Objective. To develop valid instruments for the assessment of disease-related damage and disease activity in Sjögren's syndrome (SS).Methods. Data on 206 patients with primary SS were collected in 12 Italian centers. Each patient was scored by 1 investigator, on the basis of a global assessment of the degree of disease damage and disease activity. Patients judged to have active disease at the time of enrollment underwent a second evaluation after 3 months. Univariate and multivariate analyses were performed to select the clinical and serologic variables that were the best predictors of damage and of disease activity, and these variables were used to construct the Sjögren's Syndrome Disease Damage Index (SSDDI) and the Sjögren's Syndrome Disease Activity Index (SSDAI). The weight of each variable in the index was determined by the  coefficients in multivariate regression models. Scores obtained using the SSDDI and the SSDAI were compared with scores initially given by the investigators. Finally, a receiver operating characteristic (ROC) curve was used to determine the cutoff value in the SSDAI with the highest level of accuracy in identifying patients with a significant level of disease activity.Results. A multivariate model with 9 variables was the best predictor of investigator scores of damage. The scores obtained using the SSDDI were closely correlated with investigator ratings (R ؍ 0.760, P < 0.0001). A model composed of 11 variables was the best predictor of investigator scores of disease activity. The scores obtained using the SSDAI were strongly correlated with the investigator ratings both at the time of enrollment and 3 months after enrollment (R ؍ 0.872, P < 0.0001, and R ؍ 0.817, P < 0.0001, respectively). The differences between scores given by investigators at study enrollment and after 3 months, a measure of variation of disease activity over time, were also closely correlated with the differences calculated using the SSDAI (R ؍ 0.683, P < 0.0001). The ROC curve analysis showed that patients with the highest level of
Ultrasound detects effusion and synovial proliferation caused by synovitis. The study was undertaken to evaluate the signs of synovitis in patients with primary Sjögren's Syndrome (SS). Joint effusion was detected and synovial thickness was measured in the suprapatellar synovial bursa. Results have been compared with those obtained by sonographic assessment of knee joint in patients with secondary SS and RA. with secondary SS and connective tissue diseases, with RA, and in healthy subjects. Synovial thickening was demonstrated in all the diseases (higher grades of thickening were found in secondary SS with RA and in RA). Joint effusion was present with significantly higher frequency in secondary SS with RA and in RA. Results demonstrated signs of slight synovitis in primary SS. More severe synovitis was found both in secondary SS with RA and in RA. This is the first sonographic study demonstrating slight synovitis in primary SS.
Sjögren's syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands, mainly the salivary and lachrymal glands, with consequent persistent dryness of the mouth and eyes. In addition to the clinical manifestations related to the exocrine gland involvement, a consistent prevalence of patients may present systemic manifestations. Some of these can be ascribed to the periepithelial extension of lymphocytic infiltration whilst others are determined by an immunomediated process affecting small-or medium-size vessels. While the use of tear and saliva substitutes and local or systemic stimulation of residual secretions represent the mainstays of the therapy of sicca component, different immunomodulating or immunosuppressive agents are usually required to treat extraglandular features, similarly to what happens in other connective tissue diseases. In the last few years, the advancement in the understanding the pathogenetic mechanisms of this disorder and the availability of new biologic target therapies seem to offer completely new therapeutic options. The use of B cell depleting or modulating therapies has achieved promising results.
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