Table Of ContentJohn A. Cosh . John V. Lever
Rheull1atic Diseases
and the Heart
With 112 Figures
Springer-Verlag
London Berlin Heidelberg New York
Paris Tokyo
John A Cosh, MA MD (Cantab), FRCP
Honorary Consultant Physician, Royal National Hospital for
Rheumatic Diseases, Upper Borough Walls, Bath BAllRL and
Royal United Hospital, Combe Park, Bath BA13NG, UK
John V Lever, BSc MBChB, FRCPath
Consultant Pathologist, Royal National Hospital for Rheumatic
Diseases, Upper Borough Walls, Bath BAllRL and Royal United
Hospital, Combe Park, Bath BAl 3NG, UK
ISBN-13 :978-1-4471-1645-5 e-ISBN-13 :978-1-4471-1643-1
DOl: 10.1007/978-1-4471-1643-1
British Library Cataloguing in Publication Data
Cosh, John A. (John Arthur), 1915-
Rheumatic diseases and the heart. 1. Man. Heart. Rheumatic diseases I. Title
II. Lever, John V. (John Vernon), 1938-616.1'27
ISBN-13 :978-1-4471-1645-5
Library of Congress Cataloging·in-Publication Data
Cosh, John A.
Rheumatic diseases and the heart. Includes bibliographies and index. 1. Heart
Diseases. 2. Rheumatism-Complications and sequelae. I. Lever, John V. (John
Vernon), 1938-. II. Title. [DNLM: 1. Heart Disease~omplications. 2. Rheumatic
Heart Disease~omplications. 3. Rheumatism--<:omplications. WG 200 C834r]
RC682.C69 1988 616.7'23 88-24848
ISBN-13 :978-1-4471-1645-5
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Foreword
What medicine lacks in an increasingly specialised world is the
medical polymath (or should it be medical bimath?) - the man who is
an acknowledged expert in more than one field of medicine.
Super§pecialisation in the professional sense is an attempt to know
more and more about less and less. It can be the microscopist's view
of reality and such a microcosm can become also a refuge from
realities. One reality difficult for the very specialised doctor to accept
is that diseases don't exist. Only people with diseases exist and people
do not always conform to convenient categories and compartments.
It would be nice if they did but somehow they never do.
A cherished medical tradition in Britain is that specialists are not
hatched straight from medical schools. Indeed, young doctors are
actively discouraged from taking too narrow an interest but are
plunged for at least four years into the unspecific pool of general
medicine until they have developed a broad awareness of the totality
of human disease. Before this they are not allowed to narrow their
sights on one particular target.
In this metamorphosis one or two gifted individuals somehow
manage to retain not just their general background but also a world
class reputation in more than one speciality. The late Philip Ellman
was a respiratory physician in one hospital and a rheumatologist in
another. What more natural than that he would make the major
contribution to the concept of rheumatoid lung and establish the idea
of rheumatoid disease. And there are many other examples. Just such
a bimath in the Ellman tradition is John Cosh, cardiologist in one
hospital, rheumatologist in another. His unique and perhaps unre
peatable experience is the basis of this work. It is a double distillation
a
of lifetime's work in two fields of medicine.
Cardiac manifestations of disease are only too often a cause of
death. But this has allowed John Lever, as pathologist, the
opportunity to view and illustrate what actually happens to the heart
and its surroundings as a result of these diseases.
vi Foreword
Readers will find a dozen conditions described here in which
patients may present both cardiac and rheumatic problems. Some are
rare manifestations of common conditions such as rheumatoid
arthritis. Some are curious associations like hyperlaxity of joints
associated with a prolapsing or floppy mitral valve. The classical
association of cardiac and joint disease, namely rheumatic fever with
rheumatic heart disease, is now happily rare. The virtual disappear
ance of rheumatic fever and rheumatic heart disease from affluent
countries gives one hope that some day the other painful and
sometimes lethal conditions described in this book will also be
banished from the earth.
Bath Allan St. J. Dixon
1988
Preface
For many years rheumatology has been one of the growing areas in
medicine. Our insight into the nature of the rheumatic diseases has
developed rapidly in the last three decades. As knowledge of their
immunological basis expands, our understanding has grown of their
pathology, their clinical manifestations and their epidemiology, even
though their ultimate causes still elude us.
We have come to see many of the rheumatic diseases as systemic
disorders whose effects are by no means confined to the joints and
musculo-skeletal system. Among their systemic effects there are
often important lesions of the heart and sometimes of the great
vessels. It is with these lesions that our text is concerned.
For nearly two centuries it could be said that rheumatic fever was
the only rheumatic disease known to involve the heart. Its import
ance lay in its frequency and in its often devastating effects on the
heart, rather than in its transient effects on the joints. But as
rheumatic fever has now almost vanished in western countries, and its
legacy of subsequent heart disease lessens, a greater relative
importance begins to attach to the cardiac lesions of the chronic
rheumatic diseases - rheumatoid arthritis, systemic lupus, ankylosing
spondylitis and others. Such lesions are, of course, overshadowed in
number by the commoner forms of heart disease, by coronary disease
in particular. Indeed, if a patient with, say, rheumatoid arthritis is
found to have a heart lesion, this may well have a separate cause,
unrelated to rheumatoid arthritis, arising by coincidence.
Unfortunately, in many parts of the world rheumatic fever and the
cardiac lesions that follow it continue tll be of major importance
today. For this reason we have dealt fully with these topics.
Moreover, the account given of the haemodynamic disturbances
caused by a rheumatic heart valve lesion applies equally to similar
lesions with a different underlying pathology.
The cardiac lesions caused by the different rheumatic diseases may
seem superficially alike: they may involve the pericardium, myocar-
viii Preface
dium or valves or combinations of these. But closer study of the
pathology shows that there are specific features in each type, and that
the morbid and histological changes in the heart reflect the special
features of the underlying disease itself.
Our aim therefore in this book is to introduce the general features
of each main rheumatic disease, and some uncommon ones, followed
by a description of the cardiac lesions found in that disease. Where
appropriate we seek to show how the cardiac pathology in each
disease is characteristic of the pathology of that disease as a whole.
The rheumatologist, in his concern for his patient's physical
problems may not always pay due attention to the heart. It is
important for him to appreciate that heart disease may arise as an
integral part of the rheumatic disease process, and that sometimes the
cardiovascular problems may have to take precedence over those of
the rheumatism.
This book is written for the general medical reader and assumes no
special knowledge of cardiology or of rheumatology. But we hope
that it will be of use to the cardiologist who may have little experience
of rheumatology as well as to the rheumatologist who may have little
experience of cardiology. We apologise therefore if some sections
seem unduly elementary to some readers.
Bath John Cosh
1988 John Lever
Acknowledgements
We wish to express our thanks to Mrs. Gina Machin and Mrs. Sally
Jenner of the Medical Photographic Department at the Royal United
Hospital, Bath, for their help with photographs and illustrations, and
to Mrs. Kate Clark, Medical Librarian, Royal United Hospital and
her staff.
We have been greatly helped by many of our collegues at the Royal
National Hospital for Rheumatic Diseases, particularly Professor
Allan Dixon and Professor Paul Bacon, now of Birmingham, and
Professor Peter Maddison, Dr. Tony Clarke and Dr. David Scott.
Also Drs. Ronald Bishton and Elizabeth Hall, pathologists, Dr.
Edgar Moore, radiologist and Dr. RD Thomas, cardiologist, of the
Royal United Hospital.
We also wish to thank Dr. Frank Ross, radiologist, of Bristol Royal
Hospital for echocardiograms and cine-angiograms, and Professor
JWB Bradfield for the use of material from the Pathology Depart
ment, University of Bristol.
Finally, we thank Dr. Richard Jacoby for his help in reviewing the
text of the manuscript.
Contents
1 Rheumatic Fever ......................................................... 1
Introduction ................................................................. 1
History ........................................................................ 1
Aetiology ..................................................................... 1
Incidence and Epidemiology ............................................ 3
Rheumatic Fever in the Third World ............................... 5
Immunopathology ......................................................... 6
Morbid Anatomy ........................................................... 7
Clinical Features............................................................ 9
Investigations ............................................................... 14
Diagnosis..................................................................... 16
Treatment.................................................................... 17
References ................................................................... 20
2 Rheumatic Heart Disease I ............................................ 23
Introduction ................................................................. 23
Incidence ..................................................................... 23
Pathology .................................................................... 24
General .................................................................... 24
Valve Lesions ............................................................ 24
Myocardium .............................................................. 27
Pericardium ............................................................... 27
The Lungs ................................................................. 27
Natural History ............................................................. 27
Mitral Valve Lesions ...................................................... 31
Mitral Stenosis: Haemodynamics .................................... 31
Mitral Stenosis: Clinical Effects ..... ....... .......... ......... ....... 31
Mitral Incompetence: Haemodynamics ............................ 33
Mitral Incompetence: Clinical Effects ...... .............. ..... ..... 34
Aortic Valve Lesions ...................................................... 35
Aortic Incompetence: Haemodynamics ........................... 35
x Contents
Aortic Incompetence: Clinical Effects ............................. 36
Aortic Stenosis: Haemodynamics ................................... 38
Aortic Stenosis: Clinical Effects ..................................... 39
Tricuspid and Pulmonary Valves ....................................... 39
The Myocardium ........................................................... 40
References ................................................................... 41
3 Rheumatic Heart Disease II ........................................... 43
Symptoms .................................................................... 43
Complications ............................................................... 43
Arrhythmias .............................................................. 43
Pulmonary Embolism .................................................. 45
Systemic Embolism ..................................................... 46
Infective Endocarditis .................................................. 46
Diagnostic Procedures .................................................... 46
Radiology ................................................................. 46
Electrocardiography .................................................... 49
Echocardiography ....................................................... 49
Angiocardiography ..................................................... 53
Differential Diagnosis ..................................................... 54
Prognosis ..................................................................... 57
Medical Management ..................................................... 58
Surgical Management ....................... ,............................. 59
References ................................................................... 63
4 Infective Endocarditis .................................................. 65
Introduction ................................................................. 65
Aetiology......................................... ............................ 65
Pathology .................................................................... 66
Immunology ................................................................. 67
Clinical Features ............................................................ 69
Classification .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . 69
Clinical Effects ........................................................... 69
Musculoskeletal Symptoms ........................................... 72
Diagnosis ..................................................................... 73
Treatment .................................................................... 74
Prophylaxis .................................................................. 75
References ................................................................... 76
5 Rheumatoid Arthritis: Rheumatoid Heart Disease I:
Pericarditis ................................................................... 77
Introduction ................................................................. 77
Immunology ................................................................. 77
Pathology .................................................................... 79
The Synovium ............................................................ 79
Nodules .................................................................... 80
Vasculitis .................................................................. 82
Contents xi
Clinical Features............................................ ................ 82
Arthritis .................................. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
Extra-articular Lesions. ... ........ ........... .......... .... ...... ...... 84
Pleurisy .................................................................... 85
Rheumatoid Heart Disease .............................................. 87
Summary .................................................................. 87
History ..................................................................... 88
Pericarditis ................................................................... 88
Incidence .................................................................. 88
Pathology ................................................................. 89
Clinical Features ......................................................... 92
Diagnosis of Pericarditis ............................................... 95
Treatment of Pericarditis .............................................. 97
Complications of Pericarditis ......................................... 99
References ................................................................... 103
6 Rheumatoid Heart Disease II: Valve Lesions, Heart Block,
Juvenile Arthritis ........................................................... 105
Valve Lesions ............................................................... 105
Incidence .................................................................. 105
Pathology ................................................................. 106
Clinical Features ............... ....... ................ .... .... ........ ... 111
Diagnosis .......... ........ ......... ......... ...... ....... ........ ......... 112
Treatment ................................................................. 115
Heart Block.................................................................. 116
Incidence.................................................................. 116
Pathology ................................................................. 117
Clinical Features ....... ...... ......... ... ........ ..... ........ ..... ... ... 118
Diagnosis .................................................................. 123
Treatment ................................................................. 125
Myocarditis .................................................................. 125
Coronary Arteritis ......................................................... 127
Juvenile Arthritis ........................................................... 127
Pericarditis ................................................................ 129
Valve Lesions ............................................................ 130
Adult Still's Disease ....... ........ ......... ........ ... .... ....... ...... 130
References ................................................................... 131
7 Systemic Lupus Erythematosus ...................................... 133
Introduction ................................................................. 133
Prevalence ................................................................... 134
Immunopathology ......................................................... 134
Autoantibodies .......................................................... 134
Aetiology .................................................................. 135
Vasculitis .................................................................. 136
Pathology ................................................................. 138
Drug-Induced SLE ...................................................... 138
Overlap Syndromes ..................................................... 139
