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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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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. |
format | Online Article Text |
id | pubmed-7408693 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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