Heterogeneity of Sjögren's Syndrome: The state of the art for clinical Practice
Keywords:
Sjögren's syndrome, autoimmunity, molecular mimicry, sicca syndrome, BiomarkersAbstract
Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by xerostomia and xerophthalmia due to salivary gland dysfunction. In addition to glandular involvement, any organ system can be compromised by the pathogenesis of this disease. For this reason, SS is characterized by pleomorphic clinical manifestations, the characteristics and severity of which can vary greatly from one patient to another. SS is defined as primary, when it occurs alone, or secondary, when it manifests in association with other systemic autoimmune diseases. Regarding its pathogenesis, different experimental models have been proposed, involving genetic and environmental factors to explain its development. Whatever they are, it seems to be crucial for the development of the disease the emergence of aberrant self-reactive B lymphocytes, leading to the production of autoantibodies and the formation of immune complexes. The diagnosis of Sjögren's syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests that include histopathology of salivary glands and autoantibodies. New classification criteria and disease activity scores have been developed primarily for research purposes. However, such criteria are also useful in daily clinical practice. The care of Sjögren's syndrome should be coordinated by the rheumatologist. It ranges from local and symptomatic therapies aimed at controlling dryness to systemic medications, including disease-modifying agents and biological agents. The aim of this review article is to summarize the recent literature on Sjögren's syndrome, from its pathogenesis to current therapeutic options.References
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