Multiple microlithiasis in bilateral parotid glands as the initial clinical manifestation of primary Sjögren’s syndrome

We report a rare case of Sjögren’s syndrome (SS) with multiple microliths in the bilateral parotid glands. A 41-year-old man presented to our department with mild pain in the region of the right parotid gland. The dental examination was negative except for the parotid regions. The right region was moderately swollen and the left mildly swollen. Plain radiography revealed multiple calculi in the bilateral parotid glands. Ultrasonography showed heterogenic parenchyma, with microliths and cystic lesions in the parotid glands and heterogenic echotexture in the submandibular glands. Immunologic tests and the Schirmer test confirmed the diagnosis of SS. As the patient had no classic symptoms of SS, the bilateral multiple microliths were the first sign, facilitating the final diagnosis. Early diagnosis of SS is highly relevant because the proper therapy can be initiated. Adequate follow-up and, especially, control of the disease activity by identifying the predictive factors, are the primary objectives of SS management, enabling personalized treatment of this malignant disease. This case is a good example of how detection of calcifications in the bilateral parotid glands by plain radiography can help diagnose SS at an early stage.     

Primary Sjögren syndrome in childhood: report of a case and review of the literature

Sj?gren syndrome (SS) in childhood is a rare and possibly underdiagnosed condition. The purpose of this study is to report a case of primary SS (PSS) in a 4-year-old Venezuelan girl and to review the pertinent literature. The patient presented with bilateral recurrent parotid enlargement, predominantly on the right side. She did not complain of dry mouth or eyes; however, decreased stimulated salivary flow rate and positive Schirmer and rose bengal tests were obtained. Sialography, sonograms, and a computed tomography scan of the parotid glands revealed pathologic changes consistent with SS. Anti-SS-A and anti-SS-B antibodies were present. Evaluation for antibodies against cytomegalovirus, Epstein-Barr virus, and HIV rendered negative results. Histopathologic examination of incisional biopsies of the right parotid and labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. Taken together, these findings are consistent with the diagnosis of juvenile PSS. The salient features of this rare disease are summarized on the basis of a comprehensive review of the epidemiologic, clinical, and serologic findings of the previously reported cases of PSS in children.     

Sj?gren's syndrome and differential diagnosis of parotid gland hypertrophy

Some auto-immune systemic pathologies can first be detected through a very characteristic symptomatology which must be known for an early diagnosis. This is particularly the case with primary Sj?gren syndrome (sicca syndrome). Based on a well documented case, this article is a review of the etiopathogenesis, symptomatology and investigation of the Sj?gren syndrome. The differential diagnosis of the parotid gland hypertrophies is also discussed, based on a systematic approach.     

Imaging features of the lacrimal and salivary glands of patients with IgG4-related Mikulicz’s disease: a report of three cases

We present ultrasonographic (US) and magnetic resonance (MR) imaging features of the lacrimal and salivary glands of three patients with IgG4-related Mikulicz??s disease (IgG4-MD). US revealed punched-out, soap-bubble, or reticular patterns of parenchymal hypoechogenicity of the involved lacrimal, parotid, and submandibular glands. Conventional MR imaging features were less specific. However, apparent diffusion coefficients of many of the involved glands were reduced compared with healthy glands. We discuss possible histological changes in the affected glands that were attributable to the observed imaging features. We also discuss the differences in imaging features between IgG4-MD and Sj?gren??s syndrome (SS). We conclude that US may be a useful adjunct for diagnosing IgG4-MD and that MR sialography could effectively distinguish IgG4-MD from SS.     

Sjögren syndrome: a review for the maxillofacial surgeon

Sj?gren syndrome is a multisystemic condition that predominantly involves the salivary and lacrimal glands. Also known as sicca complex, the disease often is underdiagnosed and poorly understood. This article provides a comprehensive review on Sj?gren syndrome, with an emphasis on diagnosis and treatment modalities.     

Sjogren syndrome: a review of the literature and a case report

Sj?gren syndrome is one of the most prevalent autoimmune diseases in which the body's immune system mistakenly attacks its own moisture producing glands. Although Sj?gren syndrome occurs in all age groups in both women and men, women in their fourties are the most affected. Sj?gren's syndrome can occur alone or in the presence of another connective tissue disease, respectively called primary and secundary Sj?gren syndrome. When two of the three clinical hallmarks: keratoconjunctivitis sicca, xerostomia or connective tissue disease are present, Sj?gren 's syndrome should be considered. To confirm the diagnosis of Sj?gren's syndrome several tests are required. e.g. blood tests, ophthalmologic tests and oral tests. Rheumatologists have the primary responsibility for managing Sj?gren's syndrome. Other specialists can treat the related symptoms. The incidence of lymphoma is higher in patients with Sj?gren's syndrome than in the general population. Therefore patients must be monitored carefully for the development of related autoimmune diseases, lymphoma and other complications. Sj?gren's syndrome is serious but generally not fatal if complications are diagnosed and treated early.     

Sequential quantitative scintigraphy of parotid glands with chronic inflammatory diseases]

Sequential quantitative scintigraphy of parotid glands was performed in 16 cases with recurrent parotitis in childhood, 33 with chronic obstructive parotitis, 37 with Sj?gren's syndrome, 4 with sialadenosis and 30 normal controls. Uptake function was normal, but excretion was retarded in recurrent parotitis in childhood. Chronic obstructive parotitis exhibited that excretion was obstructed and the uptake and excretion function between bilateral parotids was marked different. In Sj?gren's syndrome, uptake was very low and excretion was heavily delayed. Sialadenosis showed that excretion was retarded and uptake function was not definitely involved. The possible mechanism of the scintigraphical features were analyzed combined with the histopathological findings.     

Relationship between sialographic findings of parotid glands and histopathologic finding of labial glands in Sj?gren's syndrome. Relation to clinical and immunologic findings.

We investigated a relationship between sialographic findings of parotid glands and histopathologic changes of labial glands, in relation to Sj?gren's syndrome. We examined 107 patients, suspected to have Sj?gren's syndrome because of dry mouth and dry eyes, in whom no other autoimmune diseases were diagnosed. Parotid sialography identified 37 patients with punctate, globular, cavitary, or destructive sialectasia (S-positive group). These sialographic findings were not seen in the other 70 patients (S-negative group). Among these patients, 54 demonstrated periductal lymphocytic infiltration in the labial glands (H-positive group), whereas the other 53 patients did not demonstrate this histologic finding (H-negative group). Significant differences in stimulated parotid salivary flow rate and incidence of keratoconjunctivitis sicca were observed between patients in the S-positive and the S-negative groups. Differences in the salivary flow rate and the incidence of keratoconjunctivitis sicca were also seen between patients in the H-positive and the H-negative groups, although the differences were not statistically significant. The rate of incidence of serum rheumatoid factor, anti-SS-A, and anti-SS-B antibodies were significantly higher in the S-positive and the H-positive groups than in their negative counterparts.     

Periductal lymphocytic infiltration of salivary glands in Sj?gren's syndrome with relation to clinical and immunologic findings

We studied 113 patients who were suspected to have Sj?gren's syndrome (SS) because they had dry mouth and dry eyes, and who were determined as not having any other autoimmune disease, to clarify the relationship of periductal lymphocytic infiltration of salivary glands to clinical and immunologic findings in relation to SS. Periductal lymphocytic infiltrations were observed in the labial and/or the parotid glands of 57 patients (P-group). The salivary glands of the other 56 patients did not demonstrate obvious histologic changes (N-group). The stimulated salivary flow rate of parotid glands of the patients in both the P- and N-groups was significantly reduced when compared with healthy persons. However, no difference in the flow rate was observed between the P- and N-groups. In contrast, the percentage of patients in the P-group with keratoconjunctivitis sicca was significantly higher than that of patients in the N-group. Percentage of gamma-globulin fraction and IgG level in the sera of the patients in the P-group were both significantly higher than those in the N-group. The percentages of patients who demonstrated the serum rheumatoid factor, anti-SS-A, and anti-SS-B antibodies were also significantly higher in the P-group than in the N-group.     

干扰素调节因子1、3、7在原发性舍格伦综合征中的表达

目的：检测原发性舍格伦综合征（primary Sjgren＇s syndrome,pSS）患者血细胞中干扰素调节因子（IRF）1、3、7的表达水平,并检测IRF1在pSS患者腮腺组织中的表达。方法：采用实时定量PCR,对37例pSS患者和24例对照组患者血细胞中IRF1、3、7的水平进行检测,比较分析其在两组之间的差异。采用免疫组化和免疫荧光法检测IRF1在pSS患者腮腺组织中的表达。应用SAS6.12软件包对数据进行t检验。结果：实时定量PCR法检测结果发现,pSS组的IRF1mRNA表达量显著高于对照组,两组之间有显著差异（P=0.00001）,pSS组的IRF1是对照组的2.17倍,而IRF3和IRF7在两组之间无显著差异（P=0.3813,0.4501）。免疫组化和免疫荧光发现,pSS组患者腮腺组织中的导管上皮细胞、淋巴细胞和上皮岛均为IRF1阳性;而对照组腮腺组织中仅导管上皮细胞为IRF1阳性。结论：IRF1在原发性舍格伦综合征患者中存在异常表达。     

Parotitis as the initial sign of juvenile Sjögren's syndrome

Parotid swelling may be associated with a variety of glandular disorders in children. This case report describes the characteristic features of juvenile Sj?gren's syndrome in an adolescent girl who presented with recurrent and bilateral parotid gland enlargement. Special emphasis is placed on an age-specific differential diagnosis for major salivary gland enlargements.     

Radionuclide scan imaging of bilateral Warthin’s tumor and chronic inflammation in the submandibular glands of a patient with a history of heavy smoking

Papillary cystadenoma lymphomatosum, or Warthin’s tumor, is a benign tumor, primarily localized in the parotid glands. The tumor is more common in men than women, and it occurs usually in the fifth and sixth decades of life. Smoking seems to be one of the predisposing factors. The case of a 65-year-old man is described; he presented with a chief complaint of swelling in the bilateral parotid and submandibular glands. Imaging revealed a benign tumor in the right parotid gland, but only inflammation in both submandibular and the left parotid glands. A biopsy was carried out after rapidly increased swelling of the left parotid and left submandibular glands. Histopathological examinations revealed chronic sialadenitis. Superficial parotidectomies were performed 7 and 21 months after the initial visit on the right parotid and the left parotid glands, respectively. Histopathological examination revealed Warthin’s tumor in both parotid glands, with fibrogenesis and chronic sialadenitis in the submandibular glands.     

Oral sugar clearance and other caries-related factors of stimulated whole saliva in patients with secondary Sj?gren syndrome.

OBJECTIVE: To compare oral sugar clearance, salivary flow rate, buffer capacity, salivary counts of mutans streptococci and lactobacilli in whole saliva, and root caries in subjects with secondary Sj?gren syndrome with those of healthy subjects. METHOD AND MATERIALS: Twenty subjects with secondary Sj?gren syndrome and 20 healthy subjects (age 30 to 55 years; all women) rinsed their mouth with a 20% sucrose solution. Before the rinse and 2, 5, 10, and 30 minutes after the rinse, 2 paper disks were soaked with saliva, 1 under the tongue and 1 in the mandibular vestibule. The salivary sucrose concentration was determined enzymatically. The clearance time and the area under the sugar clearance curve (AUC) were calculated. RESULTS: Patients with secondary Sj?gren syndrome and with low salivary flow had longer sugar clearance times in the mandibular buccal vestibule and sublingual regions than did healthy individuals (P = .000 and P = .000, respectively). Significant differences of AUC values between the groups for the buccal and sublingual regions were also found (P = .000 and P = .003, respectively). CONCLUSION: Subjects with secondary Sj?gren syndrome had longer sugar clearance time, related to low salivary flow, high counts of cariogenic microorganisms, and decayed and filled surfaces in the root.     

Bilateral parotid voluminous masses: a case report

The case of a 75-year-old woman with voluminous bilateral symmetrical masses of the parotid glands is reported, and the literature relevant to the differential diagnosis in bilateral neoplastic and nonneoplastic parotid masses is reviewed. The presurgical workup was based on clinical evaluations, nuclear magnetic resonance imaging findings, and cytologic examination to obtain the differential diagnosis. Benign or malignant tumors were excluded. Surgery was performed for esthetic reasons and in consideration of progressive enlargement of lesions. Histopathologic examination of resected masses revealed benign lymphoepithelial cysts with polyclonal infiltration of lymphoid cells. Serological testing for tuberculosis, cytomegalovirus, human immunodeficiency virus, and Epstein-Barr virus (EBV) and testing using the in situ hybridization technique for the presence of cytomegalovirus and EBV were negative for productive infection or viral replication. A diagnosis of retention cysts was suspected on the basis of presurgical evaluation, histopathological examination, and serologic analysis. In this case, bilateral obstruction of the parotid ducts by a mobile denture probably played the main role in the pathogenesis and development of bilateral parotid retention cysts, because any other specific cause for the pathogenesis was not found with diagnostic tools.     

Immunopathology of Sj?gren-like disease in NZB/HZW mice.

In vitro responsiveness of spleen cells from NZP/NZW (B/W) female mice, aged 2 through 40 weeks, to the mitogens phytohemagglutinin (PHA), concanavalin-A (Con-A) and lipopolysaccharide (LPS) was determined. An age-related decline in proliferative response of splenic cells was found; this decline correlated with the age of onset and progression of lymphoid infiltration in lacrimal and salivary glands in the mice. The worsening with age of this lymphoid infiltrate, coupled with the decline in responsiveness to mitogens, suggests a complex immunopathologic process with destruction of glands terminating in a disease similar to the Sj?gren syndrome in humans.     

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目的    探讨生芦润燥汤治疗干燥综合征的临床疗效。方法    选取2007年5月至2008年12月上海中医药大学附属市中医医院风湿科及口腔科门诊收治的60例干燥综合征患者,随机分为试验组和对照组,每组30例。试验组服用生芦润燥汤治疗,对照组服用硫酸羟氯喹治疗,两组疗程均为12个月。治疗期间每3个月随访1次,观察并记录两组患者治疗前后的症状、体征及实验室检查结果。结果    试验组疗效与对照组疗效有显著差异（P < 0.01）;在改善症状、体征和中医征候方面,试验组明显优于对照组（P < 0.01）。结论    生芦润燥汤治疗干燥综合征有显著疗效。     

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.     

Kaposi's sarcoma of the parotid gland in acquired immunodeficiency syndrome

A patient with acquired immunodeficiency syndrome complained of a dry mouth and bilateral swelling of the parotid glands. Kaposi's sarcoma (KS) was found within the tissue of the parotid gland. Although KS is a common manifestation of acquired immunodeficiency syndrome, the presence of KS in the salivary glands is very rare. The human immunodeficiency virus I (HIV-I) genome was not identified in parotid glandular cells or in KS cells in this case, although the virus was detected in infiltrating lymphocytes and in tissue macrophages.     

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.