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Sjögren’s syndrome is an autoimmune condition characterised by lymphocytic infiltration of the salivary and lacrimal glands, causing saliva and tears to dry up . In addition to the symptoms of dryness (which may also affect the skin, vagina, and respiratory system) and fatigue there may be extra-glandular signs and autoantibodies present in the serum (anti-SSA and/or anti-SSB).
Sjögren’s syndrome can be primary (pSS) or secondary (sSS) to another systemic disease (rheumatoid arthritis, systemic lupus erythematosus, or scleroderma). Furthermore, it can be associated with other organ-specific autoimmune disorders, principally autoimmune thyroiditis and primary biliary cirrhosis. The main risk in terms of progression is lymphoma, which affected 5 - 10% of patients followed for more than 15 years .
This means that Sjögren’s syndrome is at the crossroads between autoimmune disease and lymphoproliferative disorders, and it is therefore an excellent model for understanding the link between autoimmunity and lymphoid proliferation.
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