Abstract

Sjögren's syndrome (SS) or sicca syndrome was described by Swedish ophthalmologist Sjögren in the year 1933 for the first time. The etiology of the SS is multifunctional and includes a combination of genetic predisposition and environmental as well as epigenetic factors. It is an autoimmune disease characterized by features of systemic autoimmunity, dysfunction, and inflammation in the exocrine glands (mainly salivary and lacrimal glands) and lymphocytic infiltration of exocrine glands. In fact, the involvement of lacrimal and salivary glands results in the typical features of dry eye and salivary dysfunction (xerostomia). Only in one-third of the patients also present systemic extraglandular manifestations. T cells were originally considered to play the initiating role in the autoimmune process, while B cells were restricted to autoantibody production. In recent years, it is understood that the roles of B cells are multiple. Moreover, autoantibodies and blood B cell analysis are major contributors to a clinical diagnosis of Sjögren's syndrome. Recently, there has been rising interest in microRNA implication in autoimmunity. Unfortunately, to date, there are only a few studies that have investigated their participation in SS etiopathogenesis. The purpose of this work is to gather the data present in the literature to clarify this complex topic.

Highlights

  • IntroductionSjögren’s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands that results in eye and mouth dryness [1]

  • Other miRNAs, epigenetic factors, as well as viral elements that possibly harbor in the affected tissues, might participate in the pathogenesis of this disease

  • With the increasing number of studies, revealing miRNA deregulation in PBMCs, sera, and saliva in s syndrome (SS), the identification of serum miRNA to mirror activation state of lymphocytic subsets may become an innovative tool to provide pivotal information about the nature of the immune responses occurring in autoimmune disease

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Summary

Introduction

Sjögren’s syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands that results in eye and mouth dryness [1]. The SS prevalence is approximately 3% of the worldwide adults and has been reported to rarely affect children [2]. Epidemiological studies underline the marked predilection for female, with a ratio of 9 : 1 to male, with age between 20 and 50 years [3]. The disorder was described by Mikulicz in 1892, but only in 1933, Dr Henrik Sjögren published an article on a cluster of women presenting keratoconjunctivitis sicca, lymphoid infiltrations of the conjunctiva, cornea, lacrimal and parotid glands, a history of arthritis, and swelling of the salivary glands, in order to distinguish the SS from xerophthalmia [4]

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