Abstract
Sjögren’s syndrome (SS) is a systemic autoimmune disorder characterized by lymphocytic infiltration of the exocrine glands, especially the salivary and lacrimal glands, resulting in dry mouth and eyes. However, in most patients, the syndrome extends beyond the exocrine glands affecting parenchymal organs and small-sized arteries producing various extraglandular manifestations. The disease is common, with a prevalence ranging between 0.1% and 0.5% of the general population. Clinical, histopathological, and molecular data highlighted the central role of tissue epithelia in the pathogenesis of SS, justifying the etiologic term “autoimmune epithelitis” for the syndrome. The B cell component plays an important role in the pathogenic process by mediating systemic vasculitic manifestations and may evolve into non-Hodgkin’s lymphomas. In the current chapter, the recent pathogenetic aspects, the clinical picture with emphasis on skin involvement and the treatment modalities are being discussed.
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Goules, A.V., Chatzis, L., Tzioufas, A.G. (2023). Sjögren’s Syndrome. In: Katsambas, A.D., Lotti, T.M., Dessinioti, C., D'Erme, A.M. (eds) European Handbook of Dermatological Treatments. Springer, Cham. https://doi.org/10.1007/978-3-031-15130-9_88
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DOI: https://doi.org/10.1007/978-3-031-15130-9_88
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