313
Autoimmune Liver Disease
Management and Clinical Practice
Edited by
James Neuberger
University Hospital BirminghamQueen Elizabeth HospitalEdgbastonBirmingham, UK
Gideon M. Hirschfield
University of TorontoTorontoOntario, Canada
This edition first published 2020 © 2020 by John Wiley & Sons Ltd
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, electronic, mechanical, photocopying, recording or otherwise, except as permitted by law. Advice on how to obtain permission to reuse material from this title is available at http://www.wiley.com/go/permissions.
The right of James Neuberger, Gideon M. Hirschfield to be identified as the author of editorial work has been asserted in accordance with law.
Registered Office(s)
John Wiley & Sons, Inc., 111 River Street, Hoboken, NJ 07030, USA
John Wiley & Sons Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK
Editorial Office 9600 Garsington Road, Oxford, OX4 2DQ, UK
For details of our global editorial offices, customer services, and more information about Wiley products visit us at www.wiley.com.
Wiley also publishes its books in a variety of electronic formats and by print‐on‐demand. Some content that appears in standard print versions of this book may not be available in other formats.
Limit of Liability/Disclaimer of Warranty The contents of this work are intended to further general scientific research, understanding, and discussion only and are not intended and should not be relied upon as recommending or promoting scientific method, diagnosis, or treatment by physicians for any particular patient. In view of ongoing research, equipment modifications, changes in governmental regulations, and the constant flow of information relating to the use of medicines, equipment, and devices, the reader is urged to review and evaluate the information provided in the package insert or instructions for each medicine, equipment, or device for, among other things, any changes in the instructions or indication of usage and for added warnings and precautions. While the publisher and authors have used their best efforts in preparing this work, they make no representations or warranties with respect to the accuracy or completeness of the contents of this work and specifically disclaim all warranties, including without limitation any implied warranties of merchantability or fitness for a particular purpose. No warranty may be created or extended by sales representatives, written sales materials or promotional statements for this work. The fact that an organization, website, or product is referred to in this work as a citation and/or potential source of further information does not mean that the publisher and authors endorse the information or services the organization, website, or product may provide or recommendations it may make. This work is sold with the understanding that the publisher is not engaged in rendering professional services. The advice and strategies contained herein may not be suitable for your situation. You should consult with a specialist where appropriate. Further, readers should be aware that websites listed in this work may have changed or disappeared between when this work was written and when it is read. Neither the publisher nor authors shall be liable for any loss of profit or any other commercial damages, including but not limited to special, incidental, consequential, or other damages.
Library of Congress Cataloging‐in‐Publication Data
Names: Neuberger, James, editor. | Hirschfield, Gideon M., editor.
Title: Autoimmune liver disease : management and clinical practice / edited by James Neuberger, University Hospital Birmingham, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK, Gideon M. Hirschfield, University of Toronto, Toronto, Ontario, Canada.
Description: Hoboken, NJ : Wiley‐Blackwell, 2020. | Includes bibliographical references and index.
Identifiers: LCCN 2019051484 (print) | LCCN 2019051485 (ebook) | ISBN 9781119532606 (hardback) | ISBN 9781119532613 (adobe pdf) | ISBN 9781119532620 (epub)
Subjects: LCSH: Liver–Diseases–Immunological aspects. | Autoimmune diseases.
Classification: LCC RC846 .A87 2020 (print) | LCC RC846 (ebook) | DDC 616.3/62079–dc23
LC record available at https://lccn.loc.gov/2019051484 LC ebook record available at https://lccn.loc.gov/2019051485
Cover Design: Wiley
Cover Images: Immunofluorescence Microscopy © Beano5/Getty Images, Prostate Gland © BeholdingEye/Getty Images, PET CT scan © springsky/Shutterstock, Human organ liver © Marochkina Anastasiia/Shutterstock
Preface
Over the last three decades there have been many advances in the understanding of autoimmune liver disease (AILD). The advances in understanding the pathogenesis of these diseases is starting to lead to more specific therapies both for slowing or arresting progression and for treatment of the symptoms. Many people with AILD see delays in diagnosis and are misdiagnosed; treatments tend at best to control progression rather than induce resolution. Symptoms are often not treated because of either poor knowledge or inadequate therapies. Liver transplantation, a highly effective therapy for those with end‐stage disease, has its inherent risks and complications and is really a reflection of a failure to treat the original disease. Recurrence of AILD after transplantation, which manifests despite sufficient immunosuppression to prevent allograft rejection and in a foreign HLA milieu, must provide clues to some of the processes that lead to AILD but the lessons remain obscure.
We have been pleased to edit this volume which we hope will not only provide a balanced and comprehensive background to the pathological and clinical aspects of AILD but also stimulate further research to provide effective therapies. We are indebted to the authors who have worked so hard to summarize complex fields. They represent some of the world leaders in this area and we are grateful to them for their time. We know there is some overlap and minor conflicts between chapters but we have retained these as we would like chapters to be comprehensive and self‐contained.
We would also like to thank Deirdre Barry, Yogalakshmi Mohanakrishnan and Baskar Anandraj at Wiley for their support, patience and editorial skills and, most importantly, our families who have been patient during this time.
Finally, we thank you for reading this volume and hope you find it stimulating and provoking. Whilst autoimmune liver diseases are rare, they are impactful, and every opportunity to raise awareness, standards and knowledge is an opportunity for better patient care.
James Neuberger
Gideon M. Hirschfield
Contributors
Eleanor Barnes PhD, FRCP, F Med Sci Professor of Hepatology and Experimental Medicine, Translational Gastroenterology Unit, Nuffield Department of Medicine, University of Oxford; and Oxford