Abstract
Sjögren’s syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren’s syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren’s syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren’s syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren’s syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren’s syndrome, starting from its pathogenesis to current therapeutic options.
Highlights
Sjögren’s Syndrome (SS) is a systemic chronic autoimmune disorder of unknown etiology characterized by salivary and lacrimal glands immune-mediated damage, leading to dryness of the mouth and eyes
SS is defined as "primary" if it is not associated with other diseases or "secondary" if it occurs in association with an underlying autoimmune connective tissue disorder, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or systemic sclerosis (SSc)
SS owes its eponym to the Swedish ophthalmologist Henrik Samuel Conrad Sjögren (1899–1986) that firstly correlated the triad of keratoconjunctivitis sicca, xerostomia and polyarthritis in 1933 [2]
Summary
Sjögren’s Syndrome (SS) is a systemic chronic autoimmune disorder of unknown etiology characterized by salivary and lacrimal glands immune-mediated damage, leading to dryness of the mouth (xerostomia) and eyes (xerophthalmia). Dryness may affect other mucosal surfaces such as airways, digestive tract and vagina, resulting in the clinical picture of "sicca syndrome" or "sicca complex." As a. SS is defined as "primary" if it is not associated with other diseases or "secondary" if it occurs in association with an underlying autoimmune connective tissue disorder, such as RA, SLE or systemic sclerosis (SSc). SS owes its eponym to the Swedish ophthalmologist Henrik Samuel Conrad Sjögren (1899–1986) that firstly correlated the triad of keratoconjunctivitis sicca, xerostomia and polyarthritis in 1933 [2]
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