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Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development
OBJECTIVES: To study the clinical, serological and histologic features of primary Sjögren’s syndrome (pSS) patients with early (young ≤35 years) or late (old ≥65 years) onset and to explore the differential effect on lymphoma development. METHODS: From a multicentre study population of 1997 consecut...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7604905/ https://www.ncbi.nlm.nih.gov/pubmed/33193443 http://dx.doi.org/10.3389/fimmu.2020.594096 |
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author | Goules, Andreas V. Argyropoulou, Ourania D. Pezoulas, Vasileios C. Chatzis, Loukas Critselis, Elena Gandolfo, Saviana Ferro, Francesco Binutti, Marco Donati, Valentina Zandonella Callegher, Sara Venetsanopoulou, Aliki Zampeli, Evangelia Mavrommati, Maria Voulgari, Paraskevi V. Exarchos, Themis Mavragani, Clio P. Baldini, Chiara Skopouli, Fotini N. Fotiadis, Dimitrios I. De Vita, Salvatore Moutsopoulos, Haralampos M. Tzioufas, Athanasios G. |
author_facet | Goules, Andreas V. Argyropoulou, Ourania D. Pezoulas, Vasileios C. Chatzis, Loukas Critselis, Elena Gandolfo, Saviana Ferro, Francesco Binutti, Marco Donati, Valentina Zandonella Callegher, Sara Venetsanopoulou, Aliki Zampeli, Evangelia Mavrommati, Maria Voulgari, Paraskevi V. Exarchos, Themis Mavragani, Clio P. Baldini, Chiara Skopouli, Fotini N. Fotiadis, Dimitrios I. De Vita, Salvatore Moutsopoulos, Haralampos M. Tzioufas, Athanasios G. |
author_sort | Goules, Andreas V. |
collection | PubMed |
description | OBJECTIVES: To study the clinical, serological and histologic features of primary Sjögren’s syndrome (pSS) patients with early (young ≤35 years) or late (old ≥65 years) onset and to explore the differential effect on lymphoma development. METHODS: From a multicentre study population of 1997 consecutive pSS patients, those with early or late disease onset, were matched and compared with pSS control patients of middle age onset. Data driven analysis was applied to identify the independent variables associated with lymphoma in both age groups. RESULTS: Young pSS patients (19%, n = 379) had higher frequency of salivary gland enlargement (SGE, lymphadenopathy, Raynaud’s phenomenon, autoantibodies, C4 hypocomplementemia, hypergammaglobulinemia, leukopenia, and lymphoma (10.3% vs. 5.7%, p = 0.030, OR = 1.91, 95% CI: 1.11–3.27), while old pSS patients (15%, n = 293) had more frequently dry mouth, interstitial lung disease, and lymphoma (6.8% vs. 2.1%, p = 0.011, OR = 3.40, 95% CI: 1.34–8.17) compared to their middle-aged pSS controls, respectively. In young pSS patients, cryoglobulinemia, C4 hypocomplementemia, lymphadenopathy, and SGE were identified as independent lymphoma associated factors, as opposed to old pSS patients in whom SGE, C4 hypocomplementemia and male gender were the independent lymphoma associated factors. Early onset pSS patients displayed two incidence peaks of lymphoma within 3 years of onset and after 10 years, while in late onset pSS patients, lymphoma occurred within the first 6 years. CONCLUSION: Patients with early and late disease onset constitute a significant proportion of pSS population with distinct clinical phenotypes. They possess a higher prevalence of lymphoma, with different predisposing factors and lymphoma distribution across time. |
format | Online Article Text |
id | pubmed-7604905 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-76049052020-11-13 Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development Goules, Andreas V. Argyropoulou, Ourania D. Pezoulas, Vasileios C. Chatzis, Loukas Critselis, Elena Gandolfo, Saviana Ferro, Francesco Binutti, Marco Donati, Valentina Zandonella Callegher, Sara Venetsanopoulou, Aliki Zampeli, Evangelia Mavrommati, Maria Voulgari, Paraskevi V. Exarchos, Themis Mavragani, Clio P. Baldini, Chiara Skopouli, Fotini N. Fotiadis, Dimitrios I. De Vita, Salvatore Moutsopoulos, Haralampos M. Tzioufas, Athanasios G. Front Immunol Immunology OBJECTIVES: To study the clinical, serological and histologic features of primary Sjögren’s syndrome (pSS) patients with early (young ≤35 years) or late (old ≥65 years) onset and to explore the differential effect on lymphoma development. METHODS: From a multicentre study population of 1997 consecutive pSS patients, those with early or late disease onset, were matched and compared with pSS control patients of middle age onset. Data driven analysis was applied to identify the independent variables associated with lymphoma in both age groups. RESULTS: Young pSS patients (19%, n = 379) had higher frequency of salivary gland enlargement (SGE, lymphadenopathy, Raynaud’s phenomenon, autoantibodies, C4 hypocomplementemia, hypergammaglobulinemia, leukopenia, and lymphoma (10.3% vs. 5.7%, p = 0.030, OR = 1.91, 95% CI: 1.11–3.27), while old pSS patients (15%, n = 293) had more frequently dry mouth, interstitial lung disease, and lymphoma (6.8% vs. 2.1%, p = 0.011, OR = 3.40, 95% CI: 1.34–8.17) compared to their middle-aged pSS controls, respectively. In young pSS patients, cryoglobulinemia, C4 hypocomplementemia, lymphadenopathy, and SGE were identified as independent lymphoma associated factors, as opposed to old pSS patients in whom SGE, C4 hypocomplementemia and male gender were the independent lymphoma associated factors. Early onset pSS patients displayed two incidence peaks of lymphoma within 3 years of onset and after 10 years, while in late onset pSS patients, lymphoma occurred within the first 6 years. CONCLUSION: Patients with early and late disease onset constitute a significant proportion of pSS population with distinct clinical phenotypes. They possess a higher prevalence of lymphoma, with different predisposing factors and lymphoma distribution across time. Frontiers Media S.A. 2020-10-19 /pmc/articles/PMC7604905/ /pubmed/33193443 http://dx.doi.org/10.3389/fimmu.2020.594096 Text en Copyright © 2020 Goules, Argyropoulou, Pezoulas, Chatzis, Critselis, Gandolfo, Ferro, Binutti, Donati, Zandonella Callegher, Venetsanopoulou, Zampeli, Mavrommati, Voulgari, Exarchos, Mavragani, Baldini, Skopouli, Fotiadis, De Vita, Moutsopoulos and Tzioufas http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Goules, Andreas V. Argyropoulou, Ourania D. Pezoulas, Vasileios C. Chatzis, Loukas Critselis, Elena Gandolfo, Saviana Ferro, Francesco Binutti, Marco Donati, Valentina Zandonella Callegher, Sara Venetsanopoulou, Aliki Zampeli, Evangelia Mavrommati, Maria Voulgari, Paraskevi V. Exarchos, Themis Mavragani, Clio P. Baldini, Chiara Skopouli, Fotini N. Fotiadis, Dimitrios I. De Vita, Salvatore Moutsopoulos, Haralampos M. Tzioufas, Athanasios G. Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development |
title | Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development |
title_full | Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development |
title_fullStr | Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development |
title_full_unstemmed | Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development |
title_short | Primary Sjögren’s Syndrome of Early and Late Onset: Distinct Clinical Phenotypes and Lymphoma Development |
title_sort | primary sjögren’s syndrome of early and late onset: distinct clinical phenotypes and lymphoma development |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7604905/ https://www.ncbi.nlm.nih.gov/pubmed/33193443 http://dx.doi.org/10.3389/fimmu.2020.594096 |
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