Sj?gren's syndrome.

Sj?gren's syndrome (SS) is a progressive autoimmune rheumatic disorder. Its precise etiology is unknown, although several contributing factors have been identified. One theory is that the condition results from complications related to infection with the Epstein-Barr virus. Primary exposure to or reactivation of Epstein-Barr virus elicits expression of the human leukocyte antigen complex. This is recognized by T lymphocytes (CD 4+) resulting in the release of cytokines (tumor necrosis factor, interleukin-2, interferon-gamma, and others). A genetic marker specific for Sj?gren's syndrome, HLA-DR4, has been identified. According to the World Health Organization, the prevalence of Sj?gren's syndrome is unknown. A recent epidemiologic study in Sweden estimated the prevalence in the adult population to be 2.7%. In the United States, 10 years ago, the number of patients with Sj?gren's syndrome was thought to be fewer than 100,000. This number today is estimated to be more than 1 million. Sj?gren's syndrome has been reported in nearly every major country of the world, and the geographic distribution of cases appears to be relatively uniform. Sj?gren's syndrome typically affects women (90%) during the fourth or fifth decade of life. Isolated cases of Sj?gren's syndrome in children have been reported.     

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.     

Salivary gland involvement in autoimmune thyroiditis, with special reference to the degree of association with Sj?gren's syndrome.

From a total of 63 patients with autoimmune thyroiditis, 19 cases were further investigated to determine the degree of concomitant morphologic and functional salivary gland changes. For comparison, 21 of a total of 28 cases of primary Sj?gren's syndrome were also examined. Of the 19 cases of autoimmune thyroiditis, 11 showed various degrees of salivary gland involvement on the basis of an analysis of lower lip salivary gland biopsy specimens, scintigraphy of the parotid, and unstimulated whole sialometry. Six of these cases fulfilled the criteria of primary Sj?gren's syndrome. A remarkably high proportion of dark-staining acini was observed in the lower lip biopsy specimens of our patients with thyroiditis (8 of 19, 42%) and less among our patients with primary Sj?gren's syndrome (5 of 21, 24%). We conclude that significant involvement of salivary glands may occur in cases of autoimmune thyroiditis, which indicates that common mechanisms may frequently be operative in the development of thyroid and salivary gland immune disease.     

Sarcoidosis (Heerfordt syndrome): a case report

We report the case of a 22-year-old woman who is suspected of having primary Sj?gren s syndrome. She complaining of bilateral swelling of eyelids and the parotid glands of three weeks duration. Physical examination revealed a bilateral enlargement of both parotid glands, which were solid and painful. Sj?gren s syndrome was suspected at that stage, and the serologic and specific analysis were done. All these tests didn t find any autoimmune or visceral features typical of Sj?gren s syndrome and autoantibodies were negative. During follow-up time the right facial nerve palsy developed. Pulmonary radiography revealed bihilar lymphadenopathy and labial salivary gland biopsy revealed non-caseating granuloma. The patient was classified as having stage I sarcoidosis. This case demonstrates the importance of being aware of the leading clinical signs and symptoms in case of Heerfordt syndrome.     

Histochemical study of labial salivary glands in Sj?gren's syndrome.

Human labial salivary gland biopsies of patients presenting connective tissue diseases associated with Sj?gren's syndrome were submitted to a polysaccharide histochemistry study. The normal acinar secretion is an association of neutral polysaccharides with a sulphosialomucin. In Sj?gren's syndrome, there is a great reduction in the secretory activity of the acinar cells, but no qualitative change was observed. The pathogenesis of this decreased production and its importance regarding the clinical manifestations of Sj?gren's syndrome are discussed.     

Cytokine concentrations in stimulated whole saliva among patients with primary Sjögren's syndrome, secondary Sjögren's syndrome, and patients with primary Sjögren's syndrome receiving varying doses of interferon for symptomatic treatment of the condition: a preliminary study

Sj?gren's syndrome is an autoimmune disorder which causes diminished salivary flow due to autoimmune sialoadenitis. This decrease in saliva flow is the result of inflammation and atrophy of the salivary glands. Most treatment regimens are palliative in nature, but treatment with interferon (IFN) holds promise for Sj?gren's syndrome sufferers. Several studies have investigated cytokine concentrations in the salivary glandular tissues from Sj?gren's syndrome patients; however, there is little information concerning cytokine expression in saliva. This is especially true with respect to treatment modalities and their effects on local cytokines. A clinical study was conducted to determine salivary interleukin (IL)-6, IFN, and IL-2, concentrations among subjects diagnosed with primary and secondary Sj?gren's syndrome and a healthy control group. The primary Sj?gren's syndrome showed significantly higher salivary IL-2 and salivary IL-6 than the control and secondary Sj?gren's groups. There were no between group differences for salivary IFN concentrations. In addition, the study assessed salivary IL-6, IFN, and IL-2 concentrations among 18 Sj?gren's syndrome patients before and after administration of IFN via the oral mucosal route. The results of the study showed that the mean values for the pre- and post-treatment groups for stimulated whole saliva flow rates were 3.15 and 3.74 ml/5 min, respectively. The post-treatment group exhibited a 16.8% increase in stimulated whole saliva flow rates. The salivary IL-6 concentration was 53.3% lower for the post-treatment group (17.79) as compared to the baseline value (33.35). The values for salivary IFN and salivary total protein were virtually unchanged from their baseline values. Salivary IL-2 values, however, were 50% lower in the post-treatment group (3.07) when compared to their respective baseline values (6.10). The results of this study suggest that healthy individuals exhibit lower salivary IL-2 and IL-6 as compared to individuals suffering from primary and secondary Sj?gren's syndrome. The results also suggest that administration of IFN via the oral mucosal route may increase salivary flow rates and depress certain cytokines (IL-2, IL-6) associated with inflammatory destruction of salivary glandular tissues in Sj?gren's syndrome patients.     

Oral and periodontal status in Sj?gren's syndrome.

Sj?gren's syndrome is a multi-system chronic inflammatory disease of the exocrine glands. Inflammation of the salivary glands leads to reduction in salivary output, which imposes a significant impact on oral health. Dentists and dental hygienists are the primary healthcare providers to identify early signs and symptoms of Sj?gren's syndrome. Early diagnosis of Sj?gren's syndrome is fundamental for effective management of the disease.     

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 IgA in Sj?gren patients

Salivary flow-rate, Na, K and IgA were measured in 12 KCS patients for diagnosis of Sj?gren's syndrome. 8 had elevated salivary IgA concentrations. The IgA was analysed on anti-IgA plates and on anti-secretory IgA plates. The results indicate that the elevation in salivary IgA in Sj?gren patients is due to secretory IgA.     

Sj?gren's syndrome: clinical and laboratory features, immunopathogenesis, and management

Sj?gren's syndrome may be accompanied by local oral problems such as dry mouth, rampant caries, candidosis, or sialadenitis, but it is a systemic autoimmune disorder with wide repercussions, including a small premalignant potential. This article reviews the clinical and immunopathogenic features, as well as the etiology, of Sj?gren's syndrome and discusses the diagnosis and management of oral complications.     

Oral lichen planus and Sjogren's syndrome. 2 cases of association

We described two female patients, aged 55 and 49 years respectively, who fulfilled the diagnostic clinical and histological criteria for lichen planus and Sj?gren's syndrome. The association of either lichen planus or Sj?gren's syndrome to autoimmune disorders seems not to be fortuitous and common pathogenic mechanisms have been proposed. In the literature, however, the association between lichen planus and Sj?gren's syndrome has been infrequently reported.     

Primary sialoangiectasia--a diagnostic pitfall in Sj?gren's syndrome: case report.

A case of primary sialoangiectasia, which in this case was initially misdiagnosed as Sj?gren's syndrome, is described. Other diseases, including HIV infection, psoriatic arthritis, and acute parotitis, may cause glandular changes similar to the changes found in the syndrome. Therefore, sialography must be combined with other methods of assessment of the oral cavity when suspicion is high for Sj?gren's syndrome. Properly applied, sialography provides essential information regarding the severity of glandular damage and the progression of the disease.     

常见口腔黏膜疾病患者行种植修复相关问题的研究进展

随着修复技术的发展及人们对生活质量要求的提高,伴有缺牙的口腔黏膜病患者行种植修复的需求越来越高.受口腔黏膜病困扰的缺牙患者行种植修复是否可行,效果如何目前并无定论,亦无针对口腔黏膜病患者行种植修复的相关治疗指南.本文就常见的口腔黏膜病(如口腔扁平苔藓、自身免疫性大疱性疾病和舍格伦综合征等)患者行种植修复的相关研究进展进...     

Effect of Sialor in treatment of xerostomia in Sj?gren's syndrome

Sialor, a sialogogue, was studied in a double-blind crossover trial in patients with autoimmune exocrinopathy (Sj?gren's syndrome). Sialor appears to frequently alleviate the symptoms of xerostomia and increase salivary flow rate, and it is rarely associated with side effects. Sialor is useful in management of xerostomia in patients with Sj?gren's syndrome and may also be of value in the treatment of xerophthalmia.     

Sialochemistry in Sj?gren's syndrome.

The value of sialochemistry in the study of Sj?gren's syndrome was explored by comparative examination of a spectrum of parotid components in twelve subjects with a positive diagnosis of the disease and twelve control subjects with normal gland function. The subjects with Sj?gren's syndrome all exhibited a marked reduction in flow rate and phosphate concentration and a marked elevation in sodium and chloride concentration. The concentration of IgA was somewhat elevated (commensurate with reduced flow rate); the levels of IgG, IgM, and albumin were normal. The major functional abnormality in the parotid gland in Sj?gren's syndrome appears to be luminal transport in the ductal region; leakage of serum components is minimal. Sialochemistry can be helpful in differentiating Sj?gren's disease from other diseases of the salivary gland and in assessing degree of pathologic change.     

腮腺舍格伦综合征与非霍奇金淋巴瘤相关性分析

目的:了解腮腺非霍奇金淋巴瘤与舍格伦综合征的临床及发病机制的相关性，正确诊断和治疗舍格伦综合征，尽早明确有无恶性变。方法：对142例口腔颌面部的非霍奇金淋巴瘤中21例发生在腮腺的非霍奇金淋巴瘤，及3例从合格伦综合征演变成淋巴瘤的病例进行分析。结果：21例腮腺区非霍奇金淋巴瘤的局部表现主要为肿块、反复肿胀，与类肿瘤型舍格伦综合征的一般特征和腮腺表现有相关性，其中3例腮腺淋巴瘤患者有明确的舍格伦综合征病史。结论：舍格伦综合征与腮腺非霍奇金淋巴瘤的发生发展，以及临床表现有相关性，部分类肿瘤型舍格伦综合征可演变为淋巴瘤，临床表现和免疫学改变可早期判断舍格伦综合征有无恶性变。     

风湿免疫疾病患者口腔健康状况调查分析

目的通过流行病学调查方法了解风湿免疫患者口腔健康状况,为风湿免疫患者口腔疾病的预防与治疗提供依据。方法对中国医科大学附属盛京医院风湿免疫科病房309例患者进行口腔专科检查,填写调查问卷,检查结果采用SPSS软件进行统计学分析。结果风湿免疫疾病患者口腔健康者不足4%,牙周炎患病率为91.9%,患龋率为67.2%。口腔卫生与性别、年龄、学历、口腔护理情况及吸烟显著相关;牙周病的发生与年龄、口腔护理、吸烟相关;患龋率高低与性别、年龄、吸烟相关。风湿免疫疾病中,牙周炎发病率最高的是骨关节炎、皮肌炎及类风湿性关节炎;舍格伦综合征患者患龋率最高。结论口腔健康水平可能与风湿免疫疾病相关,应从多角度分析风湿免疫疾病患者口腔健康不良原因,采取积极预防措施。     

Diagnostic value of sialography with both the conventional and digital subtraction techniques in children with primary and secondary Sj?gren's syndrome.

OBJECTIVE: The application of anamnestic data on siccative symptoms required for classifying adult Sj?gren's syndrome is limited in childhood. Instrumental test procedures are therefore necessary for objectively recording the oral and ophthalmologic manifestations of the disease. The aim of this study was to clarify the sialographic changes that occur in Sj?gren's syndrome in children. STUDY DESIGN: A total of 23 sialograms were obtained with both conventional and digital subtraction techniques in 21 children with primary (10 girls and 1 boy) or secondary Sj?gren's syndrome (10 girls). The films were assessed by 3 physicians and submitted for a consensus analysis if necessary. RESULTS: The pathologic features observed in the children varied from a slightly narrowed ductal system to multiple peripheral ductal ectasias and completely destroyed parenchyma. Sialographic examinations demonstrate that, with progressing disease, regression of acinar dilatations and rarification of the ductal system occur. CONCLUSION: The results show that the spectrum of sialographically recordable lesions in Sj?gren's syndrome in children is greater than is described thus far in the literature.     

Orofacial manifestations of mixed connective tissue disease with an uncommon serologic evolution.

Mixed connective tissue disease is a multisystemic disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by a high titer of antibody to ribonucleoprotein. Orofacial manifestations of mixed connective tissue disease include trigeminal neuralgia-like pain, neuropathy, features suggestive of Sj?gren's syndrome, and lymphadenopathy. Our recent experience with one patient with trigeminal neuropathy, facial paralysis, Sj?gren's syndrome, and aseptic meningitis as early manifestations of the disease, together with an uncommon serologic evolution, is described.     

Connective tissue disorders and the mouth

The connective tissue disorders frequently give rise to orofacial manifestations, especially dry mouth because of Sj?gren's syndrome. In addition, the systemic complications of such diseases may impact upon the provision of oral health care. The present article reviews the consequences of connective tissue disorders of relevance to oral health care providers. Clinical Relevance: Connective tissue disorders can give rise to oral manifestations and systemic complications that may occasionally compromise primary oral health care.