Primary Sjögren’s syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. pSS is the most frequent connective tissue disease after rheumatoid arthritis, affecting 0.3–3% of the population. Women are more prone to develop pSS than men, with a sex ratio of 9:1. Considered in the past as innocent collateral passive victims of autoimmunity, the epithelial cells of the salivary glands are now known to play an active role in the pathogenesis of the disease. The aetiology of the “autoimmune epithelitis” still remains unknown, but certainly involves genetic, environmental and hormonal factors. Later during the disease evolution, the subsequent chronic activation of B cells can lead to the development of systemic manifestations or non-Hodgkin’s lymphoma. The aim of the present comprehensive review is to provide the current state of knowledge on pSS. The review addresses the clinical manifestations and complications of the disease, the diagnostic workup, the pathogenic mechanisms and the therapeutic approaches.
Serum levels of S100A8/A9 are significantly raised in SLE versus pSS patients and healthy controls and can be correlated to a disease activity index. S100A8/A9 is a more relevant marker of infection in SLE patients.
Objective. To investigate the expression and localization of aquaporin 5 (AQP5) in salivary glands and salivary gland function in the NOD mouse.Methods. All experiments were performed using NOD and BALB/c mice (ages 8 weeks and 24 weeks). Real-time reverse transcription-polymerase chain reaction, Western blotting, and immunohistochemical analysis were used to study the expression and distribution of AQP5 in salivary glands. In addition, salivary gland function was determined.Results. Compared with the levels in BALB/c mice, relative AQP5 messenger RNA levels were not significantly modified in the parotid glands from NOD mice of both ages but were significantly increased in the submandibular glands from NOD mice of both ages. Western blot analyses of both salivary gland membranes revealed that the level of AQP5 protein was increased in 24-week-old NOD mice. Important inflammatory infiltrates were observed in the submandibular glands, but not in the parotid glands, from 24-week-old NOD mice. The 8-week-old and 24-week-old BALB/c mice and the 8-week-old NOD mice showed AQP5 primarily at the apical membrane of the salivary gland acinus. In contrast, in acini from the submandibular glands (but not the parotid glands) from 24-week-old NOD mice, AQP5 staining was reduced at the apical membrane but was increased at the basal membrane. A moderately statistically significant decrease in pilocarpine-stimulated salivary flow was observed in 24-week-old NOD mice compared with that in agematched BALB/c mice.Conclusion. Submandibular glands from 24-week-old NOD mice displayed inflammatory infiltrates, increased AQP5 protein expression, and impaired AQP5 distribution. However, the moderately statistically significant decrease in the salivary flow rate in these mice did not match the extent of AQP5 misdistribution.Sjögren's syndrome (SS) is an autoimmune disorder characterized by lymphocyte infiltration of salivary and lacrimal glands, leading to glandular hypofunction that often results in characteristic symptoms of dry mouth and dry eyes (1). Typical salivary gland biopsy specimens obtained from patients with SS show lymphocytic infiltrates, with an associated reduction (up to 50%) in the number of acinar cells (2). The roles of reduced numbers of acinar cells and associated glandular dysfunction in the pathogenesis of SS are well characterized (1). Several factors have been proposed to contribute to impaired salivary flow, including the proinflammatory cytokines interleukin-1 and tumor necrosis factor ␣ (which were shown to inhibit both basal and stimulated secretion [1]), decreased protein kinase C levels in salivary gland acinar cells from patients with SS (3), as well as the presence of antibodies against type 3 muscarinic receptor in patients with primary SS (4).
Sjö gren's syndrome (SS) is an autoimmune disorder characterized by ocular and oral dryness as well as systemic manifestations. The immunopathogenesis of SS is complex with different intricate factors. Because of the delay in the appearance of symptoms and due to ethical issues it is very difficult to study the wide array of factors intervening in the pathogenesis of SS in human patients. To circumvent this problem, different animal models have been elaborated for studying the different subsets of the aspects of the physiopathology of this disease. In this review, we focus on the mouse models that have been established to deepen our insight into the immunopathogenesis of SS. Oral Diseases (2007) 13, [366][367][368][369][370][371][372][373][374][375]
IL-33 is released in pSS, and acts with IL-12 and IL-23 to favour the secretion of IFNγ by NK and NKT cells.
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