O R L OXFORD RHEUMATOLOGY LIBRARY Systemic Lupus Erythematosus O R L OXFORD RHEUMATOLOGY LIBRARY Systemic Lupus Erythematosus Edited by Professor Caroline Gordon Professor of Rheumatology, College of Medical and Dental Sciences, University of Birmingham, UK Professor David Isenberg Diamond Jubilee Arthritis Research UK Professor of Rheumatology, University College London, London, UK 1 3 Great Clarendon Street, Oxford, OX2 6DP, United Kingdom Oxford University Press is a department of the University of Oxford. It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries © Oxford University Press 206 The moral rights of the authors have been asserted First Edition published in 206 Impression: All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, 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, by licence or under terms agreed with the appropriate reprographics rights organization. Enquiries concerning reproduction outside the scope of the above should be sent to the Rights Department, Oxford University Press, at the address above You must not circulate this work in any other form and you must impose this same condition on any acquirer Published in the United States of America by Oxford University Press 98 Madison Avenue, New York, NY 006, United States of America British Library Cataloguing in Publication Data Data available Library of Congress Control Number: 20595530 ISBN 978–0–9–87398–0 Printed in Great Britain by Clays Ltd, St Ives plc 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 clinical procedures with the most up-to-date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this work. Except where otherwise stated, drug dosages and recommendations are for the non-pregnant adult who is not breast-feeding Links to third party websites are provided by Oxford in good faith and for information only. Oxford disclaims any responsibility for the materials contained in any third party website referenced in this work. Contents Contributors vii Abbreviations ix History of systemic lupus erythematosus Manuel F. Ugarte-Gil and Graciela S. Alarcón 2 Aetiopathogenesis of systemic lupus erythematosus 7 Peter Lloyd, Sarah Doaty, and Bevra H. Hahn 3 Epidemiology of systemic lupus erythematosus 23 Caroline Gordon and S. Sam Lim 4 Clinical features of systemic lupus erythematosus 45 Ben Rhodes and Caroline Gordon v 5 Laboratory tests and investigations 63 Anthony Isaacs and David Isenberg 6 Conventional treatments in systemic lupus erythematosus 79 Anisur Rahman 7 Biologic therapies in systemic lupus erythematosus 89 Maria Mouyis and David Isenberg 8 Juvenile systemic lupus erythematosus 03 Claire Louise Murphy, Yiannis Ioannou, and Nicola Ambrose 9 Management of special situations in systemic lupus erythematosus 7 April Jorge and Rosalind Ramsey-Goldman Index 35 Contributors Dr Graciela S. Alarcón Dr S. Sam Lim Division of Clinical Immunology and Department of Medicine, Division Rheumatology, University of Alabama of Rheumatology, Emory University, at Birmingham, AL, USA Atlanta, GA, USA Dr Nicola Ambrose Dr Peter Lloyd Adolescent and Adult Rheumatology University of California Los Angeles, Unit, University College London Los Angeles, CA, USA Hospitals, London, UK Dr Maria Mouyis Dr Sarah Doaty Centre for Rheumatology, University University of California Los Angeles, College Hospital, London, UK Los Angeles, CA, USA Dr Claire Louise Murphy Professor Caroline Gordon Adolescent and Adult Rheumatology vii College of Medical and Dental Sciences, Unit, University College London University of Birmingham, UK Hospitals, London, UK Professor Bevra H. Hahn Professor Anisur Rahman University of California Los Angeles, Centre for Rheumatology, University Los Angeles, CA, USA College London, London, UK Dr Yiannis Ioannou Professor Rosalind Ramsey-Goldman Adolescent and Adult Rheumatology Unit, University College London Northwestern University Feinberg Hospitals, London, UK School of Medicine, Chicago, IL, USA Dr Anthony Isaacs Dr Ben Rhodes Centre for Rheumatology, University Department of Rheumatology, College Hospital, London, UK Queen Elizabeth Hospital Birmingham, Birmingham, UK Professor David Isenberg Dr Manuel F. Ugarte-Gil Centre for Rheumatology, University College London, London, UK Hospital Guillermo Almenara Irigoyen, EsSalud, Lima, Peru Dr April Jorge Northwestern University Feinberg School of Medicine, Chicago, IL, USA Abbreviations ACR American College of Rheumatology 6-MP 6-mercaptopurine ACC American College of Cardiology ACIP Advisory Committee on Immunization Practices (USA) AHA American Heart Association ALMS Aspreva Lupus Management Study ALP alkaline phosphatase ALT alanine amino transferase ANA antinuclear antibodies anti-Sm anti-Smith antibodies apoA- apolipoprotein A- APPLE Atherosclerosis Prevention in Pediatric Lupus Erythematosus ix APRIL a proliferation-inducing ligand APS antiphospholipid syndrome APTT activated partial thromboplastin time AST aspartate aminotransferase AutoAb autoantibody BAFF B cell activating factor BAL bronchoalveolar lavage BCMA B cell maturation antigen BICLA BILAG-based composite lupus assessment BILAG British Isles Lupus Assessment Group BLyS B lymphocyte stimulator inhibitor BMD bone mineral density BMI body mass index Breg B-regulatory (cell) CAPS catastrophic antiphospholipid syndrome CCP cyclic citrullinated peptide CI confidence interval CIMT carotid intima-media thickness CIN cervical intraepithelial neoplasia CK creatine kinase CKD chronic kidney disease CLASI cutaneous lupus erythematosus disease area and severity index