Primary Sclerosing Cholangitis

Primary Sclerosing Cholangitis

Lisa M. Forman Editor Primary Sclerosing Cholangitis Current Understanding, Management, and Future Developments

Editor Lisa M. Forman Department of Gastroenterology- Hepatology University of Colorado 1635 Aurora Court, B-154 Aurora, CO 80045 USA ISBN 978-3-319-40906-1 ISBN 978-3-319-40908-5 (ebook) DOI 10.1007/978-3-319-40908-5 Library of Congress Control Number: 2016959029 Springer International Publishing Switzerland 2017 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. Printed on acid-free paper This Springer imprint is published by Springer Nature

Preface Primary Sclerosing Cholangitis: Current Understanding, Management, and Future Developments grew out of a need I perceived within the fields of hepatology and liver transplantation. Primary sclerosing cholangitis (PSC) is a rare disease, with an incidence ranging from 0.04 to 1.30 per 1000,000 person years. Patients with PSC have variable presentations and there is significant variability in progression and prognosis. As a clinician, it has been frustrating that up until recently there has been little we could offer patients with PSC, other than liver transplantation, and little that we knew about its pathogenesis. In the liver community, much effort has been made into finding a cure for hepatitis C, a much more common chronic liver disease. With the development of successful antiviral therapies for hepatitis C, there has been a renewed interest into potential treatments of cholestatic diseases. Indeed, it is an exciting time for PSC. Great work has been done to further clarify the role of genetics, immunology, and the microbiome with regard to the development and progression of PSC. Although liver transplantation remains the definitive treatment for advanced PSC, there are multiple new agents that are in clinical trials which will hopefully halt and even improve the fibrosis and subsequent complications associated with PSC. Endoscopic techniques have vastly improved over the past decade, and cholangiocarcinoma, a once universally fatal disease, can now be cured with liver transplantation. In this book, recognized international experts in cholestatic diseases review the epidemiology, pathophysiology, current and future management of PSC, its variants, and associated complications. Up-to-date data regarding genetics, cholangiocyte biology, and immunology of PSC are presented. I hope that this publication will be of interest and utility to the medical and scientific community at large, with the ultimate goal of improving our understanding and treatment of this orphan disease. Aurora, CO, USA Lisa M. Forman v

Acknowledgments I appreciate and gratefully acknowledge the dedicated, hardworking members of the Liver Team at the University of Colorado Denver: Gregory Everson, MD; James Burton, MD; Hugo Rosen, MD; Amanda Wieland, MD; Michael Kriss, MD; Igal Kam, MD; Thomas Bak, MD; Michael Wachs, MD; Trevor Nydam, MD; Kendra Conzen, MD; Catherine Ray, RN, BSN, MA; Maura McCourt, RN; Holley Reitz, RN; Kaitlyn Paus, RN; Catherine Behnke, RN; Mindy Stewart, RN; Lori McCoy, RN; Amy Huntsman, RN; Jaime Cisek, RN; Sarah Tise, PA; Lindsay Pratt, PA; Deidre Ellis, Administrator of the Transplant Center, Tracy Steinberg, RN, MS, CCTC; Lana Schoch, RN; Lauren Basham, RN; Amanda Kober, RN; Andrea Chester, RN, Kathleen Orban, RN, CCTC; Danica Farrington, RN; Jenny Sanderson, LCSW; John Scheid, LSW; and finally, my research team-jennifer DeSanto, RN; Halley Isberg, BA; and Allison Pabisch BS. I am lucky to work with such a dedicated group of people who are committed to the care and management of patients with liver disease. Special thanks to Ricky Safer and her wonderful advocacy group, PSC Partners Seeking a Cure. Most importantly, I wish to acknowledge all my patients with PSC and their families whose stories have inspired me, who have put their trust in me, and who have challenged me to become a better physician. Finally, this book would not have been possible without the efforts of the authors I appreciate their enthusiasm, critical thinking, and willingness to participate in this project. vii

Contents 1 Epidemiology and Natural History of Primary Sclerosing Cholangitis................................... 1 Christopher L. Bowlus 2 Malignancy and Primary Sclerosing Cholangitis: Cholangiocarcinoma, Hepatocellular Carcinoma, and Gallbladder Carcinoma............................. 13 Larissa Muething and James R. Burton Jr. 3 Primary Sclerosing Cholangitis-Associated Inflammatory Bowel Disease............................. 29 Blair Fennimore, Emilie H. Regner, and Mark E. Gerich 4 Overlap Syndromes of Primary Sclerosing Cholangitis....... 41 Albert J. Czaja 5 IgG4-Related Sclerosing Cholangitis...................... 59 Tamsin Cargill, Emma L. Culver, and Roger W. Chapman 6 Pediatric Primary Sclerosing Cholangitis................... 73 Dania Molla-Hosseini and Cara L. Mack 7 Cholangiocyte Biology.................................. 83 Lorena Loarca, María José Lorenzo Pisarello, Leslie Morton, Bing Q. Huang, Steven O Hara, Patrick Splinter, and Nicholas LaRusso 8 Genetics of Primary Sclerosing Cholangitis................. 99 Tom Hemming Karlsen and Gideon M. Hirschfield 9 Immunology of Primary Sclerosing Cholangitis............ 111 John M. Vierling 10 Pruritus in Primary Sclerosing Cholangitis: New Insights into Cause and Treatment................... 133 Mark G. Swain 11 Ursodeoxycholic Acid Treatment in Primary Sclerosing Cholangitis................................. 145 James H. Tabibian and Keith D. Lindor ix

x Contents 12 Future Therapies for Primary Sclerosing Cholangitis....... 153 Craig Lammert and Raj Vuppalanchi 13 Noninvasive Imaging of Primary Sclerosing Cholangitis: A Radiologic Perspective.................... 167 Paul D. Russ 14 Endoscopic Evaluation and Management of Primary Sclerosing Cholangitis................................. 181 Hazem T. Hammad and Raj J. Shah 15 Percutaneous Biliary Intervention in Patients with Primary Sclerosing Cholangitis...................... 195 Thor Johnson and Janette D. Durham 16 Liver Transplantation for PSC.......................... 203 Kendra Conzen and Trevor L. Nydam 17 Recurrent Primary Sclerosing Cholangitis After Liver Transplantation.................................. 211 James F. Trotter and Mark G. Swain Index.................................................... 219

Contributors Christopher L. Bowlus, MD Division of Gastroenterology and Hepatology, University of California Davis, Sacramento, CA, USA James R. Burton Jr. University of Colorado Hospital, Anschutz Outpatient Pavilion, Aurora, CO, USA Tamsin Cargill, MBBS, BSc(Hons) Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK Nuffield Department of Medicine, University of Oxford, Oxford, UK Roger W. Chapman, MD, FRCP, FAASLD Nuffield Department of Medicine, University of Oxford, Oxford, UK Nuffield Department of Medicine, Translational Gastroenterology Unit, Level 5, John Radcliffe Hospital, Oxford, UK Kendra Conzen, MD Division of Transplant Surgery, Department of Surgery, University of Colorado School of Medicine, Aurora, CO, USA Emma L. Culver, MBChB, BSc(Hons), MRCP, PhD Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK Nuffield Department of Medicine, University of Oxford, Oxford, UK Albert J. Czaja, MD Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN, USA Janette D. Durham, MD Division of Interventional Radiology, University of Colorado School of Medicine, Aurora, CO, USA Blair Fennimore, MD Assistant Professor of Medicine, Division of Gastroenterology and Hepatology, University of Colorado School of Medicine, 12700 E. 19th Ave. MS B-146, Aurora, CO 80045, USA Mark E. Gerich, MD Assistant Professor of Medicine, Division of Gastroenterology and Hepatology, University of Colorado School of Medicine, 12700 E. 19th Ave. MS B-146, Aurora, CO 80045, USA Hazem T. Hammad, MD Section of Interventional Endoscopy, Division of Gastroenterology and Hepatology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA xi

xii Contributors Gideon M. Hirschfield Centre for Liver Research, NIHR Birmingham Liver Biomedical Research Unit, University of Birmingham, Birmingham, UK Bing Q. Huang Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA Thor Johnson, MD Division of Interventional Radiology, University of Colorado School of Medicine, Aurora, CO, USA Tom Hemming Karlsen Norwegian PSC Research Center, Division of Cancer Medicine, Surgery and Transplantation, Department of Transplantation Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway Section of Gastroenterology, Division of Cancer Medicine, Surgery and Transplantation, Department of Transplantation Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway Research Institute of Internal Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital, Oslo, Norway Craig Lammert, MD Division of Gastroenterology and Hepatology, Indiana University School of Medicine, Indianapolis, IN, USA Nicholas LaRusso Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA Keith D. Lindor, MD College of Health Solutions, Arizona State University, Phoenix, AZ, USA Lorena Loarca Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA Cara L. Mack, MD Section of Pediatric Gastroenterology, Hepatology and Nutrition, Children s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA Dania Molla-Hosseini, MD Section of Pediatric Gastroenterology, Hepatology and Nutrition, Children s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, USA Leslie Morton Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA Larissa Muething University of Colorado Hospital, Anschutz Outpatient Pavilion, Aurora, CO, USA

Contributors xiii Trevor L. Nydam, MD Division of Transplant Surgery, Department of Surgery, University of Colorado School of Medicine, Aurora, CO, USA Steven O Hara Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA María José Lorenzo Pisarello Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA Emilie H. Regner, MD Assistant Professor of Medicine, Division of Gastroenterology and Hepatology, University of Colorado School of Medicine, 12700 E. 19th Ave. MS B-146, Aurora, CO 80045, USA Paul D. Russ, MD, FACR Department of Radiology, University of Colorado Hospital, University of Colorado Denver, Anschutz Medical Campus, Aurora, CO, USA Raj J. Shah, MD, FASGE, AGAF Section of Interventional Endoscopy, Division of Gastroenterology and Hepatology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA Patrick Splinter Division of Gastroenterology and Hepatology, Mayo Clinic Center for Signaling in Gastroenterology, Mayo Clinic, Rochester, MN, USA Mark G. Swain, MD, MSc, FRCPC, FAASLD Cal Wenzel Family Foundation Chair in Hepatology, Professor of Medicine, Head, Division of Gastroenterology and Hepatology, University of Calgary, Section Head, Section of Gastroenterology and Hepatology, Calgary Zone, Alberta Health Services, TRW Building, 3280 Hospital Dr., NW, Calgary, Alberta, Canada T2N 4N1 James H. Tabibian, MD, PhD Division of Gastroenterology, University of Pennsylvania, Philadelphia, PA, USA Center for Endoscopic Education, Innovation, and Training, University of Pennsylvania, Philadelphia, PA, USA James F. Trotter, MD Director, Hepatology, Baylor University Medical Center, 3410 Worth Street #860, Dallas, TX 75246, USA John M. Vierling, MD, FACP, FAASLD Division of Abdominal Transplantation, Department of Medicine and Surgery, Baylor College of Medicine, Baylor-St. Luke s Medical Center, Houston, TX, USA Raj Vuppalanchi, MD Division of Gastroenterology and Hepatology, Indiana University School of Medicine, Indianapolis, IN, USA