CLINICAL GASTROENTEROLOGY Series Editor George Y. Wu University of Connecticut Health Center, Farmington, CT, USA For further volumes: http://www.springer.com/series/7672
Elizabeth J. Carey Keith D. Lindor Editors Cholestatic Liver Disease Second Edition
Editors Elizabeth J. Carey Division of Gastroenterology and Hepatology Mayo Clinic Phoenix, AZ, USA Keith D. Lindor Mayo Clinic Phoenix, AZ, USA ISSN 2197-7399 ISSN 2197-7704 (electronic) ISBN 978-1-4939-1012-0 ISBN 978-1-4939-1013-7 (ebook) DOI 10.1007/978-1-4939-1013-7 Springer New York Heidelberg Dordrecht London Library of Congress Control Number: 2014943498 Springer Science+Business Media New York 2014 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. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher s location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. 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. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Humana Press is a brand of Springer Springer is part of Springer Science+Business Media (www.springer.com)
Preface Despite advances in the understanding and management of the cholestatic liver diseases, these disorders continue to pose clinical challenges to patients and their medical practitioners. This second edition of Cholestatic Liver Disease aims to update the reader on the classic entities of primary biliary cirrhosis and primary sclerosing cholangitis while also providing insights on more recently described problems: IgG4-associated cholangitis, cholestasis after hematologic transplantation, advances in biliary endoscopy, and new treatment options such as the farnesoid X receptor agonists. We hope that Cholestatic Liver Disease will be a valuable resource for those interested in learning about these diseases and for those who treat patients affected by the cholestatic liver disorders. The goal of this textbook is to provide scientific updates from leading experts while reviewing the clinical evaluation, management, and recent advances in the cholestatic liver diseases. It is our hope that Cholestatic Liver Disease will be a useful reference for learners and practitioners alike. Phoenix, AZ, USA Elizabeth J. Carey, M.D. Keith D. Lindor, M.D., B.S. v
Contents 1 Diagnosis of Cholestasis... 1 Andrea A. Gossard 2 Drug-Induced Cholestasis... 13 Einar S. Björnsson 3 Primary Biliary Cirrhosis... 33 Marina G. Silveira 4 Primary Sclerosing Cholangitis... 51 Meredith Borman and Bertus Eksteen 5 IgG4 Related Cholangitis... 87 Said Al Mamari, Emma L. Culver, and Roger W. Chapman 6 Rare Causes of Cholestasis... 111 Tamir Miloh and Ozlem Pinar Bulut 7 Cholestatic Variants of Viral Disease and Alcohol... 127 Mary A. Atia and Bashar Aqel 8 Cholestasis Related to Systemic Disorders... 143 Anitha Yadav and Elizabeth J. Carey 9 Complications of Cholestasis... 163 Abrar Ahmed and Marlyn J. Mayo 10 Cholestasis Post-Liver Transplantation... 189 Denise M. Harnois and Kymberly D.S. Watt vii
viii Contents 11 Cholestasis in the Hospitalized Patient... 201 Anne M. Larson 12 Endoscopic Management of Biliary Obstruction... 229 James H. Tabibian and Todd H. Baron Index... 253
Contributors Abrar Ahmed, M.D. Division of Digestive & Liver Diseases, University of Texas Southwestern Medical Center, Dallas, TX, USA Bashar Aqel, M.D. Division of Gastroenterology & Hepatology, Mayo Clinic Arizona, Scottsdale, AZ, USA Mary A. Atia, M.D. Division of Gastroenterology & Hepatology, Mayo Clinic Arizona, Scottsdale, AZ, USA Todd H. Baron, M.D. Division of Gastroenterology & Hepatology, Advanced Therapeutic Endoscopy, Department of Medicine, University of North Carolina, Chapel Hill, NC, USA Einar S. Björnsson, M.D., Ph.D. Gastroenterology and Hepatology, Landspitali University Hospital, Reykjavik, Iceland Meredith Borman, M.D., Ph.D., F.R.C.P.C. Snyder Institute for Chronic Diseases, University of Calgary, Calgary, AB, Canada Division of Hepatology and Gastroenterology, University of Calgary, Calgary, AB, Canada Ozlem Pinar Bulut, M.D. Pediatric Gastroenterology, Hepatology & Nutrition, Phoenix Children s Hospital, Phoenix, AZ, USA Elizabeth J. Carey, M.D. Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, AZ, USA Roger W. Chapman, M.D., F.R.C.P. Translational Gastroenterology, Oxford University Hospital Trust, Oxford, UK Emma L. Culver, B.Sc. (Hons), M.B.Ch.B., M.R.C.P. Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK Bertus Eksteen, M.B.Ch.B., Ph.D., F.R.C.P. Medicine, Division of Hepatology and Gastroenterology, Foothills Hospital, University of Calgary, Calgary, AB, Canada ix
x Contributors Andrea A. Gossard, M.S., C.N.P. Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA Denise M. Harnois, D.O. Department of Transplant, Mayo Clinic, Jacksonville, FL, USA Anne M. Larson, M.D. Swedish Liver Center, Swedish Health Systems, Seattle, WA, USA Said Al Mamari, M.D., M.R.C.P. (UK) Liver Unit, Sultan Qaboos Hospital, Salalah, Dhofar, Oman Marlyn J. Mayo, M.D. Division of Digestive & Liver Diseases, University of Texas Southwestern Medical Center, Dallas, TX, USA Tamir Miloh, M.D. Pediatrics Department, Phoenix Children s Hospital, Phoenix, AZ, USA Marina G. Silveira, M.D. Gastroenterology and Hepatology, Cleveland, OH, USA James H. Tabibian, M.D. Division of Gastroenterology & Hepatology, Department of Medicine, Mayo Clinic, Rochester, MN, USA Kymberly D.S. Watt, M.D. Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA Anitha Yadav, M.D. Transplant Hepatology, Mayo Clinic Arizona, Phoenix, AZ, USA