Sjögrens Syndrome Practical Guidelines to Diagnosis and
Therapy 1st Edition
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Editors
Robert I. Fox, MD, Ph.D.                          Carla M. Fox, RN
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,
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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 “quality of life” of
the patient with Sjögren’s Syndrome (SS). Patients have equated the “impact
of sicca symptoms” at a level of disability equal to moderate angina and
would be willing to “trade two to four years of (their) life expectancy” to
be free of these symptoms. For example, a dry mouth alters the types of foods
a patient can eat and thus interferes with “eating together.” 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 deficiency.
    Extraglandular manifestations can range from vasculitis to lymphopro-
liferative infiltrates 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 “Primer of Rheumatic Diseases,” a major
teaching instrument for residents and fellows in training.
    This volume differs from other recent review publications that have
appeared in “current opinion journals” 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 surgi-
cal 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 pro-
teomics 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.
                                                                             v
vi                                                                                  Preface
   Since the SS patient sees a wide variety of clinical and surgery spe-
cialists, we have included authors from many of the relevant areas to
review their specialty’s 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 “pearls of wisdom.” By integrating the expertise by these eminent
scholars in their respective clinical fields, we try to accomplish the funda-
mental goal of this book—to 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 reflects our goal to recognize
the outstanding contributions of each of these groups located around the
world, but also emphasizes that the “medical world” 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 dra-
matically 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 intercon-
nective 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 finds this book to be a useful “first stop on
Osler’s journey” toward the procurement and utilization of reputable informa-
tion that promotes the superior medical and nursing care that our courageous
Sjögren’s 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 volume—without 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
 1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . .    3
   Robert I. Fox and Carla M. Fox
 2 A Note from Sweden: Recollection of Henrik Sjögren . . . . .           11
   Elke Theander and Frank A. Wollheim
 3 Myths, Pearls, and Tips Regarding Sjögren’s Syndrome . . .             15
   Robert I. Fox, Manuel Ramos-Casals, and John H. Stone
 4 Providing 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
   and Disease Status Indexes in Sjögren’s Syndrome . . . . . .           59
   Claudio Vitali, Chiara Baldini, and Stefano Bombardieri
 6 Histopathology and Glandular Biopsies
   in Sjögren’s Syndrome . . . . . . . . . . . . . . . . . . . . . .      73
   Roland Jonsson, Kathrine Skarstein, and Malin V. Jonsson
 7 Imaging Technology in Sjögren’s Syndrome:
   Non-invasive Evaluation of the Salivary Glands . . . . . . . .         83
   Jonn Terje Geitung and Malin V. Jonsson
Part III Mechanisms of Pathogenesis
 8 Genomics and Viruses in Sjögren’s Syndrome . . . . . . . . .           93
   Kathy L. Moser and John B. Harley
 9 Autoantibodies and Autoantigens in Sjögren’s Syndrome . . . 111
   Kazuhisa Nozawa, Minoru Satoh, Seunghee Cha,
   Yoshinari Takasaki, and Edward K.L. Chan
                                                                          vii
viii                                                                            Contents
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
       Yrjö T. Konttinen, Alberto Vivó Porcar, Pauliina
       Porola, Katja Koskenpato, María Lorés Rodriguez,
       Raimo Pöllänen, 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
13     Pathogenesis: Emphasis on Dry Eye and the Role of
       the Lacrimal Functional Unit in Sjögren’s Syndrome . . . . . 203
       Michael E. Stern and Stephen C. Pflugfelder
14     Oral and Dental Manifestations of Sjögren’s
       Syndrome: Current Approaches to Diagnostics
       and Therapy . . . . . . . . . . . . . . . . . . . . . . . . . . . 221
       Malin V. Jonsson, Nicolas Delaleu,
       Mihaela C. Marthinussen, and Roland Jonsson
15     Etiology and Pathogenesis of Sjögren’s Syndrome
       with Special Emphasis on the Salivary Glands . . . . . . . . . 243
       Nicolas Delaleu, Menelaos N. Manoussakis,
       Haralampos M. Moutsopoulos, and Roland Jonsson
16     Otolaryngologic Manifestations of Sjögren’s
       Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 269
       Jacqui E. Allen and Peter C. Belafsky
17     Extraglandular Manifestations of Sjögren’s
       Syndrome (SS): Dermatologic, Arthritic, Endocrine,
       Pulmonary, Cardiovascular, Gastroenterology,
       Renal, Urology, and Gynecologic Manifestations . . . . . . . 285
       Robert I. Fox
18     Therapy of Dermatologic, Renal, Cardiovascular,
       Pulmonary, Gynecologic, Gastro-enterologic,
       Urologic and Fibromyalgia Manifestations Including
       Special Considerations at the Time of Surgery . . . . . . . . . 317
       Robert I. Fox and Carla M. Fox
19     Current Treatment of Extraglandular Manifestations
       with Disease-Modifying and Immunosuppressive Agents . . . 337
       Athanasios G. Tzioufas and Haralampos M. Moutsopoulos
Contents                                                                             ix
           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
              in Sjögren’s Syndrome in Japan . . . . . . . . . . . . . . . . . 405
              Yoichi Nakagawa and Ichiro Saito
           24 New Approaches for the Management of Dry Mouth
              and Dry Eye in Sjogren’s Syndrome in Japan . . . . . . . . . 415
              Kazuo Tsubota
           25 Sjögren’s Syndrome in Australia: Clinical Practice
              and Research . . . . . . . . . . . . . . . . . . . . . . . . . . . 423
              Tom Gordon and Maureen Rischmueller
           26 Primary Sjögren’s Syndrome: Report from India . . . . . . . 425
              Ramnath Misra, Sapan Pandya, and Debashish Danda
           27 Sjögren’s Syndrome in China . . . . . . . . . . . . . . . . . . 429
              Jing He and Zhan-Guo Li
           28 Sjögren’s Syndrome in China . . . . . . . . . . . . . . . . . . 433
              Chak-sing Lau
           29 Sjögren’s Syndrome in Israel: A Scientific Point
              of View . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437
              Moshe Tishler and Yehuda Shoenfeld
           Part VI   Future Perspectives
           30 Looking into the Future—The EULAR Disease
              Activity Scores: Toward a Consensual Evaluation
              of Primary Sjogren’s Syndrome . . . . . . . . . . . . . . . . . 443
              Raphaèle Seror, Philippe Ravaud, Claudio Vitali,
              Simon J. Bowman, and Xavier Mariette
           31 Looking 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
x                                                                               Contents
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
Contributors
Ilias Alevizos Sjögren’s Syndrome Clinic, National Institute of Dental and
Craniofacial Research, Bethesda, MD, USA, 
[email protected]Elaine Alexander Sjögren’s Syndrome Foundation Medical and Scientific
Advisory Board, San Diego, CA, USA, 
[email protected]Jacqui E. Allen Department of Otolaryngology, University of Auckland,
Auckland, New Zealand, 
[email protected]Chiara Baldini Rheumatology Unit, University of Pisa, Pisa, Italy,
[email protected]Peter C. Belafsky The Center for Voice and Swallowing, University
of California at Davis, Davis, CA, USA, 
[email protected]Julius Birnbaum Division of Rheumatology, The Jerome L. Greene
Sjögren’s Syndrome Center, Johns Hopkins Bayview Medical Center,
Baltimore, MD, USA, 
[email protected]Stefano Bombardieri Rheumatology Unit, University of Pisa, Pisa, Italy,
[email protected]Hendrika Bootsma Department of Rheumatology and Clinical
Immunology, University of Groningen, Groningen, The Netherlands;
University Medical Center, Groningen, The Netherlands,
[email protected]Simon J. Bowman Department of Rheumatology, University Hospitals
Birmingham, Birmingham, England, 
[email protected]Steven E. Carsons SUNY at Stony Brook School of Medicine, Stony
Brook, NY, USA; Division of Rheumatology, Allergy and Immunology,
Winthrop-University Hospital, Mineola, NY, USA, 
[email protected]Seunghee Cha Department of Oral Surgery and Diagnostic Sciences,
University of Florida College of Dentistry, Gainesville, FL, USA;
Department of Oral Biology, University of Florida College of Dentistry,
Gainesville, FL, USA, 
[email protected]fl.edu
Edward K.L. Chan Department of Oral Biology, University of Florida
College of Dentistry, Gainesville, FL, USA, echan@ufl.edu
                                                                          xi
xii                                                                         Contributors
John A. Chiorini Department of Molecular Physiology and Therapeutics
Branch, National Institute of Dental and Craniofacial Research, Bethesda,
MD, USA, 
[email protected]Philip L. Cohen Section of Rheumatology, Department of Medicine,
Temple University School of Medicine, Philadelphia, PA, USA,
[email protected]Debashish Danda Department of Clinical Immunology and
Rheumatology, Christian Medical College, Vellore, India,
[email protected]Nicolas Delaleu Broegelmann Research Laboratory, The Gade Institute,
University of Bergen, Bergen, Norway; Rheumatology Clinic, Scripps
Memorial Hospital and Research Foundation, La Jolla, CA, USA,
[email protected]Carla M. Fox Rheumatology Clinic, Scripps Memorial Hospital and
Research Foundation, La Jolla, CA, USA, 
[email protected]Robert I. Fox Rheumatology Clinic, Scripps Memorial Hospital and
Research Foundation, La Jolla, CA, USA, 
[email protected]Jonn Terje Geitung Haraldsplass Deaconess University Hospital, Bergen,
Norway, 
[email protected]; 
[email protected]Tom Gordon Department of Immunology, Flinders Medical Center,
Flinders, Australia, t.gordon@flinders.edu.au
Jacques-Eric Gottenberg Centre de Recherche Biomédical Strasbourgeois
(CRBS), Hôpitaux Universitaires de Strasbourg, Université de Strasbourg,
Strasbourg, France, 
[email protected]Katherine Morland Hammitt Sjögren’s Syndrome Foundation,
Lynchburg, VA, USA, 
[email protected]John B. Harley Division of Rheumatology, Cincinnati Children’s Hospital
Medical Center, Cincinnati, OH, 
[email protected]Jing He Department of Rheumatology and Immunology, People’s
Hospital, Peking University Medical School, Beijing, China,
[email protected]Gustaaf W. van Imhoff Department of Hematology, University of
Groningen, Groningen, The Netherlands; University Medical Center,
Groningen, The Netherlands, 
[email protected]Malin V. Jonsson Institute of Medicine - Section for Rheumatology,
University of Bergen, Bergen, Norway, 
[email protected]Roland Jonsson Broegelmann Research Laboratory, The Gade Institute,
University of Bergen, Bergen, Norway; Department of Rheumatology,
Haukeland University Hospital, Bergen, Norway,
[email protected]Contributors                                                                           xiii
               Yrjö T. Konttinen Department of Medicine, Biomedicum Helsinki,
               Helsinki University Central Hospital, Helsinki, Finland; ORTON
               Orthopaedic Hospital of the ORTON Foundation, Helsinki, Finland; COXA
               Hospital for the Joint Replacement, Tampere, Finland,
               yrjo.konttinen@helsinki.fi
               Katja Koskenpato Department of Medicine, Biomedicum Helsinki,
               Helsinki University Central Hospital, Helsinki, Finland,
               katja.koskenpato@helsinki.fi
               Chak-sing Lau Division of Rheumatology and Clinical Immunology,
               Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong
               Kong, China, 
[email protected]               Zhan-Guo Li Department of Rheumatology and Immunology, People’s
               Hospital, Peking University Medical School, Beijing, China,               
[email protected]               Menelaos N. Manoussakis Department of Pathophysiology, School of
               Medicine, University of Athens, Athens, Greece, 
[email protected]               Xavier Mariette Assistance Publique-Hôpitaux de Paris (AP-HP),
               Hôpital Bicêtre, Institut Pour la Santé et la Recherche Médicale (INSERM)
               U 1012, Université Paris-Sud 11, Le Kremlin Bicêtre, France,               
[email protected]               Mihaela C. Marthinussen Section for Cariology, Department of Clinical
               Dentistry, Faculty of Medicine and Dentistry, Bergen,               
[email protected]               Paul E. Michelson Eye Care of La Jolla, Scripps Memorial Hospital,
               La Jolla, CA, USA; UCSD School of Medicine, La Jolla, CA, USA,               
[email protected]               Ramnath Misra Department of Immunology, Sanjay Gandhi Post
               Graduate Institute of Medical Sciences, Lucknow, India,               
[email protected]               Kathy L. Moser Arthritis and Immunology Program, Oklahoma Medical
               Research Foundation, Oklahoma City, OK, USA, 
[email protected]               Haralampos M. Moutsopoulos Department of Pathophysiology, School of
               Medicine, University of Athens, Athens, Greece, 
[email protected]               Yoichi Nakagawa Second Department of Oral and Maxillofacial Surgery,
               Tsurumi University School of Dental Medicine, Yokohama, Japan,               
[email protected]               Nikolay P. Nikolov Sjögren’s Syndrome Clinic, National Institute
               of Dental and Craniofacial Research, Bethesda, MD, USA,               
[email protected]               Kazuhisa Nozawa Department of Rheumatology and Internal Medicine,
               Urayasu Hospital Juntendo University, Chiba, Japan; Institute for
xiv                                                                          Contributors
Environment and Gender Specific Medicine, Juntendo University Graduate
School of Medicine, Chiba, Japan; Department of Rheumatology, Juntendo
Urayasu Hospital, Chiba, Japan, 
[email protected]Sapan Pandya Department of Rheumatology, Vedanta Institute of Medical
Sciences, Ahmedabad, India, 
[email protected]Stephen C. Pflugfelder Department of Ophthalmology, Baylor College of
Medicine, Houston, TX, USA, 
[email protected]Justin Pijpe Department of Oral and Maxillofacial Surgery, Catharina
Hospital Eindhoven, Eindhoven, The Netherlands; University Medical
Center, Groningen, The Netherlands, 
[email protected]Raimo Pöllänen Department of Medicine, Biomedicum Helsinki, Helsinki
University Central Hospital, Helsinki, Finland, raimo.pollanen@helsinki.fi
Alberto Vivó Porcar Department of Medicine, Biomedicum Helsinki,
Helsinki University Central Hospital, Helsinki, Finland,
[email protected]Pauliina Porola Department of Medicine, Biomedicum Helsinki, Helsinki
University Central Hospital, Helsinki, Finland, pauliina.porola@helsinki.fi
Beata D. Przybyla Department of Microbiology and Immunology,
University of Arkansas for Medical Sciences, Little Rock, AK, USA,
[email protected]Manuel Ramos-Casals Sjögren Syndrome Research Group (AGAUR),
Hospital Clinic, Barcelona, Spain, 
[email protected]Philippe Ravaud Centre d’Epidomiologie Clinique, INSERM U738,
Hôpital Hotel Dieu, Paris, France, 
[email protected]Maureen Rischmueller Department of Rheumatology, The Queen
Elizabeth Hospital/University of Adelaide, Adelaide, South Australia,
[email protected]María Lorés Rodriguez Department of Medicine, Biomedicum Helsinki,
Helsinki University Central Hospital, Helsinki, Finland,
[email protected]John G. Routsias Department of Pathophysiology, School of Medicine,
University of Athens, Athens, Greece, 
[email protected]Ichiro Saito Department of Pathology, Tsurumi University School
of Dental Medicine, Yokohama, Japan, 
[email protected]Minoru Satoh Division of Rheumatology and Clinical Immunology,
Department of Medicine, University of Florida, Gainesville, FL, USA;
Department of Pathology, Immunology, and Laboratory Medicine,
University of Florida, Gainesville, FL, USA,
[email protected]fl.edu
Raphaèle Seror Assistance Publique-Hopitaux de Paris, Hôpital Bicêtre,
Department of Rheumatology, Institut Pour la Santé et la Recherche
Contributors                                                                               xv
               Médicale (INSERM) U 802, Université Paris-Sud 11, Le Kremlin Bicêtre,
               France, 
[email protected]               Yehuda Shoenfeld Department of Medicine “B” and Zabludowicz Center
               for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel,               
[email protected]               Kathrine Skarstein Broegelmann Research Laboratory, Department
               of Immunology, The Gade Institute, University of Bergen, Bergen, Norway,               
[email protected]               Michelle Spaan Department of Medicine, Biomedicum Helsinki, Helsinki
               University Central Hospital, Helsinki, Finland,               
[email protected]               Vasily Stegaev Department of Medicine, Biomedicum Helsinki, Helsinki
               University Central Hospital, Helsinki, Finland, vasily.stegaev@helsinki.fi
               Michael E. Stern Inflammation Research Program, Allergan, Inc., Irvine,
               CA, USA, 
[email protected]               John H. Stone Division of Rheumatology, Allergy, and Immunology,
               Massachusetts General Hospital, Boston, MA, USA, 
[email protected]               Yoshinari Takasaki Department of Internal Medicine and Rheumatology,
               Juntendo University School of Medicine, Tokyo, Japan,               
[email protected]               Elke Theander Skane University Hospital Malmö, Malmö, Sweden,               
[email protected]               Moshe Tishler Department of Medicine “B”, Asaf Harofe Medical
               Center, Zerifin, Israel 70300, 
[email protected]               Pamela Traisak Section of Rheumatology, Department of Medicine,
               Temple University School of Medicine, Philadelphia, PA, USA,               
[email protected]               Kazuo Tsubota Department of Ophthalmology, Keio University School
               of Medicine, Tokyo, Japan, 
[email protected]               Athanasios G. Tzioufas Department of Pathophysiology, School
               of Medicine, University of Athens, Athens, Greece, 
[email protected]               Liisa Virkki Department of Medicine, Biomedicum Helsinki, Helsinki
               University Central Hospital, Helsinki, Finland, liisa.m.virkki@helsinki.fi
               Claudio Vitali Section of Rheumatology, Department of Internal
               Medicine, Villamarina Hospital, Piombino, Italy, 
[email protected]               Frederick B. Vivino Division of Rheumatology, Penn Presbyterian
               Medical Center, University of Pennsylvania School of Medicine,
               Philadelphia, PA, USA, 
[email protected]               Frank A. Wollheim Lund University Hospital SUS, Lund, Sweden,               
[email protected]       Part I
Introduction
                          Introduction
                                                                                                               1
                          Robert I. Fox and Carla M. Fox
                          Keywords
                          Sjogren’s syndrome • Keratoconjunctivitis sicca • Ophthalmology •
                          Otolaryngology • Oral medicine • Gastroenterology • Neurology •
                          Pathogenesis • Europe • China • India • Israel • Internet
The care of the Sjogren’s syndrome (SS) patient          Europe, the Middle East, South America,
is often shared by many specialists due to the           Australia, and Asia as well as the USA. Different
multi-system involvement of this disease. Each           medications (including herbal) and different
of these specialists reads different journals and        diagnostic methods are used in various parts
rarely attends common educational meetings.              of the world, based on medical resources and
Thus, each specialty has a slightly different view       technology available. These chapters provide
of the “elephant” in the room. The primary goal          examples about how to manage our patients
of this book is to bring together a series of experts    in a cost-efficient manner and new approaches
in different disciplines to provide a common dia-        to therapy. Also, it is important for “western
logue regarding diagnosis and therapy for SS.            physicians” to recognize “centers of excellence”
Each chapter is written in a manner that preserves       among the emerging nations of the world because
the sophistication of their respective field but that     our patients increasingly travel to these regions
employs terminology that will be clear to other          for business and pleasure. Our patients may
specialtists.                                            require medical care either for a complication of
    Although the rheumatologist frequently               their SS or for an infection that is endogenous
becomes the central “quarterback” in the treat-          to that region while travelling. It is worth noting
ment of the SS patient, he/she must be familiar          that in many parts of the world (as well as
with the diagnostic procedures and therapeutic           certain regions of the USA) the “primary” care
approaches of the other specialties to avoid             of rheumatology patients is under the direction
medication interactions and coordination of care.        of non-surgical orthopedic surgeons in part to
An additional goal for this volume is to include         the shortage of available rheumatologists. This
contributors from around the world including             volume also is directed to “non-rheumatologist”
                                                         who is providing care to the SS patient.
                                                            We are fortunate to have contributions in clin-
R.I. Fox ()                                             ical specialties from
Rheumatology Clinic, Scripps Memorial Hospital and
Research Foundation, La Jolla, CA, USA                   • Ophthalmology
e-mail: [email protected]                              • Oral medicine
R.I. Fox, C.M. Fox (eds.), Sjögren’s Syndrome, DOI 10.1007/978-1-60327-957-4_1,                           3
© Springer Science+Business Media, LLC 2011