Table Of ContentThe Sjögren’s Book
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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
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Copyright © 2005, 2012 Th e Sjögren’s Syndrome Foundation
Published by Oxford University Press, Inc.
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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
Description: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