Therapeutic Approaches

Patients with sensory complaints should be followed and a trial of immunomodulatory treatment considered if the extent of involvement and rate of progression suggest active inflammation. In the majority of pSS patients in whom sensory deficits remain stable, management is symptomatic and principles of neuropathic pain management are recommended. Treatments with documented efficacy in other neuropathic pain states include tricyclics, gabapentin, pregabalin, duloxetine, high-dose venlaxifene,...

Interstitial Lung Disease

ILD can be a manifestation of pulmonary disease in various connective tissue diseases.48 In some patients, the lung disease may be an initial presentation, occurring before the other more usual clinical complaints are apparent,25,26 and it has been estimated that 15 to 20 of patients presenting with chronic ILD have an occult connective tissue disease,49 whereas other studies have suggested that approximately 8 of primary SS patients have ILD.50 Patients who have SS may develop various ILDs,...

Info

A Values are mean (SD). b At Sjogren's syndrome diagnosis. From Theander E, Jacobsson LTH. Features of systemic lupus erythematosus in patients with primary Sjogren's syndrome. A cross-sectional analysis of the 11 items of the SLE criteria set and the levels of complement factors C3 and C4 in 100 primary Sjogren's syndrome patients. Lupus. 2005 14(3) S231 with permission. a Values are mean (SD). b At Sjogren's syndrome diagnosis. From Theander E, Jacobsson LTH. Features of systemic lupus...

Keywords

Sjogren's syndrome B cell BAFF BLyS Rituximab B-CELL HYPERACTIVITY IN SJ GREN SYNDROME B-cell hyperactivity has been recognized for a long time in Sjogren's syndrome (SS). More than 30 years ago, Talal and colleagues1 demonstrated an increased level in beta2-microglobulin (beta2-m) in the serum of patients with SS, and, more than 20 years ago, Moutsopoulos and colleagues2 found increased levels of light chains of immunoglobulins in the urine of patients with SS. Indeed, SS is the autoimmune...

Diagnosis

Differential Diagnosis of Central Nervous System-Sjogren's Syndrome A large differential diagnosis, including inflammatory, infectious, genetic, metabolic, and neoplastic disorders, must be considered in the evaluation of CNS manifestations accompanying pSS. Other primary neurologic disorders, including amyotrophic lateral sclerosis and Parkinson's disease, must occasionally be considered in the neurologic differential depending upon the clinical presentation. Evaluation for antiphospholipid...

References

Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002 61(6) 554-8. 2. Bowman SJ, Pillemer S, Jonsson R, et al. Revisiting Sjogren's syndrome in the new millennium perspectives on assessment and outcome measures. Report of a workshop held on 23 March 2000 at Oxford, UK. Rheumatology 2001 40(10) 1180-8. 3. Pillemer SR, Smith J, Fox PC, et al. Outcome...

Q

Src tyrosine kinases (Csk) by which it functions to down-regulate T-cell signaling.94 Lyp also binds the adaptor molecule, Grb2, leading to the negative regulation of T-cell signaling. In a collection of 70 pSS cases in Columbia and 308 matched controls, Gomez and colleagues93 found the 1858 T allele a risk factor for SS (OR 2.42). After genotyping a collection of 183 pSS patient samples and 172 healthy controls, however, Ittah and colleagues94 found no significant difference in the 1858 T...

Fulfillment of the Sjogrens Syndrome Classification Criteria

There is a considerable overlap between European diagnostic criteria for SS and some extrahepatic features of HCV infection.39 Extrapolating from the main studies with large series of HCV patients,6 xerostomia was observed in 158 (18 ) of 859 patients xerophthalmia in 129 (17 ) of 769 positive ocular tests in 83 (38 ) of 216 positive salivary gland biopsy (grades 3-4 of Chisholm-Mason classification) in 64 (25 ) of 251 positive ANA in 481 (18 ) of 2641 and positive RF in 357 (40 ) of 1117 HCV...

Ama

Abbreviations AMA, antimitochondrial antibodies ENA, antiextractable nuclear antigens (anti-Ro SS-A, anti-La SS-B, anti-RNP, anti-Sm) HCV, hepatitis C virus LKM, anti-liver-kidney microsomes antibodies SMA, anti-smooth muscle antibodies. From Ramos-Casals M, Font J. Extrahepatic manifestations in patients with chronic hepatitis C virus infection. Curr Opin Rheumatol 2005 17 447-55 with permission. Abbreviations AMA, antimitochondrial antibodies ENA, antiextractable nuclear antigens (anti-Ro...

Mouse Models

Animal models that resemble pSS are used to evaluate etiology and pathogenesis of SS. Several models have provided some insight into potential genetic contribution to clinical manifestations of the disease and are reviewed in detail elsewhere.131 To date, there is no single mouse model that fully recapitulates the majority of cardinal disease manifestations of human disease. A large proportion of the approximately 20 models available, however, develop sialoadenitis or dacryoadenitis, so these...

Related Autoimmune Diseases

In humans, clustering of autoimmune diseases such as SLE, RA, AITD, psoriasis, multiple sclerosis, and SS within families frequently has been documented.18 Autoimmune serologic abnormalities are frequent (up to 55 , depending on the antibody specificity) in otherwise healthy family members.19 Sharing of clinical and serologic features among related diseases also occurs. For example, subsets of patients who have SLE or SS may share similar symptoms (commonly including arthralgias, myalgias,...

Peculiarities Of The Clinical Spectrum Of Ss

Primary SS is a systemic autoimmune disease that mainly affects the exocrine glands and commonly presents as persistent dryness of the mouth and eyes because of functional impairment of the salivary and lachrymal glands. However, other epithelial organs may be involved, including liver, lung, and kidney. The histologic hallmark of SS is lymphocytic infiltration of the involved tissues (exocrine and nonexocrine epithe-lia). In early lesions the inflammatory infiltrates are mainly comprised of...

Aids

Use of anticholinergic drugs (since a time shorter than fourfold the half-life of the drug) From Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjogren's syndrome a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002 61(6) 557 with permission. syndrome was present years before the other primary disorder. The possibility of association or overlap of two autoimmune conditions is not taken into account. However,...

Inflammatory Mediators

The known CNS effects of inflammatory cytokines regulated by interferon include behavioral disturbances. For example, interleukin (IL)-1 can induce slow-wave sleep, loss of appetite, and enhanced production of corticotrophin releasing hormone (CRH). In patients with hepatitis C infection (HCV), the severity of depressive symptoms is correlated with expression of proinflammatory cytokines.106 A variety of inflammatory cytokines and chemokines, including IL2, IL6, IL10, interferon-alpha (IFN-a),...

Potential Approaches To Treatment Of Fatigue And Other Extraglandular Symptoms

The most commonly used medication in our experience to treat fatigue and joint muscle pain in PSS is hydroxychloroquine. Fox and colleagues30 reported improvement in arthralgia and myalgia (five-point Likert scale) in an open-label retrospective study in 50 patients treated for a minimum of 2 years with hydroxychloroquine 6 to 7 mg kg d. Kruize and colleagues31 in 2003 performed a small crossover study in 19 patients over 2 years (12 months on placebo or hydroxychloroquine 400 mg d switched to...

Assessment Of Salivary Gland Function

Determination of a salivary flow rate or evaluation of salivary function is intrinsically a crude measurement that is probably best for identifying those individuals who are at either extreme of salivary production, that is either too little or plenty of saliva. This determination may be done by salivary scintigraphy or by the cost- and time-efficient method of spitting into a preweighed tube according to a specific protocol (Boxes 1 and 2). To date, researchers have been unable to precisely or...

Lymphomagenesis in Hepatitis C Virus and Sjogrens Syndrome Patients

Lymphomagenesis in HCV patients might be initiated by the chronic stimulation of polyclonal B cells by the virus51 and the compartmentalization of HCV quasispecies in blood mononuclear cells,52 with the subsequent development of specific B-cell clonal expansions53,54 and procarcinogenic mutations.55,56 Vallat and colleagues54 suggested that B-cell clonality in the blood and liver may be a marker of lymphoma development in some HCV patients. Machida and colleagues55 reported that both acute and...

Pathogenic Autoantibodies

Autoantibodies potentially contribute to neuropathology by a variety of mechanisms, including immune complex mediated vasculitis, direct targeting of surface antigen leading to injury to myelin or neuronal tissue, and targeting of postganglionic receptors by functional autoantibody. Antiphospholipid antibodies (aPL) are associated with stroke, migraine, seizures, and transverse myelitis.73 Although described in 5 to 14 of pSS patients, aPL antibody seems not to be associated with CNS disease in...

Promotion Of Remineralization And Reversal Of The Demineralization Process

Sjogren Wound

The early carious lesion can be reversed by the remineralization process. The use of fluoride is critical for the process of remineralization. The action of fluoride is considered to be topical, promoting remineralization and inhibiting demineralization of teeth. Additionally, it may inhibit plaque bacteria. There are many brands and types of topical fluorides available that can be professionally or self-applied. For those at lowest risk, a 0.05 sodium fluoride rinse (ACT rinse, Fuoriguard,...

Focal Central Nervous System Disorders

The clinical profile of CNS abnormalities described in association with pSS is similar to that reported in CNS lupus, and includes optic neuropathy, hemiparesis, movement disorders, brainstem and cerebellar syndromes, recurrent transient ischemic attacks, and motor neuron syndrome. Differentiation between SLE and pSS with CNS involvement can be particularly difficult, as focal neurologic deficits, oligoclonal bands in the cerebral spinal fluid CSF , and abnormalities on MRI are detected in both...

Present Understanding Of Dry Eye Disease And Implications For Therapy

Nomenclature Eye Picture

A recent international workshop designed to collate and critique in an evidence-based manner the accumulated knowledge concerning dry eye disease and its treatment published a comprehensive report in 2007.1 The Dry Eye Workshop provided guidance to clinicians and researchers regarding the definition, classification, epidemiology, and research findings, and treatment guidelines for dry eye disease. The consensus definition of dry eye disease was stated to be that dry eye is a multifactorial...