Abstract
Sjögren’s syndrome is a chronic autoimmune disorder of unknown origin. The disease affects mainly the exocrine glands, particularly the salivary and lacrimal glands, resulting in dry mouth and eyes. In more than a half of patients, it extends beyond the exocrine glands affecting parenchymal organs and small-size arteries producing extraglandular manifestation. The disease is common, affecting around 0.5 % of the general population, ranking in second incidence among the systemic autoimmune disorders, after rheumatoid arthritis. The last years’ several clinical and molecular information demonstrated the central role of the epithelial cell in the pathogenesis and clinical expression of the disease and coined the etiologic name “autoimmune epithelitis” for the syndrome.
In the present chapter, the clinical picture with emphasis to skin involvement, recent pathogenetic aspects, and treatment modalities are discussed.
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Dafni UG, Tzioufas AG, Staikos P, Skopouli FN, Moutsopoulos HM. Prevalence of Sjögren’s syndrome in a closed rural community. Ann Rheum Dis. 1997;56:521–5.
Hall JC, Casciola-Rosen L, Berger AE, et al. Precise probes of type II interferon activity define the origin of interferon signatures in target tissues in rheumatic diseases. Proc Natl Acad Sci U S A. 2012;109:17609–14.
Mavragani CP, Moutsopoulos HM. The geoepidemiology of Sjögren’s syndrome. Autoimmun Rev. 2010;9:A305–10.
Moutsopoulos HM. Sjögren’s syndrome: autoimmune epithelitis. Clin Immunol Immunopathol. 1994;72:162–5.
Pavlakis PP, Alexopoulos H, Kosmidis ML, Stamboulis E, Routsias JG, Tzartos SJ, Tzioufas AG, Moutsopoulos HM, Dalakas MC. Peripheral neuropathies in Sjogren syndrome: a new reappraisal. J Neurol Neurosurg Psychiatry. 2011;82:798–802.
Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X, Tzioufas AG. Topical and systemic medications for the treatment of primary Sjögren’s syndrome. Nat Rev Rheumatol. 2012;8:399–411.
Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 2000;29(5):296–304.
Triantafyllopoulou A, Moutsopoulos H. Persistent viral infection in primary Sjogren’s syndrome: review and perspectives. Clin Rev Allergy Immunol. 2007;32:210–4.
Tzioufas AG, Moutsopoulos HM. Ultrasonography of salivary glands: an evolving approach for the diagnosis of Sjögren’s syndrome. Nat Clin Pract Rheumatol. 2008;4:454–5.
Tzioufas AG, Kapsogeorgou EK, Moutsopoulos HM. Pathogenesis of Sjögren’s syndrome: what we know and what we should learn. J Autoimmun. 2012;39(1–2):4–8.
Voulgarelis M, Giannouli S, Tzioufas AG, Moutsopoulos HM. Long term remission of Sjögren’s syndrome associated aggressive B cell non-Hodgkin’s lymphomas following combined B cell depletion therapy and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). Ann Rheum Dis. 2006;65:1033–7.
Voulgarelis M, Tzioufas AG, Moutsopoulos HM. Mortality in Sjögren’s syndrome. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S66–71.
Voulgarelis M, Ziakas PD, Papageorgiou A, Baimpa E, Tzioufas AG, Moutsopoulos HM. Prognosis and outcome of non-Hodgkin lymphoma in primary Sjögren syndrome. Medicine (Baltimore). 2012;91:1–9.
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Tzioufas, A.G., Moutsopoulos, H.M. (2015). Sjögren’s Syndrome. In: Katsambas, A.D., Lotti, T.M., Dessinioti, C., D’Erme, A.M. (eds) European Handbook of Dermatological Treatments. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45139-7_89
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DOI: https://doi.org/10.1007/978-3-662-45139-7_89
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