Sjögren’s Syndrome: Beyond Sicca Involvement

作者: Manuel Ramos-Casals , Pilar Brito-Zerón , Albert Bové Boada , Antoni Sisó-Almirall

DOI: 10.1007/978-0-85729-358-9_5

关键词:

摘要: Sjogren syndrome (SS) is a systemic autoimmune disease that presents with sicca symptomatology of the major mucosal surfaces. The clinical spectrum this condition often extends to involvement (extraglandular manifestations) and may be complicated by development lymphoma. SS one most prevalent diseases (with an estimated 0.5–3 million sufferers in United States), primarily affects perimenopausal women. When symptoms appear previously healthy ­person, classified as primary SS. This chapter summarizes recent work focused on extending characterizing acute and/or complex presentations patients SS, including wide variety internal organs. As general rule, management extraglandular features should targeted specific organ involved. mainstays such treatment regimens remain glucocorticoids immunosuppressive agents. Such therapies are more likely affect favorably manifestations even though patients’ intense complaints pertain features. Severe, life-threatening has rarely been reported There now substantially data outcome which indicate predominantly extraepithelial expression (often associated cryoglobulinemia) monitored managed differently from periepithelial or sicca-limited disease.

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