Sjögren’s Syndrome: The Clinical Spectrum of Male Patients

Chatzis, Loukas; Pezoulas, Vasileios C.; Ferro, Francesco; Gandolfo, Saviana; Donati, Valentina; Binutti, Marco; Callegher, Sara Zandonella; Venetsanopoulou, Aliki; Zampeli, Evangelia; Mavrommati, Maria; Argyropoulou, Ourania D; Michalopoulos, George; Exarchos, Themis P.; Baldini, Chiara; Skopouli, Fotini N.; Fotiadis, Dimitrios I.; Vita, Salvatore De; Moutsopoulos, Haralampos Μ.; Tzioufas, Athanasios G.; Goules, Andreas V.

doi:10.3390/jcm9082620

Cited by 39 publications

(30 citation statements)

References 23 publications

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“…The increased severity of the ocular surface disease noted in female TSP-1−/− mice are further supported by LG histology studies, in which inflammatory infiltrates in female mice were detected at a younger age (12 weeks) as compared to those reported in male mice (24 weeks) [35]. These findings are consistent with the widely reported overall sex bias in autoimmunity, as well as the observed higher frequency of oral and ocular dryness in female SS patients [36]. The gender differences in clinical manifestations noted in TSP-1 deficient mice also point to a potential role of estrogen-dependent mechanisms underlying SS pathology in this model that remain to be addressed [37].…”

Section: Thrombospondin-1 (Tsp-1) Deficient Micesupporting

confidence: 85%

Mouse Models of Sjögren’s Syndrome with Ocular Surface Disease

Masli

Dartt

2020

IJMS

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Sjögren’s syndrome (SS) is a systemic rheumatic disease that predominantly affects salivary and lacrimal glands resulting in oral and ocular dryness, respectively, referred to as sicca symptoms. The clinical presentation of ocular dryness includes keratoconjunctivitis sicca (KCS), resulting from the inflammatory damage to the ocular surface tissues of cornea and conjunctiva. The diagnostic evaluation of KCS is a critical component of the classification criteria used by clinicians worldwide to confirm SS diagnosis. Therapeutic management of SS requires both topical and systemic treatments. Several mouse models of SS have contributed to our current understanding of immunopathologic mechanisms underlying the disease. This information also helps develop novel therapeutic interventions. Although these models address glandular aspects of SS pathology, their impact on ocular surface tissues is addressed only in a few models such as thrombospondin (TSP)-1 deficient, C57BL/6.NOD.Aec1Aec2, NOD.H2b, NOD.Aire KO, and IL-2Rα (CD25) KO mice. While corneal and/or conjunctival damage is reported in most of these models, the characteristic SS specific autoantibodies are only reported in the TSP-1 deficient mouse model, which is also validated as a preclinical model. This review summarizes valuable insights provided by investigations on the ocular spectrum of the SS pathology in these models.

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Section: Thrombospondin-1 (Tsp-1) Deficient Micesupporting

confidence: 85%

Mouse Models of Sjögren’s Syndrome with Ocular Surface Disease

Masli

Dartt

2020

IJMS

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Section: Introductioncontrasting

confidence: 70%

“… 8 The 20:1 female-to-male ratio was considerably different from what has been commonly described in many previous studies. 8 …”

Section: Introductioncontrasting

confidence: 70%

“…6,7 But recently it has been shown that the ratio of female to male patients with pSS was 20:1. 8 The 20:1 female-to-male ratio was considerably different from what has been commonly described in many previous studies. 8 It has been reported that the monocytes infiltrated by exocrine glands are mainly CD4 + T cells, but other cells are also involved including CD8 + T cells, dendritic cells, and plasma cells.…”

Section: Introductioncontrasting

confidence: 70%

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Molecular Mechanism of Sphingosine-1-Phosphate Receptor 1 Regulating CD4+ Tissue Memory in situ T Cells in Primary Sjogren’s Syndrome

Yang¹,

Yang²,

Wang³

et al. 2021

IJGM

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Objective Although extensive research has been carried out on CD4 + T cells infiltrating the labial glands in patients with primary Sjögren’s Syndrome (pSS), it is still unclear how CD4 + T cells remain in the labial gland tissue and develop into tissue resident cells. The aim of this study was to investigate the molecular mechanism by which CD4 + T reside in labial glandular tissue of pSS patients. Methods Lymphocyte infiltration in labial salivary glands (LSG) of pSS patients was detected by H&E staining. Expression of sphingosine-1-phosphate receptor 1 (S1PR1) in LSG was examined by Immunohistochemistry. Immunofluorescence analyses were utilized to detect the co-expression of CD4, CD69 and S1PR1 in T cells of LSG of pSS patients. Expression of gene S1pr1 in peripheral blood CD4 + T cells of healthy controls and pSS patients was detected by quantitative real-time PCR (QPCR). QPCR was used to examine the expression of gene S1pr1, Klf2, and Cd69 in the CD4 + T cells that were co-cultured in vitro with cytokines TNF-α, TGF-β, and IL-33. Results S1PR1 was expressed in the infiltrating monocytes in LSG of pSS patients, and S1PR1 was weakly or even not expressed in cytoplasm of CD4 + CD69 + T RM cells of LSG in patients with pSS. Expression of gene S1pr1 in peripheral blood CD4 + T cells of pSS patients was about three-fifths of that of healthy controls (P < 0.05). Expression of genes S1pr1 (P < 0.001) and Klf-2 (P < 0.001) was significantly decreased, and the expression of gene Cd69 (P < 0.05) was significantly increased in peripheral blood CD4 + T cells of pSS patients co-cultured in vitro with cytokines TNF-α, TGF-β, and IL-33. Conclusion Our study suggests that the decrease of S1pr1 gene expression may provide a molecular basis for promoting the tissue retention and development of CD4 + CD69 + T RM cells.

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“…It seems that male patients with pSS are more prone to developing extraglandular complications such as ILD and lymphoma, which could be explained partly by the fact that male patients often have a more progressive disease. 25 , 26 In addition, age at diagnosis ⩾50 years old was identified as a poor prognostic factor. Similarly, results from Skopouli et al .…”

Section: Discussionmentioning

confidence: 99%

Ten-year survival analysis of patients with primary Sjögren’s syndrome in China: a national prospective cohort study

Qian

et al. 2021

Therapeutic Advances in Musculoskeletal

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Aims: To investigate the long-term survival of patients with primary Sjögren’s syndrome (pSS) in China. Methods: Patients with pSS who fulfilled the 2002 American–European Consensus Group classification criteria were prospectively enrolled from 2004 to 2011. Their baseline clinical, laboratory, and therapeutic data were collected. The primary endpoint was all-cause death by January 2018. The standard mortality ratio (SMR) was calculated by comparing with age-matched and sex-matched mortality data of the general population. Kaplan–Meier curves were obtained by time-to-event analysis. Univariate and multivariate Cox hazards regression analyses were performed to identify risk factors for mortality. Results: A total of 1054 patients were enrolled and 834 patients were followed up for a median of 94.8 months, with 48 confirmed deaths. The total SMR was 3.63 [95% confidence interval (CI) 2.60–4.66]. The 3-, 5-, and 10-year survival rates were 98.4%, 97.5%, and 92.9%, respectively. Infection, malignancy, and respiratory failure were the top three causes of mortality. We identified male sex [hazard ratio (HR) = 3.00, 95% CI 1.23–7.31], age at diagnosis ⩾50 years of age (HR = 7.69, 95% CI 3.01–19.62), thrombocytopenia (HR = 1.93, 95% CI 1.01–3.72), and interstitial lung disease (HR = 5.96, 95% CI 2.24–15.82) as the independent prognostic factors of death. Conclusions: The mortality rates of Chinese patients with pSS are higher than those of the general population. Male patients of elder age at diagnosis complicated with thrombocytopenia and interstitial lung disease might be suggestive for poorer survival and require closer follow up.

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Sjögren’s Syndrome: The Clinical Spectrum of Male Patients

Cited by 39 publications

References 23 publications

Mouse Models of Sjögren’s Syndrome with Ocular Surface Disease

Mouse Models of Sjögren’s Syndrome with Ocular Surface Disease

Molecular Mechanism of Sphingosine-1-Phosphate Receptor 1 Regulating CD4+ Tissue Memory in situ T Cells in Primary Sjogren’s Syndrome

Ten-year survival analysis of patients with primary Sjögren’s syndrome in China: a national prospective cohort study

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