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Contents Preface: Sjo ¨ gren’s Syndrome ix Michael T. Brennan Sjo ¨ gren’s Syndrome: An Update on Epidemiology and Current Insights on Pathophysiology 1 Tove R. Reksten and Malin V. Jonsson Primary Sjo ¨ gren’s syndrome (pSS) is an autoimmune chronic inflammatory disorder affecting 0.2% to 3.0% of the population, with a 9:1 female to male ratio. Features are oral and ocular dryness, local and systemic autoantibody production, and progressive focal mononuclear cell infiltration in the affected salivary and lacrimal glands. Lymphoma is the most severe complication of pSS, occurring in 4% to 5% of patients. Genetic studies identified an association with HLA and susceptibility genes in cytokine genes and genes involved in B-cell differentiation. Genetic varia- tions may help explain why disease manifestations differ among patients and sup- ports the hypothesis of certain distinct disease phenotypes. Diagnosis of Sjo ¨ gren’s Syndrome: American-European and the American College of Rheumatology Classification Criteria 13 Vidya Sankar, Jenene L. Noll, and Michael T. Brennan Classification criteria provide a formalized approach to studying course and man- agement of rheumatic disease, as well as a measure of improvement in care. Under- standing the purposes of classification criteria sets and the differences between different classification criteria is crucial for understanding rheumatic disease and for the design and conduct of clinical and epidemiologic investigations. In this article, the similarities and differences between the American-European Consensus Group Criteria (AECG) and the newly proposed American College of Rheumatology (ACR) classification criteria for Sjo ¨ gren’s syndrome and the clinical implications of switching to the ACR classification criteria from the AECG are described. Salivary Gland Biopsy for Sjo ¨ gren’s Syndrome 23 Konstantina Delli, Arjan Vissink, and Fred K.L. Spijkervet Salivary gland biopsy is a technique broadly applied for the diagnosis of Sjo ¨ gren’s syndrome (SS), lymphoma accompanying SS, sarcoidosis, amyloidosis, and other connective tissue disorders. SS has characteristic microscopic findings involving lymphocytic infiltration surrounding the excretory ducts in combination with destruc- tion of acinar tissue. This article focuses on the main techniques used for taking labial and parotid salivary gland biopsies in the diagnostic workup of SS with respect to their advantages, their postoperative complications, and their usefulness for diagnostic procedures, monitoring disease progression, and treatment evaluation. Salivary Gland Dysfunction and Xerostomia in Sjo ¨ gren’s Syndrome 35 Siri Beier Jensen and Arjan Vissink In this article, salivary gland dysfunction and xerostomia in Sjo ¨ gren’s syndrome (SS) are discussed, with a focus on the pathophysiology of salivary dysfunction in SS, the Sjo ¨ gren’s Syndrome

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Page 1: Contents

Sjogren’s Syndrome

Contents

Preface: Sjogren’s Syndrome ix

Michael T. Brennan

Sjogren’s Syndrome: An Update on Epidemiology and Current Insights onPathophysiology 1

Tove R. Reksten andMalin V. Jonsson

Primary Sjogren’s syndrome (pSS) is an autoimmune chronic inflammatory disorderaffecting 0.2% to 3.0% of the population, with a 9:1 female to male ratio. Featuresare oral and ocular dryness, local and systemic autoantibody production, andprogressive focal mononuclear cell infiltration in the affected salivary and lacrimalglands. Lymphoma is the most severe complication of pSS, occurring in 4% to5% of patients. Genetic studies identified an association with HLA and susceptibilitygenes in cytokine genes and genes involved in B-cell differentiation. Genetic varia-tions may help explain why disease manifestations differ among patients and sup-ports the hypothesis of certain distinct disease phenotypes.

Diagnosis of Sjogren’s Syndrome: American-European and the American Collegeof Rheumatology Classification Criteria 13

Vidya Sankar, Jenene L. Noll, andMichael T. Brennan

Classification criteria provide a formalized approach to studying course and man-agement of rheumatic disease, as well as a measure of improvement in care. Under-standing the purposes of classification criteria sets and the differences betweendifferent classification criteria is crucial for understanding rheumatic disease andfor the design and conduct of clinical and epidemiologic investigations. In thisarticle, the similarities and differences between the American-European ConsensusGroup Criteria (AECG) and the newly proposed American College of Rheumatology(ACR) classification criteria for Sjogren’s syndrome and the clinical implicationsof switching to the ACR classification criteria from the AECG are described.

Salivary Gland Biopsy for Sjogren’s Syndrome 23

Konstantina Delli, Arjan Vissink, and Fred K.L. Spijkervet

Salivary gland biopsy is a technique broadly applied for the diagnosis of Sjogren’ssyndrome (SS), lymphoma accompanying SS, sarcoidosis, amyloidosis, and otherconnective tissue disorders. SS has characteristic microscopic findings involvinglymphocytic infiltration surrounding the excretory ducts in combination with destruc-tion of acinar tissue. This article focuses on the main techniques used for takinglabial and parotid salivary gland biopsies in the diagnostic workup of SS with respectto their advantages, their postoperative complications, and their usefulness fordiagnostic procedures, monitoring disease progression, and treatment evaluation.

Salivary Gland Dysfunction and Xerostomia in Sjogren’s Syndrome 35

Siri Beier Jensen andArjan Vissink

In this article, salivary gland dysfunction and xerostomia in Sjogren’s syndrome (SS)are discussed, with a focus on the pathophysiology of salivary dysfunction in SS, the

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clinical presentation of dry mouth in SS, how to assess salivary gland hypofunctionand xerostomia in SS, and the impact of salivary gland dysfunction on quality of lifein patients with SS.

Oral Complications of Sjogren’s Syndrome 55

Joel J. Napenas and Tanya S. Rouleau

Numerous oral manifestations associated with salivary gland dysfunction, andparticularly Sjogren’s syndrome, have been reported in the literature. This articlediscusses the evidence on a wide range of oral manifestations associated withSjogren’s syndrome.

Management of Xerostomia and Other Complications of Sjogren’s Syndrome 63

Andres Pinto

This article provides an overview of the published literature in English in the past 63years involving the management of xerostomia and other oral complications ofSjogren’s syndrome. The evidence supporting studied interventions was evaluatedusing published criteria.

Salivary Gland Disease in Sjogren’s Syndrome: Sialoadenitis to Lymphoma 75

Michael D. Turner

Although the cause and molecular pathways of Sjogren’s syndrome are stillunknown, basic, clinical, and translational science have started to identify linkagesto other known processes. With the advent of newer, more sensitive, and moreaccurate chemokine, cytokine, and genetic analysis, the molecular progression ofthe disease may be understood. The modern technology of sialoendoscopy to treatobstructive sialoadenitis frommucous plugging, and the addition of rituximab to cur-rent chemotherapy, have allowed patients with Sjogren’s syndrome to have a betterquality of life and, if they develop lymphomatous changes, a significant increase intheir disease remission and survival rate.

The Role of Parotidectomy in Sjogren’s Syndrome 83

RafaelMadero-Visbal and ZvonimirMilas

Sjogren’s syndrome, a chronic and progressive autoimmune disorder mainly char-acterized by xerophthalmia, xerostomia, and parotid enlargement, is primarily man-aged medically, but some patients will require surgical management. Patients withSjogren’s syndrome have an increased risk of non-Hodgkin lymphoma. Superficialparotidectomy is indicated for diagnostic purposes and can be therapeutic in limitedcircumstances. Surgical indications for parotidectomy in Sjogren’s syndrome in-clude recurrent parotitis refractory to medical management; salivary gland malig-nancy; and severe, refractory pain. Surgical complications include transient orpermanent facial nerve injury, post-operative pain, persistent inflammation of rem-nant parotid tissue, Frey syndrome, and facial scarring.

Extraglandular Manifestations of Primary Sjogren’s Syndrome 91

Sabatino Ienopoli and Steven E. Carsons

Sjogren’s syndrome is a chronic autoimmune disease that typically affects the sal-ivary and lacrimal glands. Aside from the common glandular signs and symptoms,

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Contents vii

Sjogren’s syndrome may also cause mononuclear infiltration and immune complexdeposition involving extraglandular sites producing several extraglandular manifes-tations (EGM). The prevalence of EGMs varies greatly depending on the particularmanifestation. This article examines the ways that EGMs may present in patientswith primary Sjogren’s syndrome. The focus is on the more prevalent and significantEGMs including involvement of the nervous system, pulmonary manifestations, vas-culitis associated with primary Sjogren’s syndrome, and arthropathy.

Management of Extraglandular Manifestations of Primary Sjogren’s Syndrome 101

Jason J. Wuand Steven E. Carsons

Primary Sjogren’s syndrome can have multiple extra-glandular manifestationsranging from mild to severe. Treatment for extra-glandular manifestations is organspecific and therapies are targeted based on the primary organs involved. Preferredtreatment options used for extra-glandular manifestations of Sjogren’s syndromeare usually extrapolated from the physician’s experience in treating similar manifes-tations in other autoimmune conditions such as rheumatoid arthritis and systemiclupus erythematous. The lack of immunomodulating disease modifying drugs inSjogren’s syndrome can be frustrating for patients dealing with extra-glandular man-ifestations, however recent advances in the field has made the future look promisingfor new therapeutic options.

Coping Strategies and Support Networks for Sjogren’s Syndrome Patients 111

Andrea Herman, Steven Taylor, and Jenene Noll

Sjogren’s syndrome is a chronic systemic autoimmune disease that can affect anyorgan system in the body. The most common symptoms are dryness of the mouthand eyes resulting from chronic inflammation and a progressive loss of secretoryfunction. As with most individuals managing a chronic condition, patients withSjogren’s are on a multipronged path to disease and symptom management.Various coping strategies are presented in this article and the advantages and dis-advantages discussed. Additionally, how a support group functions and practicalguidance for the initiation of a Sjogren’s support group are discussed.

Index 117