Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease with gradually progressive lacrimal and salivary dysfunction, which can be symptomatic or asymptomatic and include a variety of extraglandular conditions. Secondary Sjögren’s syndrome (sSS) occurs when lacrimal and salivary dysfunction develop in patients with another autoimmune connective tissue disease (ACTD), most commonly rheumatoid arthritis. Because of the persistence and progression of secretory dysfunction, patients with either form of SS often experience significant misery. The community prevalence of pSS, using current diagnostic criteria, ranges from 0.1% to 0.6%, depending on the study design. Affected organs in patients with SS exhibit a particular pattern of chronic inflammation that is gradually progressive and uncommonly undergoes transformation to lymphoma. Patients with SS produce a variety of circulating autoantibodies, but no combination of these has yet been established as a satisfactory classification criterion for pSS, which requires multisystem diagnostic tests. Treatment of SS requires effective management of both ocular and oral secretory dysfunction, the prevention or treatment of disease sequelae, and therapy for any extraglandular conditions that occur. There is currently no single treatment that addresses all of the diverse manifestations of this disease.
KeywordsPrimary Biliary Cirrhosis Minor Salivary Gland Congenital Heart Block Keratoconjunctivitis Sicca Saliva Substitute
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