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

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...

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Autores principales: Parisis, Dorian, Chivasso, Clara, Perret, Jason, Soyfoo, Muhammad Shahnawaz, Delporte, Christine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7408693/
https://www.ncbi.nlm.nih.gov/pubmed/32698400
http://dx.doi.org/10.3390/jcm9072299
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author Parisis, Dorian
Chivasso, Clara
Perret, Jason
Soyfoo, Muhammad Shahnawaz
Delporte, Christine
author_facet Parisis, Dorian
Chivasso, Clara
Perret, Jason
Soyfoo, Muhammad Shahnawaz
Delporte, Christine
author_sort Parisis, Dorian
collection PubMed
description 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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spelling pubmed-74086932020-08-13 Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy Parisis, Dorian Chivasso, Clara Perret, Jason Soyfoo, Muhammad Shahnawaz Delporte, Christine J Clin Med Review 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. MDPI 2020-07-20 /pmc/articles/PMC7408693/ /pubmed/32698400 http://dx.doi.org/10.3390/jcm9072299 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Parisis, Dorian
Chivasso, Clara
Perret, Jason
Soyfoo, Muhammad Shahnawaz
Delporte, Christine
Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
title Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
title_full Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
title_fullStr Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
title_full_unstemmed Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
title_short Current State of Knowledge on Primary Sjögren’s Syndrome, an Autoimmune Exocrinopathy
title_sort current state of knowledge on primary sjögren’s syndrome, an autoimmune exocrinopathy
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7408693/
https://www.ncbi.nlm.nih.gov/pubmed/32698400
http://dx.doi.org/10.3390/jcm9072299
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