Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy

Abstract

Primary Sjögren’s syndrome (pSS) is a chronic systemic autoimmune rheumatic disease characterized by lymphoplasmacytic infiltration of the salivary and lacrimal glands, whereby sicca syndrome and/or systemic manifestations are the clinical hallmarks, associated with a particular autoantibody profile. pSS is the most frequent connective tissue disease after rheumatoid arthritis, affecting 0.3–3% of the population. Women are more prone to develop pSS than men, with a sex ratio of 9:1. Considered in the past as innocent collateral passive victims of autoimmunity, the epithelial cells of the salivary glands are now known to play an active role in the pathogenesis of the disease. The aetiology of the “autoimmune epithelitis” still remains unknown, but certainly involves genetic, environmental and hormonal factors. Later during the disease evolution, the subsequent chronic activation of B cells can lead to the development of systemic manifestations or non-Hodgkin’s lymphoma. The aim of the present comprehensive review is to provide the current state of knowledge on pSS. The review addresses the clinical manifestations and complications of the disease, the diagnostic workup, the pathogenic mechanisms and the therapeutic approaches.

SMADS-Mediate Molecular Mechanisms in Sjögren’s Syndrome

“…Salivary gland (SG) fibrosis is a recently associated feature of Sjögren’s syndrome (SS), a chronic inflammatory autoimmune disease and multisystem exocrinopathy characterised by dry mouth and dry eyes with wide-ranging extra-glandular involvement [ 1 ]. As can be deduced from daily clinical practice, an acute inflammation can be recovered quickly, but chronic inflammation characterizing SS, will progress to irreversible fibrosis with SGs damage and impaired regenerative capacity.…”

The Involvement of Innate and Adaptive Immunity in the Initiation and Perpetuation of Sjögren’s Syndrome

“…The pathophysiology of SS is very complex, multifactorial, and consecutive to several genetic, hormonal, environmental, and immunological risk factors. Due to its complexity, the clinical course of the pathology can be divided in several phases: An initiation phase consecutive to endogenous and exogenous factors, a dysregulation of salivary glands epithelial cells (SGECs), and an immune system activation and chronicity of inflammation induced by B cells hyperactivity [ 7 ]. The combination of all these events culminates in the destruction of the salivary gland architecture, and development of keratoconjunctivitis sicca and xerostomia.…”

SMADS-mediate Molecular Mechanisms in Sjӧgren's Syndrome

“…Salivary gland (SG) fibrosis is a recently associated feature of Sjӧgren's syndrome (SS), a chronic inflammatory autoimmune disease and multisystem exocrinopathy characterised by dry mouth and dry eyes with wide-ranging extra-glandular involvement [1]. As can be deduced from daily clinical practice, an acute inflammation can be recovered quickly, but chronic inflammation characterizing SS, will progress to irreversible fibrosis with SGs damage and impaired regenerative capacity.…”