Hughes Syndrome: A Patient’s Guide PDF
Preview Hughes Syndrome: A Patient’s Guide
Graham Hughes Hughes Syndrome A Patient’s Guide Second Edition 123 Hughes Syndrome Graham Hughes Hughes Syndrome A Patient’s Guide Second Edition Graham Hughes London Bridge Hospital London Lupus Center London United Kingdom ISBN 978-3-319-31028-2 ISBN 978-3-319-31029-9 (eBook) DOI 10.1007/978-3-319-31029-9 Library of Congress Control Number: 2016938647 © Springer International Publishing Switzerland 2016 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduc- tion on microfi lms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specifi c statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of pub- lication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper This Springer imprint is published by Springer Nature The registered company is Springer International Publishing AG Switzerland Foreword T he antiphospholipid or Hughes syndrome is not invented but is a biological aberration. In many ways, the description of a syndrome is truly a scientific effort because it is based on the accrual of data and organizing that data into something that describes an event in a large group of people. This can be a difficult task, since one has to take scientific data from a group of people with different complaints. While many such syndromes or diseases are new and have never been seen before, most have been around for years and are just now coming to light because of science. One could draw some parallels to the well-known disease lupus that was originally thought to be quite rare and of which little was known. Now the same could be said for people with the antiphospholipid syndrome which is the subject of this book. T he author of this book, Professor Graham Hughes and his colleagues described something in medicine for the first time. A very important disease, which affects millions of people around the world, was recognized early by Dr. Hughes. He saw something different in both the clinic and the laboratory for the first time, and he was the first to call this condition the antiphospholipid syndrome. The antiphospholipid syndrome affects the blood and its ability to clot. The blood from patients with this illness clots too quickly, and the consequences of this include strokes and heart attacks because of blood clots that go to places like the heart or brain. Fortunately, this does not hap- pen to everyone and the disease is easily treated with blood thinners. The overall condition is caused by the same kind of antibodies or proteins directed against self; in essence a classi- cal autoimmune disease. v vi Foreword No doubt, it will take many years to understand the why, how, and what of this new illness, but it must be explained to the people who have it in clear fashion. My patients ask me about this condition all the time. While an easy explanation you could say is not possible, patients deserve an explanation because they have to take the medicine, understand the pre- cautions and the prognoses, and experience the results of our therapy and our goals concerning treatment. I n this tome, Dr. Hughes considers you the patient. He has followed Louis Pasteur’s aphorism that “chance favors the prepared mind.” Indeed, it does since the discovery of these particular antibodies allows us to understand a complex ill- ness and gain insight into heart disease, stroke, and “hard- ened arteries.” Now you have the opportunity to understand this unfortunate but interesting biological problem. Read this work to understand antiphospholipid disease and the problems of autoimmunity through Dr. Hughes’ mas- terful efforts of description. These are the diseases of our time that piece by piece provide a better understanding of our immune systems. New York, NY, USA Robert G. Lahita , MD, PhD Preface to the Second Edition In the 15 years since the publication of this patients’ guide, so much has happened. From its small beginnings in 1983, Hughes syndrome/APS has become an important chapter in medicine – not only in the world of rheumatology but also in neurology, obstetrics, cardiology and beyond. T he number of clinical and research publications has increased year on year and the international conferences on antiphospholipid antibodies are now annual, and growing. T he medical world has come to realise that Hughes syn- drome is ‘more than’ thrombosis and miscarriage, and symp- toms and features include memory loss, seizures, ‘autonomic’ nerve problems, sleep disturbance, angina, balance problems and stillbirth. Governments and medical authorities are now recognising the importance of APS in, for example, stroke, angina, preg- nancy loss and migraine. For the patient, however, the jour- ney is often tortuous with referrals to different specialities and, in many cases, a lack of detailed knowledge of the syndrome. I believe that, at last, this is changing. There is a growing realisation that the syndrome is common and that diagnosis can result in striking therapeutic success. London, UK Graham Hughes January 2016 vii Contents 1 Sticky Blood: The Disease Is Common. . . . . . . . . . . . . 1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 For the Doctor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2 For the Patient . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 2 Main Clinical Features . . . . . . . . . . . . . . . . . . . . . . . . . . 7 What Are the Signs? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 What Triggers the Thrombosis?. . . . . . . . . . . . . . . . . . . . . 9 Positive Blood Tests. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10 3 Clotting in Veins. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 Limb Thrombosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13 4 Stroke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 5 Memory Loss and “Alzheimers”. . . . . . . . . . . . . . . . . . 25 6 Headaches, Migraine and Fits . . . . . . . . . . . . . . . . . . . . 29 Headaches and Migraine . . . . . . . . . . . . . . . . . . . . . . . . . . 29 Fits and Seizures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 30 Movement Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31 7 The Spinal Cord and “Multiple Sclerosis”. . . . . . . . . . 33 Spinal Cord Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 The Eye . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 Balance. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34 Multiple Sclerosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35 ix
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