Sjögren's syndrome

Sjögren's syndrome (SS) is a chronic autoimmune disease affecting the exocrine glands, primarily the salivary and lacrimal glands. It has been suggested that exogenous agents may trigger SS in genetically predisposed individuals. However, at present, the etiology of SS is far from being understood, and no direct evidence for any of these triggers has been presented. The salivary and lacrimal glands from patients with SS harbor unique and highly selected T‐ and B‐cell populations. Disturbance in glandular cell apoptosis may be one possible explanation for the sicca symptoms in SS. However, discrepancies between glandular destruction and salivary flow give rise to processes causing glandular dysfunction preceding or triggering glandular cell destruction. Recent reports suggested autoantibodies inhibiting neuronal innervation of acinar cells and defective water transport to be implicated in salivary secretion deficiency observed in SS. Several types of autoantibodies have been suggested to contribute to the pathogenesis of SS. However, how the tolerance to these structures is broken down is unknown at present. Studies on B‐cell activating factor indicated that diminished apoptosis and disturbed B‐cell maturation could be responsible for the occurrence of autoreactive B‐cells and B‐cell hyperreactivity. B‐cell activation may also provide a basis for lymphoma development observed in up to 5% of the patients with SS.     

Oral manifestations of Sjögren's syndrome

Sj?gren's syndrome is a common autoimmune rheumatic disease. The most common symptoms of Sj?gren's syndrome are extreme tiredness, along with dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). Saliva plays an essential role in numerous functions of the mouth. Xerostomia can be caused by medications, chronic diseases like Sj?gren's syndrome, and medical treatments, such as radiation therapy and bone marrow transplant. Xerostomia can eventually lead to difficulty in swallowing, severe and progressive tooth decay, or oral infections. Despite having excellent oral hygiene, individuals with Sj?gren's syndrome have elevated levels of dental caries, along with the loss of many teeth, early in the disease. Sj?gren's syndrome alters the protein profile and brings about a change in the composition of saliva. There is an increase in the levels of lactoferrin, beta(2)-microglobulin, sodium, lysozyme C, and cystatin C, and a decrease in salivary amylase and carbonic anhydrase. Up to 90% of individuals with Sj?gren's syndrome have antibodies targeting the Ro 60 and La autoantigens. Natural aging, regardless of Sj?gren's syndrome, is also another factor that brings about a significant change in the composition of saliva. The most prevailing cause of xerostomia in elderly persons is the use of anticholinergic medications. Currently, there is no cure for Sj?gren's syndrome, and treatment is mainly palliative.     

Primary Sjögren's syndrome

Columbia University's Salivary Gland Center (SGC) has examined more than 6,000 patients with a variety of concerns stemming from salivary gland disease and/or salivary secretory dysfunction. Not unexpectedly, the most common patient complaint centers around symptoms associated with dry mouth. Such patients are usually first seen by the dental practitioner. Because Sj?gren's syndrome (SS) causes dry mouth, and because it is a relatively common entity--encountered in about three million Americans--and because the dental profession has become aware of its classic manifestation of xerostomia, patients experiencing SS are referred in increasing numbers to the SGC for evaluation. Therefore, the authors wish to call attention to the methodology used in accurately diagnosing SS and to illustrate its signs and symptoms with a case report.     

Salivary electrophoresis in the diagnosis of Sjögren's syndrome

Objective. The purpose of this study was to investigate the potential use of salivary electrophoresis for the diagnosis of Sjögren's syndrome.Methods. Salivary protein profiles of 43 patients and 39 healthy control subjects were compared on three different gel electrophoresis systems: sodium dodecylsulfate-polyacrylamide gel electrophoresis, anionic polyacrylamide gel electrophoresis, and immobilized pH gradient gel electrophoresis (isoelectric point, 3.5−5.0).Results. Most of the patients with Sjögren's syndrome exhibited an electrophoretic profile that was different from that of the healthy control subjects. Among the three gel electrophoresis systems examined, the immobilized pH gradient system appeared to be the most reliable for Sjögren's syndrome. Tests of accuracy revealed that the immobilized pH gradient system exhibits high specificity (97%), sensitivity (95%), positive predictive value (97%), and negative predictive value (95%) in the diagnosis of Sjögren's syndrome.Conclusions. Our results suggest that salivary electrophoresis is a potentially useful test for the diagnosis of Sjögren's syndrome.     

Dental and periodontal status of Sjögren's syndrome

Background: Sjögren's syndrome (SS) is one of the most common systemic autoimmune diseases in middle‐aged women. The present study had the aim to examine the dental and periodontal condition in patients with SS in comparison with disease controls and to evaluate the influence of reduced salivary flow in the periodontal tissues. Method: We examined 24 patients with primary or secondary SS in comparison with 27 patients who had another autoimmune disease but no signs or symptoms of SS, as well as with 29 subjects who had a subjective feeling of xerostomia or xerophthalmia without exhibing an underlying disease. The clinical evaluation included examination of the oral mucosa, determination of missing, decayed and filled teeth, fixed or removable prosthetic appliances, plaque index, gingival index, probing pocket depth, probing attachment level, oral hygiene habits and frequency of dental visits. Statistical analysis was performed using the 2‐tailed Fisher exact and Kruskal‐Wallis tests. Results: No significant difference was found in the dental or periodontal condition of the 3 groups. The number of teeth, feelings and distal or mesial decay lesions correlated negatively with age, while the number of fixed prosthetic appliances correlated positively. The salivary flow was statistically lower in patients with SS and exhibited a negative correlation with the number of cervical decay lesions. It was also found that SS patients had better oral hygiene habits than subjects of the control groups. Conclusions: No significant difference could be detected concerning the dental and periodontal status of SS patients, compared with that of patients with other immune diseases as well as with that of controls who had subjective xerostomia.     

Oral and dental aspects of Sjögren's syndrome

Sj?gren's syndrome is a common condition which can result in significant physical and emotional debility. Dentists can play a pivotal role in the prompt diagnosis, investigation and management of patients with Sj?gren's syndrome. A sound understanding of the pathogenesis, presentation and current management of Sj?gren's syndrome, will enable the general dental practitioner to make a significant contribution to the oral health and general well-being of those affected by the disease. This article aims to provide the general dental practitioner with a comprehensive and practical guide to current developments and best practise in the care of these individuals.     

Usefulness of mouse models to study the pathogenesis of Sjögren's syndrome

Sjögren's syndrome (SS) is an autoimmune disorder characterized by ocular and oral dryness as well as systemic manifestations. The immunopathogenesis of SS is complex with different intricate factors. Because of the delay in the appearance of symptoms and due to ethical issues it is very difficult to study the wide array of factors intervening in the pathogenesis of SS in human patients. To circumvent this problem, different animal models have been elaborated for studying the different subsets of the aspects of the physiopathology of this disease. In this review, we focus on the mouse models that have been established to deepen our insight into the immunopathogenesis of SS.     

Lip biopsy for the diagnosis of Sjögren's syndrome: beware of the punch

A pilot study was performed to examine the presence of nerve fibres in minor salivary gland tissue samples obtained by two procedures: punch and linear incisional techniques. The study was undertaken on a convenience sample of five cryopreserved corpses (mean age 74 ± 3.5 years; three males and two females). Biopsies were performed on the buccal side of the lower lip, between the mid-line and the corner of the mouth. Each corpse had one side of the lower lip biopsied by punch and the contralateral side using a linear incision. All punch samples (100%) displayed severed nerve fibres, whereas no nerve fibres (0%) could be identified in the samples obtained by means of the linear incision technique. While the linear incision approach permitted retrieval of at least five glands, punch biopsies did not provide enough material for the diagnosis of Sjögren's syndrome. Within the limitations of the study, our results strongly discourage the punch technique for minor salivary gland lip biopsy and provide information on the superiority of the linear incisional biopsy in terms of neural damage. These results may also promote the undertaking of clinical trials on patients in whom Sjögren's syndrome is suspected, comparing the morbidity associated with the linear incisional technique vs. minimally invasive biopsies.     

Current issues in Sjögren's syndrome

Sjögren's syndrome is a chronic autoimmune and rheumatic disorder with prominent sicca complaints from the mucous membranes because of lack of proper exocrine secretions. There is no straightforward and simple diagnostic test for Sjögren's syndrome, although several classification criteria have been designed including several oral diagnostic tests. A new set of classification criteria in a joint effort by research groups in Europe and USA has recently been presented. A large number of autoantibodies have been reported in Sjögren's syndrome where, in some cases, the antibodies are correlated with the extent and severity of disease. The finding of serum autoantibodies directed against the muscarinic M3 receptor is an important advance in understanding the pathogenesis of not only the impaired glandular function but also associated features of autonomic dysfunction in some patients. The treatment of primary Sjögren's syndrome is still mainly symptomatic.     

Multiple calcifications within the parotid gland of patients with Sjögren's syndrome

PurposeThe purpose of this study was to investigate computed tomography (CT) and clinical features relating to calcifications within the parotid gland of patients with Sjögren's syndrome (SS).MethodsData from 30 patients with SS who had been examined by CT were extracted from our radiological information database accumulated from 2001 to 2011, and their CT images were reread carefully. Of these patients, 14 (all female; age range 20–95 years; mean age 61.4 years) with calcifications within the parotid gland were retrospectively investigated with CT findings. The relationship between calcification occurrence and clinical symptoms including parotid swelling and/or saliva colic was investigated. The degree of destruction of the parotid gland on CT images was also evaluated.ResultsAll calcifications of 14 patients were located within the parotid gland, not in the parotid duct. CT images of all calcifications showed small and regular round shapes. Multiple occurrences of calcifications were recognized in 10 patients, and a solitary occurrence was seen in 4 patients. Seven patients had bilateral calcifications. There was little relationship between the occurrence of calcifications and clinical symptoms, and the severity of destruction of the parotid gland.ConclusionThe presented CT and clinical features would be peculiar to SS because too many patients lacked the typical features of sialoliths within the parotid gland.     

A current perspective on Sjögren's syndrome

Sj?gren's syndrome is a common autoimmune disorder characterized by dry mouth and dry eyes. Symptoms and signs are chronic and can be severe. The diagnosis of Sj?gren's syndrome can be confusing and time-consuming. The management can also be a significant challenge for the clinicians. However, recent genomic and proteomic developments are unlocking the mystery of the disease process as well as contributing to our ability to define, diagnose, and develop new treatment modalities for patients with this complex disorder.     

Salivary anti-spectrin autoantibodies in Sjögren's syndrome

OBJECTIVE: The purpose of this study was done to compare the anti-spectrin autoantibody levels in the parotid saliva of Sj?gren's syndrome patients and in the parotid saliva of healthy control subjects. METHODS: The salivary anti-spectrin autoantibody levels of 20 Sj?gren's patients and of 20 healthy controls were compared by means of the slot blot immunoassay and the alkaline phosphatase method. RESULTS: Various anti-spectrin autoantibody levels were detected in the saliva of both patients and controls. The color intensity of the blots was scored on a scale of 1 to 3. The scores were deemed to indicate the anti-spectrin autoantibody levels in saliva (1 = low, 2 = moderate, and 3 = high). The Mann-Whitney U test did not reveal a significant difference in the anti-spectrin autoantibody levels of patients and the anti-spectrin autoantibody levels of controls (P > or = .31). These results do not support a pathologic role for anti-spectrin autoantibody in Sj?gren's syndrome. CONCLUSIONS: The overall result of this study substantiates that anti-spectrin autoantibodies occur naturally in saliva. Their role in immune surveillance or pathology is not clear at present.     

Periodontal disease in primary Sjögren's syndrome

Occurrence of periodontal disease in Sjögrens's syndrome (SS) is still controversial. OBJECTIVE: To examine if the risk of gingival and periodontal conditions was increased in SS compared to the general population. MATERIALS AND METHODS: Fifty‐seven patients (4 men, 53 women) with primary Sjögren's syndrome (Copenhagen criteria) and an age‐matched representative sample of the general population of 80 controls (all women) were examined for gingival and periodontal disease. RESULTS: Gingival bleeding and supra‐gingival calculus did not differ among SS patients and controls. Subgingival calculus occurred more often among the younger SS patients than controls, but did not differ among the older SS patients and controls. Periodontal pockets of 4–5 mm as well as pockets >5 mm occurred with similar prevalences among the two groups. Smoking habits did not influence the results. The health status of the gingival and periodontal tissues were thus similar in SS and controls. CONCLUSION: Primary SS is not associated with increased risk of periodontal disease.     

Prevalence of periodontal disease in patients with Sjögren's syndrome

The periodontal status of 25 patients with Sjögren's syndrome was compared with that of an age-, sex-, and race-matched healthy controls. Clinical evaluation included determination of number of teeth, decayed/missing/filled surfaces, probing pocket depth, clinical attachment level, plaque index, gingival index, bleeding on probing, and measurement of alveolar bone level (examined with radiography). Results of the study suggest that patients with Sjögren's syndrome have a significantly higher plaque index score (p < 0.005), higher decayed/missing/filled surfaces scores (p < 0.05), increased alveolar bone loss (p < 0.05), deeper clinical attachment level (p < 0.05), and increased cementoenamel junction-alveolar bone crest distance (p < 0.005). Although no significant difference was found in the number of cases of “established periodontitis” between the Sjögren's syndrome and controls, odds ratio analysis suggests that patients with Sjögren's syndrome are at 2.2 times higher risk of having adult periodontitis than healthy controls.