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Sjögren’s syndrome: still not fully understood disease
Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren’s syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendriti...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4308635/ https://www.ncbi.nlm.nih.gov/pubmed/24985362 http://dx.doi.org/10.1007/s00296-014-3072-5 |
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author | Maślińska, Maria Przygodzka, Małgorzata Kwiatkowska, Brygida Sikorska-Siudek, Katarzyna |
author_facet | Maślińska, Maria Przygodzka, Małgorzata Kwiatkowska, Brygida Sikorska-Siudek, Katarzyna |
author_sort | Maślińska, Maria |
collection | PubMed |
description | Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren’s syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendritic cells) and pathways (BAFF/APRIL and interferons) leading to the development of autoimmunity is continually expanding. For clinicians, the most frequent symptoms are dryness of eyes and mouth, but often the patients have musculoskeletal symptoms and systemic manifestations. However, the increased risk of lymphoproliferative disorders in this group of patients, most commonly B-cell marginal zone lymphoma, is particularly important. Recent separation of IgG4-related diseases and attempts to create further diagnostic criteria for pSS testify to the difficulties, and at the same time a large interest, in understanding the disease so as to allow the effective treatment. This article draws attention to the problems faced by the clinician wishing to securely identify pSS by using accurate laboratory biomarkers and useful imaging tools and predict the development of complications associated with this, still not fully understood, autoimmune disease. |
format | Online Article Text |
id | pubmed-4308635 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-43086352015-01-30 Sjögren’s syndrome: still not fully understood disease Maślińska, Maria Przygodzka, Małgorzata Kwiatkowska, Brygida Sikorska-Siudek, Katarzyna Rheumatol Int Review Article Primary Sjögren's syndrome is an autoimmune disorder with external exocrine glands dysfunction and multiorgan involvement. The pathogenesis of primary Sjogren’s syndrome is still unclear; however, our knowledge of the involvement of different cells (e.g., B and T cells, macrophages and dendritic cells) and pathways (BAFF/APRIL and interferons) leading to the development of autoimmunity is continually expanding. For clinicians, the most frequent symptoms are dryness of eyes and mouth, but often the patients have musculoskeletal symptoms and systemic manifestations. However, the increased risk of lymphoproliferative disorders in this group of patients, most commonly B-cell marginal zone lymphoma, is particularly important. Recent separation of IgG4-related diseases and attempts to create further diagnostic criteria for pSS testify to the difficulties, and at the same time a large interest, in understanding the disease so as to allow the effective treatment. This article draws attention to the problems faced by the clinician wishing to securely identify pSS by using accurate laboratory biomarkers and useful imaging tools and predict the development of complications associated with this, still not fully understood, autoimmune disease. Springer Berlin Heidelberg 2014-07-02 2015 /pmc/articles/PMC4308635/ /pubmed/24985362 http://dx.doi.org/10.1007/s00296-014-3072-5 Text en © The Author(s) 2014 https://creativecommons.org/licenses/by/4.0/ Open AccessThis article is distributed under the terms of the Creative Commons Attribution License which permits any use, distribution, and reproduction in any medium, provided the original author(s) and the source are credited. |
spellingShingle | Review Article Maślińska, Maria Przygodzka, Małgorzata Kwiatkowska, Brygida Sikorska-Siudek, Katarzyna Sjögren’s syndrome: still not fully understood disease |
title | Sjögren’s syndrome: still not fully understood disease |
title_full | Sjögren’s syndrome: still not fully understood disease |
title_fullStr | Sjögren’s syndrome: still not fully understood disease |
title_full_unstemmed | Sjögren’s syndrome: still not fully understood disease |
title_short | Sjögren’s syndrome: still not fully understood disease |
title_sort | sjögren’s syndrome: still not fully understood disease |
topic | Review Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4308635/ https://www.ncbi.nlm.nih.gov/pubmed/24985362 http://dx.doi.org/10.1007/s00296-014-3072-5 |
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