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Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that primarily involves the exocrine glands, resulting in their functional impairment. The syndrome can present either alone (primary Sjögren’s syndrome (pSS)) or in the context of an underlying connective tissue disease, most commonly rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) (secondary Sjögren’s syndrome (sSS)).
SS is the second most common autoimmune rheumatic disease. It mainly affects middle-aged women, with a female to male ratio reaching 9:1.
Sjögren’s syndrome (SS) is a systemic progressive autoimmune disease characterized by a complex pathogenesis requiring a predisposing genetic background and involving immune cell activation and autoantibody production. The immune response is directed to the exocrine glands, causing the typical ‘sicca syndrome’, but major organ involvement is also often seen.
The etiology of the disease is unknown. Infections could play a pivotal role: compared to normal subjects, patients with SS displayed higher titers of anti-Epstein-Barr virus (EBV) early antigens, but lower titers of other infectious agent antibodies such as rubella and cytomegalovirus (CMV) suggest that some infections may have a protective role against the development of autoimmune disease. Recent findings seem to show that low vitamin D levels in patients with SS could be associated with severe complications such as lymphoma and peripheral neuropathy. This could open new insights into the disease etiology.
The current treatments for SS range from symptomatic therapies to systemic immunosuppressive drugs, especially B cell-targeted drugs in cases of organ involvement. Vitamin D supplementation may be an additional tool for optimization of SS treatment.
2013/04/08