Sjögren’s Syndrome
Robert I. Fox · Carla M. Fox
Editors
Sjögren’s Syndrome
Practical Guidelines to Diagnosis and Therapy
123
Editors |
Carla M. Fox, RN |
Robert I. Fox, MD, Ph.D. |
|
Scripps Memorial Hospital & Research |
Scripps Memorial Hospital & Research |
Foundation |
Foundation |
Rheumatology Clinic |
Rheumatology Clinic |
La Jolla, CA 92037, USA |
La Jolla, CA 92037, USA |
ISBN 978-1-60327-956-7 e-ISBN 978-1-60327-957-4 DOI 10.1007/978-1-60327-957-4
Springer New York Dordrecht Heidelberg London
Library of Congress Control Number: 2011932791
© Springer Science+Business Media, LLC 2011
All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden.
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Preface
“He who studies medicine without books sails an uncharted sea,
but he who studies medicine without patients does not go to sea at all.”
—Sir William Osler (1849–1916)
Symptoms of dry eyes and dry mouth negatively affect the Òquality of lifeÓ of the patient with SjšgrenÕs Syndrome (SS). Patients have equated the Òimpact of sicca symptomsÓ at a level of disability equal to moderate angina and would be willing to Òtrade two to four years of (their) life expectancyÓ to be free of these symptoms. For example, a dry mouth alters the types of foods a patient can eat and thus interferes with Òeating together.Ó Eating serves far more than nutrition needs and represents a major part of social interaction for women at home, business, and relaxation with friends. Dry eyes interfere with the ability to use a computer for prolonged periods at work or at home, since staring at a computer screen involves a markedly decreased blink rate and exacerbates symptoms of aqueous tear deÞciency.
Extraglandular manifestations can range from vasculitis to lymphoproliferative inÞltrates of many organs. SS patients have a markedly increased risk incidence of lymphoma. Many older patients diagnosed with systemic lupus erythematosus (SLE) actually have SS. Since the correct diagnosis is not made for many years in most patients, their symptoms and signs due to glandular involvement are not adequately recognized and treated. Even in rheumatology, SS is not listed in the ÒPrimer of Rheumatic Diseases,Ó a major teaching instrument for residents and fellows in training.
This volume differs from other recent review publications that have appeared in Òcurrent opinion journalsÓ or review volumes largely written by specialists in a single medical specialty. We have included a distinguished panel of authors in a broad spectrum of basic science, clinical, and surgical disciplines. Further, we have included authors from multiple areas of the world where SS has been studied.
Pathogenic studies reviewed in this volume include genetics and proteomics as well as cytokines and lymphoid regulation. The authors also emphasize that SS represents the intersection of the immune, secretory, and neural regulatory axes. An understanding of pathogenesis provides the backbone for understanding current diagnostic and therapeutic approaches. Further, basic science and astute clinical observation are the routes to future improved therapies.
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Preface |
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Since the SS patient sees a wide variety of clinical and surgery specialists, we have included authors from many of the relevant areas to review their specialtyÕs approach to diagnosis and therapy. For example, the management of SS often involves Ophthalmology, Oral Medicine, Oral Surgery, Otolaryngology, Gastroenterology, Dermatology, Nephrology, Chest Medicine, Hematology and Oncology, Neurology, and Rheumatology. Each specialty has been given the task of providing useful clinical hints as well as their Òpearls of wisdom.Ó By integrating the expertise by these eminent scholars in their respective clinical Þelds, we try to accomplish the fundamental goal of this bookÑto present cutting-edge diagnosis and therapeutic approaches to SS in one authoritative source.
The collaborative efforts of contributors in this book geographically span Europe, the Middle East, Asia, India, Australia, as well as the USA. This collection of enlightening chapters not only reßects our goal to recognize the outstanding contributions of each of these groups located around the world, but also emphasizes that the Òmedical worldÓ is increasingly a global community composed of patients and their physicians.
In different regions of the world, the disease may have subtly different presentations, and the available diagnostic and therapeutic resources are dramatically different. Our patients increasingly present to us from different parts of the world, and they also travel to different regions for work or holiday. The inclusion of authors from around the world renders this resource an interconnective conduit between US rheumatologists and our counterparts throughout the world, in order to provide a universal spectrum of educational and clinical resources for our SS patients as well as for ourselves.
It is our hope that the reader Þnds this book to be a useful ÒÞrst stop on OslerÕs journeyÓ toward the procurement and utilization of reputable information that promotes the superior medical and nursing care that our courageous SjšgrenÕs patients deserve.
We also wish to express our profound gratitude to: (a) our esteemed Authors, (b) our developmental editor Judi Brown, and (c) Springer Science+Business Media, for the immense amount of time, effort, and vision dedicated to the inception and evolution of this volumeÑwithout this degree of synergistic collaboration, this book would not have been possible.
La Jolla, California |
Robert I. Fox |
|
Carla M. Fox |
Contents
Part I |
Introduction |
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1 |
Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
3 |
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Robert I. Fox and Carla M. Fox |
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2 |
A Note from Sweden: Recollection of Henrik Sjögren . . . . . |
11 |
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Elke Theander and Frank A. Wollheim |
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3 |
Myths, Pearls, and Tips Regarding Sjögren’s Syndrome . . . |
15 |
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Robert I. Fox, Manuel Ramos-Casals, and John H. Stone |
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4Providing Information to Referring Physicians and Patients . 35
Robert I. Fox and Carla M. Fox
Part II Classification Criteria and Disease Status Activity, Histology of Salivary Gland Biopsy, and Imaging
5 |
Current Concepts on Classification Criteria |
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and Disease Status Indexes in Sjögren’s Syndrome . . . . . . |
59 |
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Claudio Vitali, Chiara Baldini, and Stefano Bombardieri |
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6 |
Histopathology and Glandular Biopsies |
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in Sjögren’s Syndrome . . . . . . . . . . . . . . . . . . . . . . |
73 |
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Roland Jonsson, Kathrine Skarstein, and Malin V. Jonsson |
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7 |
Imaging Technology in Sjögren’s Syndrome: |
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Non-invasive Evaluation of the Salivary Glands . . . . . . . . |
83 |
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Jonn Terje Geitung and Malin V. Jonsson |
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Part III Mechanisms of Pathogenesis |
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8 |
Genomics and Viruses in Sjögren’s Syndrome . . . . . . . . . |
93 |
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Kathy L. Moser and John B. Harley |
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9Autoantibodies and Autoantigens in Sjögren’s Syndrome . . . 111
Kazuhisa Nozawa, Minoru Satoh, Seunghee Cha, Yoshinari Takasaki, and Edward K.L. Chan
vii
viii |
Contents |
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10 B-cell Epitopes of Sjögren’s Syndrome-Related
Autoantigens Ro/SSA and La/SSB . . . . . . . . . . . . . . . 133
Athanasios G. Tzioufas, John G. Routsias, and Haralampos M. Moutsopoulos
11 Neurobiology and Hormonal Control of Lacrimal
and Salivary Gland Function . . . . . . . . . . . . . . . . . . 151
Yrjš T. Konttinen, Alberto Viv— Porcar, Pauliina
Porola, Katja Koskenpato, Mar’a LorŽs Rodriguez, Raimo PšllŠnen, Vasily Stegaev, Liisa Virkki, Michelle Spaan, and Beata D. Przybyla
Part IV Clinical and Therapeutic Considerations
12 Overview of Management of Dry Eye Associated with
Sjögren’s Syndrome . . . . . . . . . . . . . . . . . . . . . . . 179 Paul E. Michelson and Robert I. Fox
13Pathogenesis: Emphasis on Dry Eye and the Role of
the Lacrimal Functional Unit in Sjögren’s Syndrome . . . . . 203
Michael E. Stern and Stephen C. Pßugfelder
14Oral and Dental Manifestations of Sjögren’s Syndrome: Current Approaches to Diagnostics
and Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . |
221 |
Malin V. Jonsson, Nicolas Delaleu, |
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Mihaela C. Marthinussen, and Roland Jonsson |
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15 Etiology and Pathogenesis of Sjögren’s Syndrome |
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with Special Emphasis on the Salivary Glands . . . . . . . . . |
243 |
Nicolas Delaleu, Menelaos N. Manoussakis, |
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Haralampos M. Moutsopoulos, and Roland Jonsson |
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16 Otolaryngologic Manifestations of Sjögren’s |
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Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
269 |
Jacqui E. Allen and Peter C. Belafsky |
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17 Extraglandular Manifestations of Sjögren’s |
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Syndrome (SS): Dermatologic, Arthritic, Endocrine, |
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Pulmonary, Cardiovascular, Gastroenterology, |
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Renal, Urology, and Gynecologic Manifestations . . . . . . . |
285 |
Robert I. Fox |
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18 Therapy of Dermatologic, Renal, Cardiovascular, |
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Pulmonary, Gynecologic, Gastro-enterologic, |
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Urologic and Fibromyalgia Manifestations Including |
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Special Considerations at the Time of Surgery . . . . . . . . . |
317 |
Robert I. Fox and Carla M. Fox |
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19Current Treatment of Extraglandular Manifestations
with Disease-Modifying and Immunosuppressive Agents . . . 337
Athanasios G. Tzioufas and Haralampos M. Moutsopoulos
Contents |
ix |
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20 Lymphoproliferation and Lymphoma in Sjögren’s
Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345 Justin Pijpe, Hendrika Bootsma, and Gustaaf W. van Imhoff
21 Fatigue, Dryness, and Quality of Life as Clinical Trial
Outcomes in Primary Sjögren’s Syndrome . . . . . . . . . . . 357
Simon J. Bowman
22 The Neurological Manifestations of Sjögren’s
Syndrome: Diagnosis and Treatment . . . . . . . . . . . . . . 373
Robert I. Fox and Julius Birnbaum
Part V The Views from Japan, China, India, and Australia
23 |
New Approaches for the Management of Dry Mouth |
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in Sjögren’s Syndrome in Japan . . . . . . . . . . . . . . . . . |
405 |
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Yoichi Nakagawa and Ichiro Saito |
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24 |
New Approaches for the Management of Dry Mouth |
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and Dry Eye in Sjogren’s Syndrome in Japan . . . . . . . . . |
415 |
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Kazuo Tsubota |
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25 |
Sjögren’s Syndrome in Australia: Clinical Practice |
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and Research . . . . . . . . . . . . . . . . . . . . . . . . . . . |
423 |
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Tom Gordon and Maureen Rischmueller |
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26 |
Primary Sjögren’s Syndrome: Report from India . . . . . . . |
425 |
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Ramnath Misra, Sapan Pandya, and Debashish Danda |
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27 |
Sjögren’s Syndrome in China . . . . . . . . . . . . . . . . . . |
429 |
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Jing He and Zhan-Guo Li |
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28 |
Sjögren’s Syndrome in China . . . . . . . . . . . . . . . . . . |
433 |
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Chak-sing Lau |
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29 |
Sjögren’s Syndrome in Israel: A Scientific Point |
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of View . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
437 |
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Moshe Tishler and Yehuda Shoenfeld |
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Part VI Future Perspectives
30 Looking into the Future—The EULAR Disease Activity Scores: Toward a Consensual Evaluation
of Primary Sjogren’s Syndrome . . . . . . . . . . . . . . . . . 443
Rapha•le Seror, Philippe Ravaud, Claudio Vitali, Simon J. Bowman, and Xavier Mariette
31Looking into the Crystal Ball: Initiatives from the Sjögren’s Syndrome Foundation That Will Impact
Patient Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . 455
Elaine Alexander, Frederick B. Vivino,
Steven E. Carsons, and Katherine Morland Hammitt
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Contents |
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32 Biological Treatment for Sjögren’s Syndrome . . . . . . . . . 459
Philip L. Cohen and Pamela Traisak
33 Looking into the Future—Emerging Therapies Based
on Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . 469 Jacques-Eric Gottenberg and Xavier Mariette
34 Into the Future: Autonomic Neuropathy, MicroRNAs,
and Gene Therapy . . . . . . . . . . . . . . . . . . . . . . . . 483 Ilias Alevizos, John A. Chiorini, and Nikolay P. Nikolov
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . |
489 |