Inflammatory Muscle Disease

The presence of myositis in primary Sjogren's syndrome was studied by two groups in recent years While Lazarus and Isenberg5 detected only 1.8 of myositis overlap in their British cohort of Sjogren's syndrome patients, Lindvall and colleagues31 found that 14 of their Sjogren's syndrome patients suffer from myositis, when muscle involvement was studied both clinically and histologically. There are no reports from larger myositis cohorts that define the prevalence of Sjogren's syndrome in primary...

Autoimmune Thyroid Disease

The overlap between systemic autoimmune diseases and autoimmune thyroid disease is well known. Most studies, however, are from a time before the publication of the AECC criteria for primary Sjogren's syndrome and thus difficult to compare. Among more recent publications, the prevalence of autoimmune thyroid disease was 10 among 400 patients with primary Sjogren's syndrome followed in Hungary. Seven percent had Hashimoto thyroiditis and 3 Graves disease, about twice as frequent as in the...

Measurement Of Disease Activity And Damage In Different Systemic Autoimmune Diseases

Because specific clinical or biologic markers for the assessment of the degree of overall activity or cumulative damage in systemic autoimmune diseases do not exist, the measurement of these disease status entities can be facilitated by developing specific criteria for each disorder.2-4 Such instruments are mainly designed to precisely assess activity or damage in patients enrolled in controlled therapeutic trials, but can also be used in daily clinical care. Disease status criteria for...

The Italian Experience

An effort was first made by an Italian group to define activity and damage criteria for SS.19 To accomplish this, the method used to develop ECLAM (European Consensus Lupus Activity Measurement) was applied.20 Data was collected on a total of 206 patients recruited in 12 Italian centers. This data was used to build two multivariate models predictive of disease activity and damage, respectively. The PGA, recorded by observers for both activity and damage, represented the dependent variables for...

The British Experience

Contemporaneous with the Italian group, a British group initiated the development and validation of an activity index to assess systemic features of SS,21 based on a modified version of the previously developed BILAG (British Island Lupus Activity Group) index for systemic lupus.22 BILAG is a multidimensional nominal scale that includes eight domains that are scored separately according to changes that have occurred since the previous observation. The stratification of the domains included in...

Of North America

Sjogren's syndrome (SS) is atypical among the major autoimmune rheumatic diseases encountered by the rheumatologist. The predominance of ocular, oral mucosal, and exocrine gland pathology accompanied by the copresence of multiple organ-specific autoimmune disorders results in a somewhat unfamiliar diagnostic and therapeutic landscape. The lack of universally accepted diagnostic, classification, and outcome criteria, as well as the need for close collaboration with ophthalmologic, dental, and...

Fatigue

Fatigue is also commonly reported by patients with other rheumatic diseases, such as rheumatoid arthritis and systemic lupus erythematosus.2-4 Furthermore, fatigue is relatively common in the general population9 and can present in association with certain medical conditions (eg, hypothyroidism), or with psychiatric diseases (eg depression), or be medically unexplained (eg, chronic fatigue syndrome).9 One useful model, therefore, considers the interrelationships of biological (eg, disease...

Characterization ofBCell Lymphoma in Sjogrens Syndrome Hepatitis C Virus Patients

A recent study60 has firstly described the disease characteristics of B-cell lymphoma in SS-HCV patients, its treatment, outcome, and survival prognosis. Compared with SS-HCV patients without lymphoma, those with lymphoma had a higher frequency of parotid enlargement and vasculitis.60 In primary SS, parotid enlargement is considered as a highly suggestive clinical sign of lymphoma,3,61 whereas vasculitic features are closely associated with cryoglobulinemia62 and lymphoma,61,63 with...

References

Vivino FB, Al-Hashimi I, Khan Z, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjogren syndrome a randomized, placebo-controlled, fixed-dose, multicenter trial. P92-01 Study Group. Arch Intern Med 1999 159(2) 174-81. 2. Petrone D, Condemi JJ, Fife R, et al. A double-blind, randomized, placebo-controlled study of cevimeline in Sjogren's syndrome patients with xerostomia and keratoconjunctivitis sicca. Arthritis Rheum 2002 46(3) 748-54. 3. Fox PC,...

Assessment Of Oral Mucosa

The face is observed for fullness suggestive of salivary gland enlargement. Speech is assessed for the smacking sound of the dry oral mucosa sticking to the teeth. The lips are observed for dryness, fissuring, erythema, ulceration, and swelling. The mouth is examined starting with the labial mucosa, the buccal mucosa, the hard soft palate, the gingiva, the tongue, and the floor of mouth. A piece of gauze is used to dry the floor of the mouth to determine whether pooling occurs as saliva exits...

D

Common objective signs of dry eye disease used to grade severity. (A) Tear break-up time (fluorescein). (B) Conjunctival staining with lissamine green. (C) Corneal staining with fluorescein. (D) Filaments on the cornea in areas of confluent fluorescein staining. Treatment recommendations by severity level Education and environmental and dietary modifications Elimination of offending systemic medications Artificial tear substitutes, gels and ointments Eye lid therapy If level 1...

Management Of Dental Caries

A seminal study showed that for individuals who have compromised salivary function, excellent oral hygiene alone is insufficient to prevent rapidly progressive dental decay.12 The use of topical fluoride and diet counseling was critical for caries prevention. Protocol for determination of unstimulated whole salivary flow rate Unstimulated whole saliva collection measures saliva production under resting or basal conditions. The subject should not have had anything to eat or drink for 90 minutes...

Oral Immunosuppressive Agents

Methotrexate (MTX) has been used successfully for the treatment of RAfor 25 years. Little is known regarding the effect of MTX on secondary SS in patients who have RA. Skopouli and colleagues14 evaluated the safety and efficacy of MTX for primary SS in a 1-year, open-label pilot trial involving 18 patients one dropped out after 1 month. All patients met European Community criteria and had a positive minor salivary gland biopsy. Patients received 0.2 mg kg (10-15 mg) of MTX weekly. Significant...

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...

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

Sjogren Brain Mri

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

Dry Eye Syndrome

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...