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.
Highlights
Sjögren’s syndrome (SS) is a chronic systemic rheumatic disease characterized by lymphoplasmacytic infiltration of the exocrine glands—especially salivary and lachrymal glands—responsible for sicca syndrome and systemic manifestations
Three recurrent events are generally associated with SS: (1) a trigger phase induced by environmental factors under specific epigenetic factors, genetic predisposition and hormonal regulation; (2) the dysregulation of normal salivary gland epithelial cell (SGEC) function; (3) a chronic inflammation characterized by salivary glands (SG) infiltration made of lymphocytic cells, lymphocytes B hyperactivity and autoantibodies production [10] (Figure 1)
IL15 and WDR5 play an important role in B cell proliferation and differentiation; GNAI2 regulates B cell trafficking to the lymph nodes [61]; LTßR and CBX8 are involved in Germinal centres (GCs) formation in inflamed tissues [62,63], and BAK1 and BAX are overexpressed in B cell lymphoma [64]
Summary
Sjögren’s syndrome (SS) is a chronic systemic rheumatic disease characterized by lymphoplasmacytic infiltration of the exocrine glands—especially salivary and lachrymal glands—responsible for sicca syndrome and systemic manifestations. Henri Gougerot (1881–1955) was a French dermatologist who described in 1925 three clinical cases characterized by generalized mucous dryness (eyes, mouth, nose, trachea and vagina) associated with atrophy of the salivary glands (SG). He was the first to describe that xerostomia and ocular dryness are part of a larger sicca syndrome resulting from dysfunction of the exocrine glands or their autonomic innervation. His thesis was not successful, and he stopped his academic career but not his medical and scientific one It was only in the years 1935–1943 that Sjögren’s work was recognized and that the term “Sjögren’s syndrome” has been used since. Henrik Sjögren died of pneumonia on 17 September 1986, several years after a disabling stroke [3,4,5]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
