scholarly journals Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren's syndrome

2013 ◽  
Vol 45 (11) ◽  
pp. 1284-1292 ◽  
Author(s):  
Christopher J Lessard ◽  
◽  
He Li ◽  
Indra Adrianto ◽  
John A Ice ◽  
...  
Keyword(s):  
Immune Responses ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Adaptive Immune Responses ◽  
Adaptive Immune ◽  
Multiple Loci ◽  
Sjögren‘S Syndrome

scholarly journals Role of the Innate Immunity Signaling Pathway in the Pathogenesis of Sjögren’s Syndrome

2021 ◽  
Vol 22 (6) ◽  
pp. 3090
Author(s):  
Toshimasa Shimizu ◽  
Hideki Nakamura ◽  
Atsushi Kawakami
Keyword(s):  
Immune Responses ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Innate Immune ◽  
Type I ◽  
Type I Ifn ◽  
Adaptive Immune ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by chronic inflammation of the salivary and lacrimal glands and extra-glandular lesions. Adaptive immune response including T- and B-cell activation contributes to the development of SS. However, its pathogenesis has not yet been elucidated. In addition, several patients with SS present with the type I interferon (IFN) signature, which is the upregulation of the IFN-stimulated genes induced by type I IFN. Thus, innate immune responses including type I IFN activity are associated with SS pathogenesis. Recent studies have revealed the presence of activation pattern recognition receptors (PRRs) including Toll-like receptors, RNA sensor retinoic acid-inducible gene I and melanoma differentiation-associated gene 5, and inflammasomes in infiltrating and epithelial cells of the salivary glands among patients with SS. In addition, the activation of PRRs via the downstream pathway such as the type I IFN signature and nuclear factor kappa B can directly cause organ inflammation, and it is correlated with the activation of adaptive immune responses. Therefore, this study assessed the role of the innate immune signal pathway in the development of inflammation and immune abnormalities in SS.

scholarly journals The Involvement of Innate and Adaptive Immunity in the Initiation and Perpetuation of Sjögren’s Syndrome

2021 ◽  
Vol 22 (2) ◽  
pp. 658
Author(s):  
Clara Chivasso ◽  
Julie Sarrand ◽  
Jason Perret ◽  
Christine Delporte ◽  
Muhammad Shahnawaz Soyfoo
Keyword(s):  
Immune System ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Adaptive Immune System ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Sicca Syndrome ◽  
Neurological Involvement ◽  
Adaptive Immune ◽  
Sjögren‘S Syndrome

: Sjogren’s syndrome (SS) is a chronic autoimmune disease characterized by the infiltration of exocrine glands including salivary and lachrymal glands responsible for the classical dry eyes and mouth symptoms (sicca syndrome). The spectrum of disease manifestations stretches beyond the classical sicca syndrome with systemic manifestations including arthritis, interstitial lung involvement, and neurological involvement. The pathophysiology underlying SS is not well deciphered, but several converging lines of evidence have supported the conjuncture of different factors interplaying together to foster the initiation and perpetuation of the disease. The innate and adaptive immune system play a cardinal role in this process. In this review, we discuss the inherent parts played by both the innate and adaptive immune system in the pathogenesis of SS.

scholarly journals Immune Response Targeting Sjögren’s Syndrome Antigen Ro52 Suppresses Tear Production in Female Mice

2018 ◽  
Vol 19 (10) ◽  
pp. 2935 ◽  
Author(s):  
Marta Trzeciak ◽  
Harini Bagavant ◽  
Joanna Papinska ◽  
Umesh Deshmukh
Keyword(s):  
Immune Responses ◽  
Sjögren’S Syndrome ◽  
Dry Eye ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Lacrimal Glands ◽  
Female Mice ◽  
Tear Production ◽  
Sjögren‘S Syndrome

Autoantibodies reactive against Ro52 are present in 70% of Sjögren’s syndrome patients and are associated with higher disease severity. However, their role in causing aqueous deficient dry eye, a major cause for morbidity in Sjögren’s syndrome, is unclear. To investigate whether immune responses targeting Ro52 contribute towards the dry eye, male and female NZM2758 mice were immunized with recombinant Ro52. Tear production was measured by the phenol red thread test. Sera were analyzed for anti-Ro52 levels by immunoprecipitation. Lacrimal glands were evaluated for inflammatory foci and IgG deposits. Our results showed that, although all mice generated anti-Ro52 antibodies, only females developed a significant drop in tear production. None of the mice developed severe lacrimal gland inflammation, and female mice with anti-Ro52 showed higher levels of IgG deposits within their glands. Passive transfer of anti-Ro52 sera caused reduced tear production in female mice, but not in males. This study demonstrates for the first time that immune responses initiated by Ro52 induce aqueous dry eye, and this may be driven by anti-Ro52 antibodies. Furthermore, the sexual dimorphism in glandular dysfunction suggests that the lacrimal glands in females are more susceptible to autoantibody-mediated injury.

Viral antigens elicit augmented immune responses in primary Sjögren’s syndrome

Rheumatology ◽  
2019 ◽  
Vol 59 (7) ◽  
pp. 1651-1661 ◽  
Author(s):  
Albin Björk ◽  
Gudny Ella Thorlacius ◽  
Johannes Mofors ◽  
Elina Richardsdotter Andersson ◽  
Margarita Ivanchenko ◽  
...  
Keyword(s):  
Immune Responses ◽  
Sjögren’S Syndrome ◽  
Plasma Proteins ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Disease Symptoms ◽  
Patient Reported ◽  
Sjögren‘S Syndrome

Abstract Objectives Infections have been suggested in the pathogenesis of primary SS (pSS). Systematic studies of immune responses to microbial antigens in vivo may be performed during vaccination. In the present study, we therefore longitudinally followed patients with pSS and controls during split-virion influenza vaccination to identify pSS-specific cellular, transcriptomic and serological responses. Methods Patients without treatment (pSSUntr, n = 17), on hydroxychloroquine-treatment (pSSHCQ, n = 8), and healthy controls (n = 16) were included. Antibody titres were determined by ELISA. Plasma proteins were measured by proximity extension assay. Monocyte gene expression was assessed by Nanostring. Routine laboratory tests were performed and clinical disease symptoms were registered by questionnaires. Results pSSUntr developed higher vaccine-specific IgG titres compared with controls. Notably, anti-Ro52 autoantibody titres increased in pSSUntr but remained unchanged in pSSHCQ. No changes in disease symptoms including EULAR Sjögren's Syndrome Patient Reported Index score were registered. Twenty-four hours after vaccination, the leucocyte count in pSSUntr decreased, with a concomitant increase of CCL7 in plasma. Transcriptomic analysis in monocytes revealed differential vaccination-related expression of the NEMO/IKBKG gene, and its higher induced expression in pSSUntr associated with higher serological vaccine responses. Moreover, titres of vaccine-specific antibodies were associated with higher vaccination-induced NF-κB signalling and higher steady-state IFN signatures in monocytes, and with the levels of several plasma proteins with soluble PD-1 displaying the strongest association. Conclusion We observed augmented innate and adaptive immune responses in pSS following viral antigen exposure suggesting an underlying hyper-responsiveness to immune challenges, supporting a role for infections driving the immunopathology and acting as environmental risk factor for pSS.

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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