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Objective: To estimate the point prevalence of primary Sjögren's syndrome (pSS) in two populations, aged 40-44 and 71-74 years, using two sets of classification criteria., Methods: The participating individuals were recruited from the Hordaland Health Study (HUSK) conducted during 1997-99. A total of 18 592 individuals born 1953-57 and 3346 individuals born 1925-27 were sent a questionnaire covering various health-related questions, including four questions about sicca symptoms. Among those answering positive to at least one of the four questions, 99 and 90 individuals born 1953-57 and 1925-27, respectively, were examined further. For diagnosis of pSS two classifications were used, the preliminary European criteria from 1993, and the revised European criteria from 1996., Results: By using the two classification criteria from 1993 and 1996, the point prevalences were 0.44% [95% confidence interval (CI) 0.34-0.57] and 0.22% (95% CI 0.15-0.32), respectively, for the population group born 1953-57. The corresponding estimates were 3.39% (95% CI 2.77-4.14) and 1.40% (95% CI 1.02-1.92) for the population born 1925-27., Conclusion: The point prevalence of pSS was approximately seven times higher in the elderly population aged 71-74 years compared to individuals aged 40-44 years, regardless of the classification criteria used.

Sjögren's syndrome (SS) is an autoimmune rheumatic disorder characterized by chronic lymphocytic infiltration and decreased secretion in the salivary glands (SGs). For some time, apoptosis has been suggested to constitute the major mechanism for acinar epithelial destruction during the effector phases; however, this is still controversial. We have recently demonstrated that despite the expression of Fas and FasL, the incidence of apoptosis is not increased in SS patients compared with control individuals. Our aim was therefore to further evaluate the expression of the pro- and anti-apoptotic Bax and Bcl-2 proteins. CD40 and CD154 expression was also investigated, as CD40 ligation has been suggested to protect cells from Fas-mediated apoptosis. Immunohistochemical staining was performed on SG tissue from primary and secondary SS patients, a group of patients with non-SS-related degenerative changes as well as on healthy control individuals. The frequency of stained cells in the foci of infiltrating mononuclear cells (IMCs) and in the acinar and ductal epithelium was determined. We found the expression of Bcl-2 but rarely Bax in SS SG IMCs. Bcl-2 in epithelial cells was sparse, while Bax expression occurred frequently and with no significant difference between the patient groups. CD40 and CD154 expression was high among SS IMCs, while CD40 levels were slightly decreased in SS epithelium compared with controls. Elevated CD154 expression was found in SS epithelium, being significantly increased in the ducts. In conclusion, our study further supports the hypothesis about apoptosis resistance among SS focal IMCs and suggests a putative protective role of CD40 ligation in SS SG epithelium.

The aim of this study was to perform a controlled in situ analysis on the incidence of apoptosis, investigate the expression of apoptosis-mediating proteins, and determine the frequency of apoptotic CD4+ and CD8+ T cells in Sjögren's syndrome (SS). The study was extended to patients with atrophy-fibrosis (AF) not related to SS, as well as to a control group. Immunohistochemistry and the terminal deoxynucleotidyl transferase mediated dUTP digoxigenin nick end labeling (TUNEL) method were applied to study the Fas and FasL expression and the incidence of apoptosis in salivary glands (SG) from patients with primary and secondary SS, AF, and controls. These methods were also combined to enable simultaneous detection of apoptotic and CD4+ or CD8+ T cells. Despite abundant expression of Fas and FasL in SS SG, apoptotic cells were not exceeding 1% in the foci of infiltrating mononuclear cells (IMC). Double staining showed that the frequency of apoptosis was low among both CD4+ and CD8+ T cells. Only a few TUNEL+ epithelial cells were found in all patient groups. Fas was expressed predominantly on SS IMC, single SS epithelial cells, and a few normal acinar cells, but not in AF SG. Although FasL was present on SS and AF IMC and epithelial cells, it was rarely detected in normal tissue. Consequently we demonstrate that Fas-induced apoptosis among SS SG is a rare event. Our findings support an earlier hypothesis indicating that IMC seem to be able to escape apoptosis, resulting in foci of inflammatory cells. Notably, however, no obvious correlation can be drawn to previous studies where a high incidence of apoptosis of epithelial cells was proposed as an important mechanism leading to decreased glandular function, which is a hallmark of SS.

The expression of the mucosal lymphocyte integrin alphaEbeta7 and its ligand E-cadherin was analyzed in sialadenitic lesions of patients with Sjögren's syndrome in order to assess the potential interactions between the mucosal and the systemic immunological compartments. Immunohistochemical evaluation of the inflammatory infiltrates in the salivary gland tissues in Sjögren's syndrome patients revealed presence of alphaEbeta7-integrin-expressing lymphocytes, in 18 of 20 biopsies examined. The ligand for alphaEbeta7, E-cadherin was also found in 18 of 20 salivary gland biopsies analysed. These results suggest homing of lymphocytes derived from the gut epithelium through the blood stream to inflamed salivary gland tissue followed by in situ interaction with E-cadherin expressing cells, the ligand for alphaEbeta7 expressing T lymphocytes.

European criteria for classification of Sjögren's syndrome have recently been developed and evaluated. We report the clinical and laboratory findings in 96 patients with primary Sjögren's syndrome who have been classified according to these new criteria. In our patient population the latency from appearance of the first symptom to diagnosis was 11 years. In addition to sicca symptoms in mucous membranes, the dominant symptoms were periodic fatigue (92%), arthralgia (82%), hoarse voice (71%), dry cough (54%) and diarrhoea (51%). Antibodies to the nuclear antigens SSA and SSB were found in respectively 22.2% and 15.6% of the patients. Two out of ten patients with both anti-SSA and anti-SSB antibodies gave birth to a child with heart block.

The aim of this study was to establish additional indicators in saliva and plasma which are associated with salivary gland inflammation in patients with primary Sjögren's syndrome (SS). ELISA assays were used to determine the concentrations of sICAM-1, sVCAM-1, sIL-2R alpha, IgA, IgG, calprotectin and albumin in parotid saliva, whole saliva and plasma samples. Soluble ICAM-1 was present in whole and parotid saliva samples from primary SS patients. Soluble VCAM-1 and sIL-2R alpha could not be detected in salivary samples from either primary SS or control subjects. IgA, IgG, calprotectin and albumin concentrations were higher in both whole and parotid saliva in the patient group compared with the control group. The results showed increased levels of calprotectin in all saliva samples compared to plasma, suggesting that calprotectin may be locally produced. Increased plasma values of sICAM-1, sVCAM-1, sIL-2R alpha, IgA, IgG and calprotectin were detected in primary SS patients when compared to controls. The output/min of IgA, IgG, calprotectin and albumin was decreased in SS patients. Plasma levels of various proteins could offer information concerning glandular and extraglandular inflammatory processes. However, salivary levels of these proteins (particularly sICAM-1) tend to reflect more the local inflammatory activity, providing a convenient and non-invasive tool for diagnosis.

The aim of this study was to analyse the nature of infiltrating cells in minor salivary glands of patients with Sjögren's syndrome (SS). Furthermore, we wanted to characterize the tissue distribution of calprotectin-producing cells in inflamed salivary gland tissue of SS and in synovial tissue of patients with rheumatoid arthritis (RA) and osteoarthritis (OA). Cryostat sections of labial salivary gland tissue from patients with SS and synovial tissue from RA and OA patients were stained (ABC-immunoperoxidase technique) using monoclonal antibodies (MoAbs) to T cells (CD3), monocytes/macrophages (CD14, CD68), and calprotectin. Monocytes and macrophages were widely distributed in focal infiltrates of salivary gland tissue from SS patients. Calprotectin markers showed a distinct staining of infiltrating macrophages and around blood vessel walls. In synovial tissue samples, calprotectin was expressed in a high percentage of cells in the synovial lining, the subsynovium, and vessel walls. The percentages of cells stained for calprotectin were significantly higher in RA than in OA and SS tissues. Antibodies to the calprotectin complex stained cells with a similar distribution as antibodies against the separate polypeptide chains of calprotectin. The localization and differentiated expression of calprotectin in these chronic inflammatory conditions indicate a role in the inflammatory process and may be an additional marker of macrophages/granulocytes in SS, RA and OA.

The aim of this study was to obtain further information about exocrine glandular immunopathology and the potential of the MRL/lpr strain as a model of Sjögren's syndrome. Immunoenzyme staining (ABC technique) and monoclonal antibodies defining CD3 T-cell receptor (TcR) alpha beta, gamma delta and TcR V beta 2, V beta 4, V beta 6, V beta 7, V beta 8.1,2, V beta 10b and V beta 11 were used to identify the mononuclear cells (MNC) in salivary gland infiltrates and lymph nodes of 2- and 4-5-month-old female MRL/lpr mice. TcR alpha beta + cells dominated clearly over TcR gamma delta + cells in both salivary glands and lymph nodes. In addition, to be expressed on lymphocyte-like cells, TcR gamma delta + cells also had a dendritic appearance. The frequency pattern of TcR expression in early inflammation (2 months) was V beta 8.1, 2 > V beta 6 > V beta 4 > V beta 10b > V beta 2 > V beta 7 > V beta 11. Clear differences in frequencies could be found between salivary glands and lymph nodes in established sialadenitis (4-5 months). Particularly V beta 4, V beta 8.1,2 and V beta 10b showed expansion in salivary glands at > or = 4 months. In conclusion, this study shows a diverse repertoire of TcR at local sites of MNC infiltration in autoimmune MRL/lpr mice. However, with increasing age it also shows a preferential utilization of certain V beta gene products.

The expression of the mucosal lymphocyte integrin α[sup E]β[sub 7] and its ligand E-cadherin was analyzed in sialadenitic lesions of patients with Sjögren's syndrome in order to assess the potential interactions between the mucosal and the systemic immunological compartments. Immunohistochemical evaluation of the inflammatory infiltrates in the salivary gland tissues in Sjögren's syndrome patients revealed presence of α[sup E]β[sub 7]-integrin-expressing lymphocytes, in 18 of 20 biopsies examined. The ligand for α[sup E]β[sub 7], E-cadherin was also found in 18 of 20 salivary gland biopsies analysed. These results suggest homing of lymphocytes derived from the gut epithelium through the blood stream to inflamed salivary gland tissue followed by in situ interaction with E-cadherin expressing cells, the ligand for α[sup E]β[sub 7] expressing T lymphocytes. [ABSTRACT FROM AUTHOR]