Inflammatory Bowel Disease Diagnosis and Therapeutics Humana Press Humana Press Edited by Russell D. Cohen, MD Inflammatory Bowel Disease Diagnosis and Therapeutics Edited by Russell D. Cohen, MD INFLAMMATORY BOWEL DISEASE CLINICAL GASTROENTEROLOGY George Y. Wu, SERIES EDITOR Acute Gastrointestinal Bleeding: Diagnosis and Treatment, edited by Karen E. Kim, 2003 Inflammatory Bowel Disease: Diagnosis and Therapeutics, edited by Russell D. Cohen, 2003 An Internist's Illustrated Guide to Gastrointestinal Surgery, edited by George Y. Wu, Khalid Aziz, Lily H. Fiduccia, and Giles F. Whalen, 2003 Chronic Viral Hepatitis: Diagnosis and Therapeutics, edited by Raymond S. Koff and George Y. Wu, 2001. Diseases of the Gastroesophageal Mucosa: The Acid-Related Disorders, edited by James W. Freston, 2001. INFLAMMATORY BOWEL DISEASE DIAGNOSIS AND THERAPEUTICS HUMANA PRESS TOTOWA, NEW JERSEY Edited by RUSSELL D. COHEN, MD The University of Chicago Medical Center, Chicago, IL © 2003 Humana Press Inc. 999 Riverview Drive, Suite 208 Totowa, New Jersey 07512 humanapress.com For additional copies, pricing for bulk purchases, and/or information about other Humana titles, contact Humana at the above address or at any of the following numbers: Tel: 973-256-1699; Fax: 973-256-8341; E-mail: humana@humanapr.com or visit our Website at humanapress.com All rights reserved. No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording, or otherwise without written permission from the Publisher. All articles, comments, opinions, conclusions, or recommendations are those of the author(s), and do not necessarily reflect the views of the publisher. Due diligence has been taken by the publishers, editors, and authors of this book to ensure the accuracy of the information published and to describe generally accepted practices. The contributors herein have carefully checked to ensure that the drug selections and dosages set forth in this text are accurate in accord with the standards accepted at the time of publication. Notwithstanding, as new research, changes in government regu- lations, and knowledge from clinical experience relating to drug therapy and drug reactions constantly occurs, the reader is advised to check the product information provided by the manufacturer of each drug for any change in dosages or for additional warnings and contraindications. This is of utmost importance when the recom- mended drug herein is a new or infrequently used drug. It is the responsibility of the health care provider to ascertain the Food and Drug Administration status of each drug or device used in their clinical practice. The publisher, editors, and authors are not responsible for errors or omissions or for any consequences from the application of the information presented in this book and make no warranty, express or implied, with respect to the contents in this publication. This publication is printed on acid-free paper. ∞ ANSI Z39.48-1984 (American National Standards Institute) Permanence of Paper for Printed Library Materials. Production Editor: Mark J. Breaugh. Cover Illustration: (Background): Single-contrast lower GI study demonstrating a shortened featureless “lead pipe” colon typical of chronic UC. See Fig. 4 on p. 96; (Left): Large, irregular Crohn's disease ulcer of the colon. Courtesy of Dr. Russell D. Cohen; (Center): Crohn’s ileitis. See Fig. 6 on p. 336; (Right): Large Pyoderma gangrenosum affecting the anterior tibial surface on the lower extremity in a patient with Crohn's disease. Courtesy of Dr. Russell D. Cohen. Cover design by Patricia F. Cleary. Photocopy Authorization Policy: Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by Humana Press, provided that the base fee of US $20.00 per copy, is paid directly to the Copyright Clearance Center at 222 Rosewood Drive, Danvers, MA 01923. For those organizations that have been granted a photocopy license from the CCC, a separate system of payment has been arranged and is acceptable to Humana Press Inc. The fee code for users of the Transactional Reporting Service is: [0-89603-909-9/03 $20.00]. Printed in the United States of America. 10 9 8 7 6 5 4 3 2 1 Library of Congress Cataloging-in-Publication Data Inflammatory Bowel Disease : diagnosis and therapeutics / edited by Russell D. Cohen. p. cm. (Clinical gastroenterology) Includes bibliographical references and index. ISBN 0-89603-909-9 (alk. paper): 1-59259-311-9 (ebook) 1. Inflammatory bowel diseases. I. Cohen, Russell D. II. Series. RC862.I53 I527 2003 616.3'44 dc21 2002032874 Disclaimer: Some images in the original version of this book are not available for inclusion in the eBook. v PREFACE One of the most vivid memories from my medical school training was seeing my first surgical operation on a patient with Crohn’s disease. The senior surgeon at Mount Sinai Hospital in New York City, the same institution at which Burrill Crohn, Leon Ginzburg, and Gordon Oppenheimer had first described the disease “terminal ileitis,” had un- doubtedly done countless operations on patients with inflammatory bowel disease in the past. Yet as we both gazed down into the patient’s open abdomen, at the “creeping fat” that seemed to be wrapping its sticky fingers around the young man’s intestines, he stated, “this is the mys- tery of Crohn’s disease—no two patients are ever the same.” What is it about the inflammatory bowel diseases, Crohn’s disease, and ulcerative colitis, that we find so intriguing? Is it the young age of the patients, many who are younger than even the medical students at- tending to them? Or is it the elusive etiology, the theory of a “mystery organism” that has yet to be identified? Perhaps it is the familial pattern of disease, where many patients have relatives with similar diseases, yet in some instances only one of a pair of identical twins is affected. Regardless of the cause, these chronic diseases with a typically early age of onset, result in a long-term commitment of the patient, their fami- lies, friends, health care providers, researchers, employers, and even health care insurers and other health-related industries. Each of these groups have their own areas of interest and understanding of these dis- eases, with a need to know particular details, as well as how to find out additional information. It is precisely with this in mind that we set out to write Inflammatory Bowel Disease: Diagnosis and Therapeutics. Our goal is to provide a comprehensive but concise overview of the myriad of issues surround- ing the inflammatory bowel diseases, written in a language targeted to those both within and outside of the medical community. The intent of many of the chapters is also to provide resources on how to get more information on a particular topic, with web page addresses, phone num- bers, and addresses of various sources Who should read this book? Patients, their friends, and families, will find answers to many of the questions that they have about the disease. vi Preface Physicians, surgeons, nurses, ostomy specialists, social workers, phar- macists, and other medical professionals will find the information help- ful in treating their patients and providing answers to many of the ques- tions that are often thrown to them. Laboratory and clinical scientists will be provided with state-of-the-art information on the diseases and future directions of research. Members of the health care, insurance, and pharmaceutical industries will find a comprehensive review of the eco- nomics of these diseases, which should be valuable in the development of sensible health care policies toward these patients. And finally, stu- dents of the medical, biological, and social sciences should read this book, as they hold the promise for future advances in our understanding and treatment of inflammatory bowel disease. I would like to thank Centocor Inc., Procter & Gamble Pharmaceuti- cals, Shire US Inc., Prometheus Laboratories Inc., and Salix Pharma- ceuticals, who have made it possible to include color photographs in this book. Russell D. Cohen, MD CONTENTS vii Preface v List of Contributors ix 1 Inflammatory Bowel Disease (Ulcerative Colitis, Crohn's Disease): Early History, Current Concepts, and 21st Century Directions 1 Joseph B. Kirsner 2 Epidemiology of Inflammatory Bowel Disease 17 Charles N. Bernstein and James F. Blanchard 3 Etiology and Pathogenesis of Inflammatory Bowel Disease 33 James J. Farrell and Bruce E. Sands 4 Genetics of Inflammatory Bowel Disease 65 Judy Cho 5 Presentation and Diagnosis of Inflammatory Bowel Disease 75 Themistocles Dassopoulos and Stephen Hanauer 6 Radiological Findings in Inflammatory Bowel Disease 91 Peter M. MacEneaney and Arunas E. Gasparaitis 7 Inflammatory Bowel Disease Markers 107 Marla C. Dubinsky and Stephan R. Targan 8 Medical Therapy of Inflammatory Bowel Disease 131 Todd E. H. Hecht, Chinyu G. Su, and Gary R. Lichtenstein 9 Surgical Management of Inflammatory Bowel Disease 157 Roger D. Hurst 10 Ostomy Care 201 Janice C. Colwell 11 Inflammatory Bowel Disease in Children and Adolescents 215 Ranjana Gokhale and Barbara S. Kirschner 12 Nutritional/Metabolic Issues in the Management of Inflammatory Bowel Disease 231 Jeanette Newton Keith and Michael Sitrin 13 Extraintestinal Manifestations of Inflammatory Bowel Disease 257 Elena Ricart and William J. Sandborn 14 Cancer in Inflammatory Bowel Disease 279 William M. Bauer and Bret A. Lashner 15 Gender-Specific Issues in Inflammatory Bowel Disease 295 Sunanda V. Kane 16 Economics of Inflammatory Bowel Disease 307 Russell D. Cohen 17 Pathologic Features of Inflammatory Bowel Disease 327 John Hart Index 351 viii Contents x Contributors BARBARA S. KIRSCHNER, MD • Section of Pediatric Gastroenterology, Hepatology, and Nutrition, The University of Chicago Children’s Hospital, Chicago, IL J OSEPH B. KIRSNER, MD, PhD, DSCI (HON) • Department of Medicine, The University of Chicago Medical Center, Chicago, IL B RET A. LASHNER, MD • Department of Gastroenterology, The Cleveland Clinic Foundation, Cleveland, OH G ARY R. LICHTENSTEIN, MD • Division of Gastroenterology, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA P ETER M. MACENEANEY, FRCR • Department of Radiology, The University of Chicago Medical Center, Chicago, IL E LENA RICART, MD • Division of Gastroenterology, Hospital de Sant Pau, Barcelona, Spain W ILLIAM J. SANDBORN, MD • Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN B RUCE E. SANDS, MD • Gastrointestinal Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA M ICHAEL SITRIN, MD • Section of GI/Nutrition, Department of Internal Medicine, The University of Chicago Medical Center, Chicago, IL C HINYU G. SU, MD • Division of Gastroenterology, Department of Medicine, Presbyterian Medical Center, University of Pennsylvania School of Medicine, Philadelphia, PA S TEPHAN R. TARGAN, MD • Inflammatory Bowel Disease Center, Cedars Sinai Medical Center, Los Angeles, CA [...]... from approx 1. 8 /10 0,000 person-years in Finland (19 56– 19 61) (10 ) to 15 .1/ 100,000 person-years in North Tees, England (19 71 19 77) (4) Generally, the incidence varies from 6.3 to 15 .1/ 100,000 person-years (4 ,11 19 ) The estimated prevalence rates in Northern Europe ranges from 58 /10 0,000 person-years in Leiden, Holland (19 83) (16 ) to 15 7 /10 0,000 person-years in the Faroe Islands (19 83) (17 ) The most... colitis in 19 90 19 94 was 14 .5 /10 0,000 in Manitoba Crohn’s Disease The incidence of Crohn’s disease in northern Europe ranges from 0.8/ 10 0,000 person-years in Oxford, England (19 51 19 60) (11 ) to 6 .1/ 10 0,000 person-years in Uppsala, Sweden (19 65 19 83) (19 ) Generally, the incidence rates have varied between 1. 6–5.4 /10 0,000 person-years with prevalence rates of 27–48 /10 0,000 person-years (12 ,16 ,36–45)... Inflammatory Bowel Disease, Fifth Edition W B Saunders, Philadelphia, PA, 19 99 Campieri M, Gionchetti P Probiotics in inflammatory bowel disease: New insight to pathogenesis or a possible therapeutic alternative Gastroenterology 19 99; 11 6 :12 46 12 49 Madsen KL, Doyle JS, Jewell LD, et al Lactobacillus species prevents colitis in interleuken -1 0 gene-deficient mice Gastroenterology 19 99 ;11 6 :11 07 11 14 Paton... entity JAMA 19 32;99 :13 23 13 29 11 Kirsner JB Inflammatory bowel disease Part I Nature and pathogenesis Part II Clinical and therapeutic aspects Disease- a-Month (Masters in Medicine) 19 91; 37: 610 –666, 673–746 12 Bernstein CN, Rawsthorne P, Wajda P, et al.: The high prevalence of Crohn’s disease in a central Canadian province: A population based epidemiologic study Gastroenterology 19 97 ;11 2:A932 13 Gilat... Fireman Z, Rozen P: Inflammatory bowel disease in Jews Front Gastrointest Res 19 86 ;11 :13 5 14 0 14 Snook JA, Dwyer L, Lee-Elliott C, et al Adult coeliac disease and cigarette smoking Gut 19 96;39:60–72 15 Logan R: Smoking and inflammatory bowel disease In Inflammatory Bowel Disease - Current Status and Future Approaches MacDermott RP, ed Elsevier Science Publishers, New York, 19 88, pp 663–670 16 Delco F, Sonnenberg... therapy of the 21st century (11 8) ACKNOWLEDGMENT This work was based in part on “Nonspecific” Inflammatory Bowel Disease (Ulcerative Colitis and Crohn’s disease) after 10 0 Years—What Next? Italian Journal of Gastroenterology and Hepatology 31: 6 51 658, 19 99 (with permission) REFERENCES 1 Kirsner JB Historical basis of the idiopathic inflammatory bowel diseases J Inflamm Bowel Dis 19 95 ;1: 2–26 2 Cameron... Pharmacol 2000;59: 317 –320 11 0 Si-Tahar M, Merlin D, Sitaraman S, Madara JL Constitutive and regulated secretion of secretory leukocyte proteinase inhibitor by human intestinal epithelial cells Gastroenterology 2000 ;11 8 :10 61 10 71 111 Ouellette AJ, Selsted ME Paneth cell defensins: Endogenous peptide components of intestinal host defense Faseb J 19 96 ;10 :12 80 12 89 11 2 Diamond G, Bevins CL B-defensins: Endogenous... counterintuitive therapy for inflammatory bowel disease Curr Treat Opt Gastroenterol 2000;3:95–98 11 6 Lovell DJ, Gianniri EH, Reiff A, et al Etanercept in children with polyarticular juvenile rheumatoid arthritis New Eng J Med 2000;342:763–769 11 7 Romagnani S Th1/Th2 cells J Inflamm Bowel Dis 19 99;5:285–294 11 8 Sands BE Therapy of inflammatory bowel disease Gastroenterology 2000; 11 8:S68–S82 18 Bernstein and... microvascular endothelium in inflammatory bowel disease Lancet 19 98;352 :17 42 17 46 41 Elson CO, Sartor RB, Tennyson GS, Riddell RH Experimental models of inflammatory bowel disease Gastroenterology 19 95 ;10 9 :13 44 13 67 42 Fedorak R, Madsen KL Naturally occurring and experimental IBD In: Kirsner JB, ed Inflammatory Bowel Disease, Fifth Edition W B Saunders, Philadelphia, PA, 19 99 43 Blumberg RS, Saubermann... New York, 19 48:99 10 4, ch 8 7 Shapiro R Regional ileitis - Summary of the literature Am J Med Sci 19 39 ;19 8:269 8 Dalziel TK: Chronic interstitial enteritis Brit Med J 19 13;2 :10 68 10 70 9 Van Kruiningen HJ Lack of support for a common etiology in Johne’s disease of animals and Crohn’s disease in humans J Inflamm Bowel Dis 19 99;5 :18 3 19 1 10 Crohn BB, Ginzburg L, Oppenheimer GD Regional ileitis - a pathologic . references and index. ISBN 0-8 960 3-9 0 9-9 (alk. paper): 1- 5 925 9-3 1 1-9 (ebook) 1. Inflammatory bowel diseases. I. Cohen, Russell D. II. Series. RC862.I53 I527 2003 616 .3'44 dc 21 2002032874 Disclaimer: Some. 19 32;99 :13 23 13 29. 11 . Kirsner JB. Inflammatory bowel disease. Part I. Nature and pathogenesis. Part II. Clinical and therapeutic aspects. Disease- a-Month (Masters in Medicine) 19 91; 37: 610 –666,. Management of Inflammatory Bowel Disease 15 7 Roger D. Hurst 10 Ostomy Care 2 01 Janice C. Colwell 11 Inflammatory Bowel Disease in Children and Adolescents 215 Ranjana Gokhale and Barbara S. Kirschner 12