The Sclera and Systemic Disorders Peter G Watson, Brian L Hazleman, Peter McCluskey, Carlos E Pavésio
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s1THE SCLERA AND SYSTEMIC DISORDERS
THIRD EDITION
s2s3THE SCLERA AND SYSTEMIC DISORDERS
THIRD EDITION
Peter G Watson MA, MB, BChir, FRCS, FRCOphth, DO Formerly Böerhaave Professor, University of Leiden, Leiden, The Netherlands Honorary Consultant, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK Honorary Consultant, Moorfields Eye Hospital NHS Foundation Trust, London, UK Brian L Hazleman MA, FRCP Consultant Rheumatologist, Clinical Rheumatology Research Group, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK Associate Lecturer, Faculty of Medicine, University of Cambridge, UK Peter McCluskey MBBS, MD, FRANZCO, FRACS Director of Save Sight Institute, Professor of Ophthalmology University of Sydney, Sydney, Australia Carlos E Pavésio MD, FRCOphth Consultant Ophthalmologist, Moorfields Eye Hospital NHS Foundation Trust London, UK
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s5Preface
This book is the result of Peter Watson's career long interest in scleral and corneal disease which was stimulated by the first patient he saw when he started training in ophthalmology in 1956. A 40-year-old man, who had just arrived in England from Jamaica, came straight from the boat to the hospital with a very painful red eye and a persistent cough. It transpired that he had scleritis and a previously unsuspected open tuberculosis. On the very same morning an elderly lady attended the clinic complaining of a rapid deterioration of vision together with a very painful red eye which also had also developed a deep peripheral corneal gutter: a Mooren's ulcer. An extensive search of the then current literature revealed that no-one had any idea of the aetiology or any satisfactory way of treating either of these diseases, so, when the opportunity arose some years later, a clinic devoted entirely to inflammatory disease of the outer coats of the eye was established at Moorfields Eye Hospital in London under the title of the Scleritis Clinic.
If progress is to be made in determining the pathogenesis of an unusual, especially chronic, disease it is important to identify the salient features of each manifestation of the disease and to follow its progress over the long term. This is particularly important in conditions such as scleritis that are so often associated with other systemic diseases. The observations made in this scleritis clinic constitute much of the content of this book, and the necessity for continuous observation of those suffering from scleritis and its allied conditions is why the clinic still operates under the care of Carlos Pavésio, another of the authors.
Since the publication of the first edition in 1977 there has been an explosion in the understanding of the pathogenesis of the immune processes that underlie the development of scleral inflammation. The second edition, published in 2004, was much more comprehensive than the first and encompassed the full range of scleral disease. It also incorporated the previously untouched pathological material and immunological expertise of Dr Richard Green from Johns Hopkins Hospital in Baltimore who has sadly since died. The second edition also incorporated the newer methods of imaging the anterior segment of the eye that make the diagnosis of scleral disease easier and more accurate. Since then imaging techniques have become even more sophisticated to the extent that new anterior segment optical coherence tomography (OCT) imaging techniques described in this volume may transform the diagnosis and management of these difficult problems. There has also been a greater understanding of the minute anatomy and physiology of the sclera and its coats contributed to this volume by Dr Robert Young. This more complete knowledge has in turn led to a greater appreciation of the pathology and to the revolutionary changes in the way scleral inflammation is currently treated; hence this edition.
On the principle that ‘Pathology is the accomplished tragedy; physiology is the basis on which our treatment rests’ (Edward Martin 1859–1938), we have given a great deal of space to the understanding of the underlying biological changes which happen in these disorders. We make no excuse for this, for, whilst the principles of treatment of the various manifestations of the disease are largely unchanged, targeted specific therapy is becoming widely available. The indications for the use of these highly effective but potentially highly toxic medications cannot be understood without a clear vision of the underlying pathology.
Scleral disease is uncommon but even so it will be seen at some time in their career by all practicing ophthalmologists and by those physicians who manage patients with connective tissue disorders. Diagnosis is not always easy but it is important that the correct diagnosis is established at presentation as all forms of scleritis are potentially blinding if treatment is delayed. Whilst episcleritis and some forms of scleritis are innocent and harmless, others with similar presentation can rapidly become severe and destructive, leading to unnecessary visual loss. This is particularly true in young people with posterior scleritis who very often have minimal symptoms. Furthermore scleritis is, not infrequently, the presenting feature of many severe and potentially fatal systemic disorders; indeed, before effective treatment was available, one quarter of those who developed necrotising scleritis were dead within five years. It is often implied that ophthalmologists do not need to know much about the systemic disease which might affect their patients because specialist expertise is readily available. This is not the case. Even if specialist care is at hand the physician to whom the patient is referred needs to know why the patient is being referred to him or her; for instance, the physical signs which are noted by the ophthalmologist may be the first indication of a systemic vasculitis (see Fig. 12.23). If the rheumatologist is informed of this, then early and appropriate treatment will be not only be sight but life saving. Throughout the world many ophthalmologists do not have the luxury of specialist help and these doctors need to have an easy accessible summary of the important conditions that might be associated with the eye disease.
The speed of advance in the understanding of immunologically induced disease makes it impossible for any individual to encompass all the manifestations of these diseases let alone an unusual condition such as scleritis. Nevertheless the general public and their advisors no longer accept this as a reason for failure to diagnose and treat an unusual condition adequately and immediately. For these reasons Brian Hazleman has described the likely associated diseases and their management in detail in this edition.
In bringing together all those conditions which affect the sclera we have tried to produce a reference book which ideally should be in every office or clinic so that those who come into contact with scleral disease can compare the patient with the illustration and description of that condition so clearly emphasised by Margreet Hogeweg's description of the changes in leprosy.
Early and effective diagnosis means early and effective treatment so we have described all the accepted techniques of investigation. Although imaging of the eye and scleral disease is advancing rapidly the instruments for recording changes are not s6universally available in an eye department. However, if there is a chance that the patient has a posterior scleritis then imaging of the eye and orbit becomes essential to confirm the diagnosis and prevent any delay in the institution of effective therapy. In this situation the help of the radiology or other departments in the hospital must be solicited.
In these specialised areas we have again sought the help of Marie Restori, consultant physicist at Moorfields Eye Hospital, and André Romano from São Paulo, Brazil whose exquisite anterior segment OCT images could well change the way that scleritis is diagnosed in the future.
There was a life and wisdom before PubMed or Medline! Many of the concepts accepted today were proposed more than a century ago; as in so many things we stand on the shoulders of giants. The bibliography is not intended to be exhaustive but the articles cited have been largely selected because they present a new concept, a relevant observation or because they are important historical documents. There are no other texts that cover the whole of scleral disease in detail. This edition of The Sclera and Systemic Disorders fills that gap by bringing together the current information about the diagnosis, the investigation, the physiology pathology and treatment of these complex conditions.
Peter G Watson
Brian L Hazleman
Peter McCluskey
Carlos E Pavésio
January 2012
s9Contributors s11Acknowledgements
We would like to acknowledge not only the help we have received from our colleagues in many medical specialties who have ensured that the recent advances in imaging and therapy have been included in this edition, but also the immense amount of work which went into the transformation from the first to the second edition. The Herculean labours and strict discipline imposed by Margreet Hogeweg, Mark Lillicrap, David Williams, Quang Dong Nguyen, and Marie Restorie were in no small way responsible for the success of that edition of the book. Its illustrators also deserve acknowledgement: Joanna Cameron, Terry Tarrant, Jane Trotman and Philip Ball.
Much of the new material presented in this edition has come from Robert Young who has, once again, given us access to his unique knowledge of the great advances in the understanding of the constitution and function of the sclera, for which we are extremely grateful. André Romano has allowed us to use his exquisite anterior segment optical coherence tomography (OCT) images before he has had chance to publish them elsewhere. This is a significant contribution to the book for, whilst the diagnosis of scleral disease will always be a clinical one, the use and extension of this technology in the clinic will facilitate a degree of accuracy in diagnosis of scleral inflammation that is not available in any other way.
Thanks to these individuals we believe that in this edition the science, clinical understanding and the methods of treatment have been brought completely up to date, ready for the advances which are bound to occur in the future. Blindness from scleral disease, if diagnosed early, is now a thing of the past. This text will ensure that all those who are likely to come in contact with these diseases will find the information they require to restore the eye to normality and prevent any visual loss.s12