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The Sjögren's Book, 4th Edition PDF

412 Pages·2011·2.14 MB·English
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The Sjögren’s Book This page intentionally left blank The Sjögren’s Book FOURTH EDITION Edited by Daniel J. Wallace, MD Associate Editors: Steven E. Carsons, MD Elaine Alexander, MD, PhD Frederick B. Vivino, MD Katherine Morland Hammitt, MA Steven Taylor, SSF CEO Assistant Editor: Patricia Spolyar, RN A publication of the Sjögren’s Syndrome Foundation and 1 1 Oxford University Press, Inc., publishes works that further Oxford University’s objective of excellence in research, scholarship, and education. Oxford New York Auckland Cape Town Dar es Salaam Hong Kong Karachi Kuala Lumpur Madrid Melbourne Mexico City Nairobi New Delhi Shanghai Taipei Toronto With offi ces in Argentina Austria Brazil Chile Czech Republic France Greece Guatemala Hungary Italy Japan Poland Portugal Singapore South Korea Switzerland Th ailand Turkey Ukraine Vietnam Copyright © 2005, 2012 Th e Sjögren’s Syndrome Foundation Published by Oxford University Press, Inc. 198 Madison Avenue, New York, New York 10016 www.oup.com Oxford is a registered trademark of Oxford University Press 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, without the prior permission of Oxford University Press. ______________________________________________________________ Library of Congress Cataloging-in-Publication Data Th e Sjogren’s book / edited by Daniel J. Wallace.—4th ed. p. cm. Rev. ed. of: New Sjogren’s syndrome handbook editor, Daniel J. Wallace; associate editors, Evelyn J. Bromet ... [et al.]. Includes bibliographical references and index. ISBN 978-0-19-973722-2 1. Sjogren’s syndrome—Popular works. I. Wallace, Daniel J. (Daniel Jeff rey), 1949- II. New Sjogren’s syndrome handbook. RC647.5.S5N49 2011 616.7’75—dc23 2011022192 ______________________________________________________________ 9 8 7 6 5 4 3 2 1 Printed in USA on acid-free paper CONTENTS Contributors ix Preface by Steven E. Carsons, MD, Founding Member, Sjögren’s Syndrome Foundation Medical and Scientifi c Advisory Board xv Introduction: Why Write a Book on Sjögren’s? by Daniel J. Wallace xvii PART ONE Introduction and Defi nitions 1. Sjögren’s Syndrome Foundation: The Patients’ and Professionals’ Key Resource and Advocate 3 STEVEN TAYLOR 2. What is Sjögren’s? 7 ARTHUR I. GRAYZEL 3. Who Develops Sjögren’s? 11 KATY M. SETOODEH AND DANIEL J. WALLACE PART TWO The Pathophysiology of Sjögren’s 4. Pathogenesis of Sjögren’s 17 IAN R. MACKAY 5. Genetics and Sjögren’s Syndrome 31 JOHN A. ICE, CHRISTOPHER J. LESSARD, AND KATHY L. MOSER 6. Sjögren’s Syndrome: An Immunological Perspective 43 ROBERT I. FOX 7. What Causes Exocrine Dysfunction in Sjögren’s? 62 JANINE AUSTIN CLAYTON, SERENA MORRISON, AND PHILIP C. FOX PART THREE Where and How Can the Body be Affected by Sjögren’s? 8. Generalized Symptoms and Signs of Sjögren’s 73 STEVEN E. CARSONS AND NEHAD R. SOLOMON v vi Contents 9. The Internal Organs in Sjögren’s 80 FOTINI C. SOLIOTIS, STUART S. KASSAN, AND HARALAMPOS M. MOUTSOPOULOS 10. The Central Nervous System and Sjögren’s: Vasculitis and Vasculopathy 92 ELAINE ALEXANDER 11. The Gastrointestinal Tract 106 MATTHEW NICHOLS 12. Gynecological Issues, Including Pregnancy 113 THOMAS R. ALLAN AND ANN PARKE 13. Lymphoma 124 ELKE THEANDER AND EVA BAECKLUND 14. Diseases Associated with Sjögren’s Syndrome 130 FREDERICK B. VIVINO 15. The Dry Eye 147 ABHA GULATI, REZA DANA, AND GARY N. FOULKS 16. The Dry Mouth 157 AVA J. WU AND TROY E. DANIELS 17. Salivary Glands, Ears, Nose, and Throat 168 ROBERT S. LEBOVICS 18. Lab Work: Blood Tests, Imaging, Biopsies, and Beyond 173 ALAN N. BAER 19. Why Is Sjögren’s Syndrome So Diffi cult to Diagnose? 191 ROBERT F. SPIERA AND HARRY SPIERA PART FOUR The Management of Sjögren’s 20. Treatment of Dry Mouth 201 PHILIP C. FOX, MABI L. SINGH, AND ATHENA S. PAPAS 21. Management of Dry Eye 210 S. LANCE FORSTOT AND GARY N. FOULKS 22. Treatment of Other Sicca Symptoms 218 A. Dry Ears, Nose, and Sinuses 218 BY SOO KIM ABBOUD B. Dry Skin 222 BY THERESA L. RAY AND JOHN R. FENYK, JR. Contents vii C. Vaginal Dryness 224 BY ELISA RODRIGUEZ TROWBRIDGE 23. Diagnosis and Management of Fatigue 228 RICHA MISHRA AND FREDERICK B. VIVINO 24. Management of Chronic Musculoskeletal Pain 235 LAN CHEN 25. Management of Serious Internal Organ Manifestations 241 DANIEL SMALL 26. Evaluation and Management of the Neurological Manifestations of Sjögren’s Syndrome 249 STEVEN MANDEL, CARLA LOPINTO-KHOURY, RAMON MANON-ESPAILLAT, WILLIAM NEIL, SCOTT PELLO, DAVID ROSHAL, AND MEREDITH SNAPP 27. Complementary and Alternative Therapies for Sjögren’s Syndrome 260 PAUL F. HOWARD AND C. KEITH WILKINSON 28. Vitamin D and Sjögren’s Syndrome 271 JEFFREY WILSON 29. Testing New Drugs and Future Directions 275 YOUNG H. LEE, E. WILLIAM ST. CLAIR, AND PHILIP L. COHEN PART FIVE Living with Sjögren’s 30. Quality of Life and Sjögren’s 287 BARBARA M. SEGAL 31. Lifestyle Issues—Emotional 293 TERI P. RUMPF AND LYNN C. EPSTEIN 32. Enhancing Resilience Through Mind/Body Interactions 301 MARGARET BAIM 33. Related Topics on Living with Sjögren’s 311 A. Sex 311 BY ANNE E. BURKE B. Sleep 318 BY H. KENNETH FISHER C. Surgery 322 BY LYNN M. PETRUZZI D. Vaccinations 329 BY RICHARD D. BRASINGTON, JR. viii Contents 34. Disability and Sjögren’s 333 THOMAS D. SUTTON AND KATHERINE MORLAND HAMMITT 35. Web, Print, and Media Resource Manual 345 Glossary 357 Appendix: Products for Sjögren’s Patients 373 Index 375 CONTRIBUTORS Soo Kim Abboud, MD Assistant Professor, Department of Otolaryngology– Head and Neck Surgery, University of Pennsylvania School of Medicine; Chief of Otolaryngology, Penn Presbyterian Medical Center, Philadelphia, Pennsylvania Elaine Alexander, MD, PhD Biomedical Consultant, San Diego, California Th omas R. Allan, MD Department of Obstetrics and Gynecology, Hartford Hospital, Hartford, Connecticut. Assistant Clinical Professor, University of Connecticut, Farmington, Connecticut Eva Baecklund, MD, PhD Senior Consultant, Unit of Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden Alan N. Baer, MD Associate Professor of Medicine and Director, Jerome L. Greene Sjögren’s Syndrome Clinic, Division of Rheumatology, Johns Hopkins University School of Medicine, and Chief of Rheumatology, Good Samaritan Hospital, Baltimore, Maryland Margaret Baim, MS, NP Clinical Director for Training, Benson Henry Institute for Mind Body Medicine, Massachusetts General Hospital, Boston, Massachusetts Richard D. Brasington, Jr., MD Professor of Medicine, Director of Clinical Rheumatology, Washington University School of Medicine, St. Louis, Missouri Anne E. Burke, MD, MPH Assistant Professor of Gynecology and Obstetrics, Johns Hopkins University School of Medicine, Bayview Medical Center, Baltimore, Maryland Steven E. Carsons, MD Chief, Division of Rheumatology, Allergy and Immunology, Winthrop University Hospital, and Professor of Medicine, SUNY, Stony Brook, Mineola, New York Lan Chen, MD, PhD Clinical Associate of Medicine and Attending Rheumatologist, Division of Rheumatology, Penn Presbyterian Medical Center; Penn Sjögren’s Center, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania ix

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Afflicting one in seventy Americans, Sj???gren's syndrome is an autoimmune disease that commonly causes dryness of the eyes, mouth, and nose, and that can lead to complications including profound fatigue, depression, and lymphoma. While there is no cure for Sj???gren's, much can be done to alleviate
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