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Sjögren syndrome

Abstract

Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be observed in children, men and the elderly. The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma. The mechanism underlying the development of SjS is the destruction of the epithelium of the exocrine glands, as a consequence of abnormal B cell and T cell responses to the autoantigens Ro/SSA and La/SSB, among others. Diagnostic criteria for SjS include the detection of autoantibodies in patient serum and histological analysis of biopsied salivary gland tissue. Therapeutic approaches for SjS include both topical and systemic treatments to manage the sicca and systemic symptoms of disease. SjS is a serious disease with excess mortality, mainly related to the systemic involvement of disease and the development of lymphomas in some patients. Knowledge of SjS has progressed substantially, but this disease is still characterized by sicca symptoms, the systemic involvement of disease, lymphocytic infiltration to exocrine glands, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies and the increased risk of lymphoma in patients with SjS.

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Figure 1: Milestones in the history of SjS compressed in 100 years (1882–1982).
Figure 2: The pathogenesis of autoimmune epitelitis as a potential explanation for SjS.
Figure 3: Systemic involvement in patients with SjS.
Figure 4: Histopathological features of the salivary gland in SjS scenarios.

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Acknowledgements

The authors thank L. Alós, Pathology Department, Hospital Clínic, IDIBAPS, Barcelona, Spain, for her assistance in preparing the histopathological images.

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Introduction (P.B.-Z.); Epidemiology (M.R.-C.); Mechanisms/pathophysiology (A.T., K.S. and R.J.); Diagnosis, screening and prevention (C.B. and P.B.-Z.); Management (E.T., H.B. and X.M.); Quality of life (S.J.B.); Outlook (M.R.-C.); Overview of Primer (M.R.-C.).

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Correspondence to Manuel Ramos-Casals.

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The authors declare no competing interests.

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Brito-Zerón, P., Baldini, C., Bootsma, H. et al. Sjögren syndrome. Nat Rev Dis Primers 2, 16047 (2016). https://doi.org/10.1038/nrdp.2016.47

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