to contribute to this edition and had themselves played a major role in the battle against believe that their chapters, emphasizing the common and tuberculosis. At that time, respiratory medicine was look- important, will contribute greatly to the value of the book. ing towards an uncertain future, as tuberculosis declined One of the benefits of writing a book such as this is the and other respiratory diseases remained firmly in the amount one learns or re-learns by reading the references realm of the generalist. Crofton and Douglas’s Respiratory necessary to check up on one’s statements. We have main- Diseases was perhaps the major factor in helping chest tained a substantial bibliography, and this includes a physicians of that era find their new role, allowing us to number of older references that give graphic original assert the importance of respiratory disease as a cause of accounts of diseases. It is not uncommon for old lessons to morbidity and mortality in both the developed and poor be forgotten and omitted from modern databases; while worlds. no textbook can hope to be as up-to-date as these data- The intervening three decades have seen great changes bases, we hope we will help readers to avoid missing in the practice and basic science of respiratory medicine, important earlier work while still keeping abreast of which is now recognized as a main-line acute specialty recent advances. We see this as a book to be used on the responsible for the care of a high proportion of the sick in ward and in the office, where clinical problems arise and all countries. That our patients are often from the least questions are asked and need clear answers. privileged sections of society has meant that funds for Anthony Seaton research and clinical care have not always been so easy to Douglas Seaton obtain as in more glamorous disciplines, but we can look back with some satisfaction to the control of tuberculosis ix ACKNOWLEDGEMENTS The observant reader will have noticed the similar names so, not least our juniors who impose a constant challenge of the two editors. We should like to acknowledge certain to keep up-to-date. aspects of our genetic and environmental heritage. Our With respect to the production of this book, we late father, Dr Ronald Seaton FRCP, was a pioneer in anti- acknowledge with gratitude the tolerance of Blackwell’s malarial chemotherapy. He inspired us to become doctors over our problems with deadlines and, especially, the and passed on to us a broad, lively and sometimes slightly courtesy and efficiency of Anna Woodford and the cynical interest in medicine. From our mother, Julia, a production staff. Our thanks also to the copy editor Jo nurse who worked with Lord Moynihan in Leeds and is Phillips for his attention to detail and his patience. We still busy looking after others as she approaches her tenth should also like to acknowledge the help of Dr Keith Kerr decade, we have inherited an aversion to a moment’s idle- in providing pathological photomicrographs, Dr Lesley ness. We have been fortunate in our teachers, notably the Gomersall for help in providing radiographs, and the late Harold Edwards who introduced us to biology and Medical Illustration Department of Aberdeen University evolutionary theory at school, Dr Colin Ogilvie of Liver- Medical School. pool who first interested us in respiratory medicine, and Most importantly, we record our gratitude to our wives, Professor Keith Morgan who introduced us to the Jill and Anja, for putting up with our prolonged absence at scientific basis of clinical and preventive medicine in the our computers and for nevertheless helping and support- wilds of West Virginia, USA. Many other teachers and col- ing us throughout this protracted endeavour. We promise leagues have of course influenced us and continue to do to spend more time with them in future. x 1 DEVELOPMENT AND STRUCTURE ANTHONY SEATON The respiratory system brings air into close relationship pattern of branching [3]. The mesenchyme itself develops with the mixed venous blood, allowing tissue respiration into the connective tissue, cartilage, smooth muscle and by uptake of oxygen into the circulation and elimination of vessels of the lung. In the first few weeks of development, carbon dioxide. In addition to this primary function, the nerve fibres arising from the ectoderm migrate into the lungs have other functions, for example water balance, the mesenchyme to give the lung its motor and sensory con- maintenance of pH, elimination of inhaled particles and nections [4]. The developing lung bud divides into two organisms, filtration of particulate matter from the circula- halves and elongates, growing caudally on either side of tion, and metabolism of certain drugs and enzymes. They the oesophagus. By about 33 days the trachea has become also serve as a vehicle for the administration of anaesthetic separated from the foregut, and pouches representing the and other drugs. The intimate contact between inspired five lobes are clearly apparent. Subsequent dichotomous air (with the multiplicity of organisms, particles and division leads to the development of the full adult com- gases that it contains) and the internal epithelium of the plement of segments by 41 days and to completion of lungs (with a surface area some three times that of the the bronchial tree as far as the terminal bronchioles by 16 body) leads not only to efficient gas exchange but also weeks [5]. While the embryonic lung is developing, to repeated opportunities for damage to the lungs and changes are also occurring in the circulatory system [6]. absorption of harmful substances into the body. In order to The primitive branchial arches come and go, leaving the understand both the normal function of the lungs and the third to form the carotids, the fourth the aorta and the pathology of the diseases with which this book is mainly sixth the pulmonary trunk (Fig. 1.1). This appears at about concerned, it is useful to know something of the develop- 32 days, becoming separated from the primitive truncus ment and structure of the organ. In particular, it is now arteriosus by the development of a spiral septum, and becoming clear that subtle influences on the development joins the vascular plexus that has already formed in the of the lung in utero and in the early months of extrauterine lung bud. By 37 days, the single ventricle of the heart has life may have important effects on lung health in later divided into two chambers, the blood supply to the lungs life, and that the seeds of later chronic airflow obstruction coming from the right side. At this stage the right sixth may be sown during the period of intrauterine lung arch artery has disappeared and the lung’s main blood development. supply, the pulmonary artery, comes solely from the left arch. Its branches divide approximately in correspon- dence with those of the bronchial tree, but so-called Development of the lungs supernumerary arteries occur with increasing frequency towards the periphery and supply structures adjacent to Development of the airways and vessels the main bronchi. Ultimately they will supply alveoli of The lung appears first as an epithelial bud at the caudal neighbouring acini when these have developed. Before end of the laryngotracheal groove on the 26th day after the formation of this pulmonary arterial supply, the lung ovulation [1,2]. It thus shares its origin with the foregut, receives its blood from pairs of segmental arteries arising reflecting the evolution in our invertebrate ancestors of a from the aorta above the coeliac axis in the region of the respiratory apparatus from the food-sieving mechanism. fetal neck. These arteries migrate caudally and eventually This bud, derived from endoderm, will form the epithe- disappear, being replaced by new bronchial arteries that lium of the airways and of the acini. As it elongates, it arise from the aorta between about 9 and 12 weeks. The becomes invested in mesenchyme derived from meso- persistence of the original primitive bronchial arteries is derm, and this mesenchymal layer exerts control over its the explanation for the occasional supply of sequestrated 1