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

Journal of Clinical Medicine
Dorian ParisisChristine Delporte

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.

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Citations

Dec 4, 2020·Journal of Clinical Medicine·Margherita Sisto
Jan 15, 2021·International Journal of Molecular Sciences·Clara ChivassoMuhammad Shahnawaz Soyfoo
Apr 4, 2021·International Journal of Molecular Sciences·Margherita SistoSabrina Lisi
Jul 7, 2021·Rheumatology and Therapy·Zhiyong Liu, Aichun Chu
Nov 4, 2021·BioDrugs : Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy·Bin WangGuixiu Shi

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Methods Mentioned

BETA
biopsies
ubiquitination
bronchoalveolar
lavage
X-ray
biopsy
biopsying
ELISA
ELISAs
electrophoresis

Software Mentioned

EMBODY
TRACTISS
JOQUER
OMERACT

Related Concepts

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Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

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