Elderly onset of primary Sjögren's syndrome: clinical manifestations, serological features and oral/ocular diagnostic tests. Comparison with adult and young onset of the disease in a cohort of 336 Italian patients.

OBJECTIVES: To study and compare the clinical and serological features of patients with elderly versus adult and younger onset of primary Sjögren's syndrome (pSS). METHODS: We analyzed retrospectively 336 consecutive pSS patients followed at our unit. They were subdivided into three groups according to the age at disease onset: elderly (>65 years), adult (>40 and ≤65 years), and young (≤40 years). Clinical and immunological features of the disease, labial salivary glands biopsy, ocular and oral tests were collected at time of diagnosis and then compared among the three groups. RESULTS: In 21 (6%) patients, disease onset occurred after the age of 65 years. At the time of diagnosis, 15 (71.4%) of these patients reported symptoms of dry mouth and 16 (76.1%) of dry eye. The most common extraglandular manifestation were arthralgias in 14 (66.7%), Raynaud's phenomenon in five (23.8%) and purpura in three (14.2%) cases. Ocular diagnostic tests (Schirmer's I and Rose-Bengal staining) were positive respectively in 17 (80%) and nine (44.4%) patients. In eight (38%) cases, unstimulated whole salivary flow showed normal values, while 12 patients (57.1%) showed positivity for salivary sialography. A focus score greater or equal to 1 per 4mm(2) was demonstrated in 11 (53.3%) of the 21 cases. CONCLUSION: Elderly onset of pSS was associated with similar incidence of the diagnostic tests positivity (parotid sialography, ocular tests, minor salivary gland biopsy) in comparison with adult and younger onset. Moreover, no statistical differences were found among the three groups concerning sex, disease duration, as well as ocular and oral symptoms.

Variability of adenoidectomy/tonsillectomy rates among children of the Veneto Region, Italy.

BACKGROUND: Despite national guidelines in 2003 aimed at limiting the recourse to tonsillectomy and/or adenoidectomy (A/T), the latter are among the most frequent pediatric surgeries performed in Italy. Aim of the study is to investigate variability of A/T rates among children of the Veneto Region, Italy. METHODS: All discharges of Veneto residents with Diagnosis-Related Groups 57-60 and ICD9-CM intervention codes 28.2 (tonsillectomy), 28.3 (adenotonsillectomy), 28.6 (adenoidectomy) were selected in the period 2000-2006 for a descriptive analysis. A multilevel Poisson regression model was applied to estimate Incidence Rate Ratios (IRR) with 95% Confidence Intervals (CI) for A/T surgery among children aged 2-9 years in 2004-2006, while taking into account clustering of interventions within the 21 Local Health Units. RESULTS: Through 2000-2006, the overall number of A/T surgeries decreased (-8%); there was a decline of adenoidectomies (-20%) and tonsillectomies (-8%), whereas adenotonsillectomies raised (+18%). Analyses on children aged 2-9 resulted in an overall rate of 14.4 surgeries per 1000 person-years (16.1 among males and 12.5 among females), with a wide heterogeneity across Local Health Units (range 8.1-27.6). At random intercept Poisson regression, while adjusting for sex and age, intervention rates were markedly lower among foreign than among Italian children (IRR = 0.57, CI 0.53-0.61). A/T rates in the 10-40 age group (mainly tonsillectomies) computed for each Local Health Unit and introduced in the regression model accounted for 40% of the variance at Local Health Unit level of pediatric rates (mainly adenoidectomies and adenotonsillectomies). CONCLUSION: A/T rates in the Veneto Region, especially adenoidectomies among children aged 2-9 years, remain high notwithstanding a decrease through 2000-2006. A wide heterogeneity according to nationality and Local Health Units is evident. The propensity to A/T surgery of each Local Health Unit is similar in different age groups and for different surgical indications.

Donor site outcome after oral mucosa harvest for urethroplasty in children and adults.

PURPOSE: We report short and long-term donor site outcomes after oral mucosa graft harvesting for urological reconstruction in a large series of patients including children, and identify possible risk factors for an untoward long-term outcome. MATERIALS AND METHODS: A total of 78 patients were evaluated. Short-term outcomes included time to restore normal oral diet, perioral sensory defect/discomfort and jaw opening impairment occurring within 4 weeks of surgery. Long-term outcomes included donor site scarring, perioral sensory defect and jaw opening impairment occurring more than 1 year postoperatively. Long-term outcomes were assessed via a questionnaire administered to patients and on clinical examination by an oral surgeon. Outcomes were compared in children (younger than 12 years at surgery) and adults, and with regard to harvesting site, graft length, length of followup and other variables. RESULTS: Two-thirds of the patients returned to a normal oral diet within 3 days postoperatively (range 1 to 8). All patients complained of perioral sensory defect/discomfort postoperatively, and 26% had jaw opening impairment. After a median followup of 7.6 years (range 1 to 13.2) perioral sensory defect was the most common complication observed (28%) in cases formally evaluated by an oral surgeon. The sensory defect was seldom perceived by the patients and never required treatment. It was statistically more common in patients undergoing surgery as adults, whereas none of the other variables proved significant. CONCLUSIONS: Oral mucosa graft harvesting is safe irrespective of age. About a quarter of patients, more commonly adults, will have a long-term perioral sensory defect. However, the defect is never perceived as bothersome.

Epithelial CXCR3-B regulates chemokines bioavailability in normal, but not in Sjogren's syndrome, salivary glands.

Expression of CXCR3-targeting chemokines have been demonstrated in several diseases, suggesting a critical role for CXCR3 in recruiting activated T cells to sites of immune-mediated inflammation. Sjögren's syndrome (SS) is an autoimmune disease characterized by a mononuclear cell infiltrate of activated T cells around the duct in the salivary gland. Analysis of minor salivary gland biopsy specimens from 20 healthy subjects and 18 patients with primary SS demonstrated that CXCR3, in particular, the B form of this receptor, is constitutively expressed by human salivary gland epithelial cells. Salivary gland epithelial cell cultures demonstrated that CXCR3 participate in removing relevant amount of agonists from the supernatant of exposed cells without mediating calcium flux or chemotaxis while retaining the ability to undergo internalization. Although in normal salivary gland epithelial cells, CXCR3 behaves as a chemokine-scavenging receptor, its role in SS cells is functionally impaired. The impairment of this scavenging function might favor chemotaxis, leading to heightened immigration of CXCR3-positive T lymphocytes. These findings suggest that epithelial CXCR3 may be involved in postsecretion regulation of chemokine bioavailability. They also support a critical role for CXCR3 in the pathogenesis of SS and identify its agonists as potential therapeutic targets.

Serum and salivary neopterin and interferon-gamma in primary Sjögren's syndrome. Correlation with clinical, laboratory and histopathologic features.

OBJECTIVE: To investigate serum and salivary neopterin and interferon-gamma as possible markers of immune system activation in primary Sjögren's Syndrome (pSS). METHODS: Serum and salivary neopterin and interferon-gamma concentrations were determined in 30 untreated patients with pSS and matched with several other clinical and laboratory parameters. RESULTS: The mean concentration of neopterin was significantly higher in pSS patients (8.12+/- 3.36 nmol/L in serum and 9.50 +/-7.61 nmol/L in saliva) than in normal controls (p<0.05). Significant correlations were found between serum neopterin and beta2-microglobulin, serum IgG as well as lip biopsy score. Salivary neopterin concentration was inversely related to Shirmer-I test, tear break-up time and stimulated salivary flow rate. Serum and salivary levels of interferon-gamma were normal and no correlation with the other parameters was found. CONCLUSION: In pSS patients serum neopterin may represent a useful marker of cell-mediated immunity. On the other hand, salivary neopterin seems to reflect theglandular damage.