scholarly journals Introductory Chapter: Autoimmune Epithelitis - Discussion about Sjögren’s Syndrome and Primary Biliary Cholangitis

2019 ◽  
Author(s):  
Maria Maślińska
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Biliary Cholangitis ◽  
Sjögren‘S Syndrome

scholarly journals Epigenetic Modifications in Generalized Autoimmune Epithelitis: Sjögren’s Syndrome and Primary Biliary Cholangitis

Epigenomes ◽  
2019 ◽  
Vol 3 (3) ◽  
pp. 15
Author(s):  
Pinelopi Arvaniti ◽  
Kalliopi Zachou ◽  
Aggeliki Lyberopoulou ◽  
Nikolaos K. Gatselis ◽  
Wesley H. Brooks ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Biliary Cholangitis ◽  
Epigenetic Changes ◽  
Disease Pathogenesis ◽  
Immune Mediated ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SjS) and primary biliary cholangitis (PBC) can be classified as a model of generalized autoimmune epithelitis based on their frequent coexistence in clinical practice and the highly specific immune mediated injury of target epithelial cells. Both of these autoimmune diseases are characterized by female predominance, highly specific circulating autoantibodies, and immune-mediated destruction of the salivary and lachrymal glands and the biliary epithelial cells, respectively. Although the genetic predisposition has been well described for both diseases, genetic studies have failed to completely elucidate their pathogenesis. The recent integration of epigenetic data, analyzing the different cellular partners, opens new perspectives and allows for better understanding of these complex and still incurable diseases. Epigenetic studies on SjS have elucidated the role of DNA methylation alterations in disease pathogenesis, while epigenetic changes that influence expression of genes on the X chromosome have been implicated in the geo-variability and occurrence of PBC. The aim of this review is to describe the advances in epigenetics in the field of autoimmune epithelitis as well as to highlight how epigenetic changes could contribute to better understanding of disease pathogenesis and progression. These advances could yield insights on novel therapeutic interventions.

scholarly journals THU0045 IDENTIFICATION OF NOVEL CENTROMERE AUTOANTIGENS IN SJÖGREN’S SYNDROME, SYSTEMIC SCLEROSIS AND PRIMARY BILIARY CHOLANGITIS.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 235.2-235
Author(s):  
N. Kajio ◽  
M. Takeshita ◽  
K. Suzuki ◽  
T. Takeuchi
Keyword(s):  
Systemic Sclerosis ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Biliary Cholangitis ◽  
Chugai Pharmaceutical ◽  
Centromere Protein ◽  
Centromere Proteins ◽  
Sjögren‘S Syndrome

Background:Anti-centromere antibodies (ACA) are detected in the serum of patients with various autoimmune diseases including Sjögren’s syndrome (SjS), systemic sclerosis (SSc) and primary biliary cholangitis (PBC). ACA positivity is correlated with clinical manifestations such as Raynaud’s phenomenon and sclerodactyly and these features are commonly seen across diseases. Although CENPB is thought to be the major antigen against ACA, autoimmune features of other centromere proteins have not been fully evaluated.Objectives:The aim of this study is to elucidate centromere autoantigens comprehensively and clarify their association with pathogenesis of SjS, SSc and PBC.Methods:A centromere protein library was created by cloning 6 single proteins and 10 complexes consisting of 35 proteins belonging to human centromere region. The centromere antigens were immobilized on beads and incubated in the serum of patients with SjS (n = 86), SSc (n = 35), PBC (n = 10), patients with two or more diseases above (n = 44), and healthy volunteers (n = 68). Autoantibodies to each centromere protein were analyzed by flow cytometry.Results:Patients had a wide variety of antibodies against most of centromere antigens including 4 newly identified autoantigens. The hierarchical clustering of each antigen distinguished 2 antigen clusters. The reactivity of autoantibodies against a centromere protein of one cluster was mutually correlated regardless of disease types, indicating that these proteins/protein complexes might be the target of ACA. In addition, our method enabled us to detect sera reacted against multiple centromere antigens in some of the ACA-negative patients with existing methods.Conclusion:We identified 4 novel centromere autoantigens and our data suggested that the main target of ACA was the protein complex rather than a single specific antigen in SjS, SSc and PBC patients. Using the combination of centromere proteins may be useful to detect ACA with higher sensitivity.References:[1]Fritzler MJ, Rattner JB, Luft LM, Edworthy SM, Casiano CA, Peebles C, Mahler M. Historical perspectives on the discovery and elucidation of autoantibodies to centromere proteins (CENP) and the emerging importance of antibodies to CENP-F. Autoimmun Rev. 2011;10:194-200.Disclosure of Interests:Nobuhiko Kajio: None declared, Masaru Takeshita: None declared, Katsuya Suzuki: None declared, Tsutomu Takeuchi Grant/research support from: Eisai Co., Ltd, Astellas Pharma Inc., AbbVie GK, Asahi Kasei Pharma Corporation, Nippon Kayaku Co., Ltd, Takeda Pharmaceutical Company Ltd, UCB Pharma, Shionogi & Co., Ltd., Mitsubishi-Tanabe Pharma Corp., Daiichi Sankyo Co., Ltd., Chugai Pharmaceutical Co. Ltd., Consultant of: Chugai Pharmaceutical Co Ltd, Astellas Pharma Inc., Eli Lilly Japan KK, Speakers bureau: AbbVie GK, Eisai Co., Ltd, Mitsubishi-Tanabe Pharma Corporation, Chugai Pharmaceutical Co Ltd, Bristol-Myers Squibb Company, AYUMI Pharmaceutical Corp., Eisai Co., Ltd, Daiichi Sankyo Co., Ltd., Gilead Sciences, Inc., Novartis Pharma K.K., Pfizer Japan Inc., Sanofi K.K., Dainippon Sumitomo Co., Ltd.

scholarly journals SJOGREN'S SYNDROME AND PRIMARY BILIARY CHOLANGITIS OVERLAP: GOOD RESPONSE TO HYDROXYCHLOROQUINE TREATMENT

2021 ◽  
Author(s):  
Heitor Buback Araujo ◽  
Thiago Loureiro Mendes ◽  
Aldren Thomazini Falçoni Júnior ◽  
Bianca Savazzini Reis ◽  
Diego Goltara Severgnine ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Biliary Cholangitis ◽  
Sjögren‘S Syndrome

scholarly journals Concomitant Sjögren’s Syndrome Was Not Associated with a Poorer Response or Outcomes in Ursodeoxycholic Acid-Treated Patients with Primary Biliary Cholangitis

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Ping Ni ◽  
Ruoting Men ◽  
Mengyi Shen ◽  
Tingting Wang ◽  
Chen Huang ◽  
...  
Keyword(s):  
Ursodeoxycholic Acid ◽  
Clinical Outcomes ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Risk Scores ◽  
Primary Biliary Cholangitis ◽  
Significant Difference ◽  
Sjögren‘S Syndrome

Aim. Patients with primary biliary cholangitis (PBC) have at least 60% probability of having an autoimmune extrahepatic condition, with the most common being Sjögren’s syndrome (SS). The impacts of SS on the response and outcomes in ursodeoxycholic acid (UDCA)-treated patients with PBC, however, remain unclear. The aim of this study was to document the biochemical responses and clinical outcomes of UDCA-treated patients with concomitant SS and to compare the findings to those of patients with PBC alone. Methods. Data from consecutive patients with PBC who visited West China Hospital affiliated with Sichuan University between October 2013 and October 2017 were reviewed retrospectively. Results. The study populations consisted of 226 patients with PBC alone and 56 with PBC/SS. The median ages, proportions of female patients, Fib-4 scores, and aspartate aminotransferase (AST)/platelet ratio index (APRI) at baseline in the two cohorts were similar. At presentation, patients with PBC/SS had higher serum IgG levels and positive rates for serum antinuclear antibody (ANA) than patients with PBC alone (all P < 0.05). There was no statistically significant difference between the rate of biochemical response to UDCA at 1 year in the PBC/SS and PBC alone groups. The UK-PBC risk scores and GLOBE scores in UDCA-treated patients in the two cohorts were also similar. During the follow-up period, the differences in the liver enzyme levels, Fib-4 scores, APRI, and incidence of liver-related adverse events were not significant. Conclusions. The results of this retrospective, single-center study suggest that the response and clinical outcomes of UDCA-treated patients with PBC are not adversely affected by concomitant SS.

The association of Raynaud’s phenomenon/syndrome with scleroderma and Sjögren’s syndrome – a meta-analysis

VASA ◽  
2008 ◽  
Vol 37 (Supplement 73) ◽  
pp. 26-32 ◽  
Author(s):  
Schlattmann ◽  
Höhne ◽  
Plümper ◽  
Heidrich
Keyword(s):  
Quantitative Analysis ◽  
Sjögren’S Syndrome ◽  
Mixture Model ◽  
Sjögren's Syndrome ◽  
Meta Analysis ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Raynaud’S Syndrome ◽  
Raynaud's Syndrome ◽  
Sjögren‘S Syndrome

Background: In order to analyze the prevalence of Raynaud’s syndrome in diseases such as scleroderma and Sjögren’s syndrom – a meta-analysis of published data was performed. Methods: The PubMed data base of the National Library of Medicine was used for studies dealing with Raynaud’s syndrome and scleroderma or Raynaud’s syndroem and Sjögren’s syndrom respectively. The studies found provided data sufficient to estimate the prevalence of Raynaud’s syndrome. The statistical analysis was based on methods for a fixed effects meta-analysis and finite mixture model for proportions. Results: For scleroderma a pooled prevalence of 80.9% and 95% CI (0.78, 0.83) was obtained. A mixture model analysis found four latent classes. We identified a class with a very low prevalence of 11%, weighted with 0.15. On the other hand there is a class with a very high prevalence of 96%. Analysing the association with Sjögren’s syndrome, the pooled analysis leads to a prevalence of Raynaud’s syndrome of 32%, 95% CI(26.7%, 37.7%). A mixture model finds a solution with two latent classes. Here, 38% of the studies show a prevalence of 18.8% whereas 62% observe a prevalence of 38.3%. Conclusion: There is strong variability of studies reporting the prevalence of Raynaud’s syndrome in patients suffering from scleroderma or Sjögren’s syndrome. The available data are insufficient to perform a proper quantitative analysis of the association of Raynaud’s phenomenon with scleroderma or Sjögren’s syndrome. Properly planned and reported epidemiological studies are needed in order to perform a thorough quantitative analysis of risk factors for Raynaud’s syndrome.

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