Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.

<h4>Purpose</h4>To report the significance of extraglandular ocular involvement and long-term systemic morbidity and mortality in primary Sjögren's Syndrome (SS).<h4>Methods</h4>This retrospective, longitudinal cohort study included consecutive patients with primary SS e...

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Main Authors: Priya M Mathews, Susan A Robinson, Anisa Gire, Alan N Baer, Esen K Akpek
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2020-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0239769
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author Priya M Mathews
Susan A Robinson
Anisa Gire
Alan N Baer
Esen K Akpek
author_facet Priya M Mathews
Susan A Robinson
Anisa Gire
Alan N Baer
Esen K Akpek
author_sort Priya M Mathews
collection DOAJ
description <h4>Purpose</h4>To report the significance of extraglandular ocular involvement and long-term systemic morbidity and mortality in primary Sjögren's Syndrome (SS).<h4>Methods</h4>This retrospective, longitudinal cohort study included consecutive patients with primary SS evaluated at a tertiary referral center. An electronic chart review was performed and all available data were extracted from clinic visits between October 1999 and March 2019. The primary outcome measures included occurrence of extraglandular ocular manifestations of SS, serological markers, prevalence of malignancy, and incidence of death.<h4>Results</h4>One hundred and twenty-six SS patients with minimum 3 years of follow-up (median 9.6, range 3.0-15.9 years, total of 1,235 patient-years) were included. Of those, 10 patients with inflammatory keratolysis or scleritis had 2.3 times greater likelihood of death compared to the rest of the cohort (OR = 2.3, 95% confidence interval [CI] 0.5 to 4.0, p = 0.01) due to SS related complications. The lifetime prevalence of any malignancy in the entire cohort was 15.5%. The most common hematologic malignancy was non-Hodgkin's lymphoma (4.8%) and the most common solid malignancy was breast cancer (6.0%). Men SS patients were more likely to have a history of or concurrent malignancy compared to women (30.0% versus 13.7%, p = 0.16) and double the mortality (OR = 2.1, 95% CI 0.09 to 1.4, p = 0.04), independent of malignancy.<h4>Conclusions</h4>SS patients with serious ocular manifestations, particularly men, may be at greater risk for mortality due to SS complications. The eye seems to be the barometer of systemic disease activity.
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spelling doaj.art-40a88fb224f147529754bed81d1b394e2022-12-21T19:08:27ZengPublic Library of Science (PLoS)PLoS ONE1932-62032020-01-01159e023976910.1371/journal.pone.0239769Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.Priya M MathewsSusan A RobinsonAnisa GireAlan N BaerEsen K Akpek<h4>Purpose</h4>To report the significance of extraglandular ocular involvement and long-term systemic morbidity and mortality in primary Sjögren's Syndrome (SS).<h4>Methods</h4>This retrospective, longitudinal cohort study included consecutive patients with primary SS evaluated at a tertiary referral center. An electronic chart review was performed and all available data were extracted from clinic visits between October 1999 and March 2019. The primary outcome measures included occurrence of extraglandular ocular manifestations of SS, serological markers, prevalence of malignancy, and incidence of death.<h4>Results</h4>One hundred and twenty-six SS patients with minimum 3 years of follow-up (median 9.6, range 3.0-15.9 years, total of 1,235 patient-years) were included. Of those, 10 patients with inflammatory keratolysis or scleritis had 2.3 times greater likelihood of death compared to the rest of the cohort (OR = 2.3, 95% confidence interval [CI] 0.5 to 4.0, p = 0.01) due to SS related complications. The lifetime prevalence of any malignancy in the entire cohort was 15.5%. The most common hematologic malignancy was non-Hodgkin's lymphoma (4.8%) and the most common solid malignancy was breast cancer (6.0%). Men SS patients were more likely to have a history of or concurrent malignancy compared to women (30.0% versus 13.7%, p = 0.16) and double the mortality (OR = 2.1, 95% CI 0.09 to 1.4, p = 0.04), independent of malignancy.<h4>Conclusions</h4>SS patients with serious ocular manifestations, particularly men, may be at greater risk for mortality due to SS complications. The eye seems to be the barometer of systemic disease activity.https://doi.org/10.1371/journal.pone.0239769
spellingShingle Priya M Mathews
Susan A Robinson
Anisa Gire
Alan N Baer
Esen K Akpek
Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.
PLoS ONE
title Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.
title_full Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.
title_fullStr Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.
title_full_unstemmed Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.
title_short Extraglandular ocular involvement and morbidity and mortality in primary Sjögren's Syndrome.
title_sort extraglandular ocular involvement and morbidity and mortality in primary sjogren s syndrome
url https://doi.org/10.1371/journal.pone.0239769
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