IgG4-Related Sclerosing Cholangitis
This book aims to raise awareness of IgG4-related sclerosing cholangitis among practicing physicians and to equip readers with a sound understanding of the principles of diagnosis and treatment. Clinical, serological, and histopathological features are cl
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IgG4-Related Sclerosing Cholangitis
Terumi Kamisawa • Myung-Hwan Kim Editors
IgG4-Related Sclerosing Cholangitis
Editors Terumi Kamisawa Department of Internal Medicine Tokyo Metropolitan Komagome Hospital Bunkyo-ku, Tokyo Japan
Myung-Hwan Kim Department of Internal Medicine ASAN Medical Center Seoul South Korea
ISBN 978-981-10-4547-9 ISBN 978-981-10-4548-6 (eBook) https://doi.org/10.1007/978-981-10-4548-6 Library of Congress Control Number: 2018951429 © Springer Science+Business Media Singapore 2019 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms 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 specific 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 publication. 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. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by Springer Nature, under the registered company Springer Nature Singapore Pte Ltd. The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721, Singapore
Preface
IgG4-related disease (IgG4-RD) is a fibro-inflammatory and immune-mediated condition with a tendency toward the tumefaction that mimics malignant or inflammatory disorders. IgG4-RD was first proposed as a systemic disease in 2003 following the recognition that a high percentage of patients with autoimmune pancreatitis (AIP) had extrapancreatic lesions that shared similar histopathological findings consisting of abundant infiltration of IgG4positive plasma cells and lymphocytes and fibrosis. IgG4-RD can affect almost any organ; only a single organ is clinically involved in some cases, whereas others show effects on two or more organs simultaneously or metachronously. IgG4-RD is also characterized by elevation of serum IgG4 levels and steroid responsiveness. IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-RD. Although about 60% of patients with IgG4-RD have biliary lesions in the proximal and/or distal bile ducts, the inclusion of a distal biliary stricture isolated to the intrapancreatic portion associated with AIP in the definition of
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