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Journal of Clinical Medicine May 2024: Sjögren's Syndrome (SS) is a chronic degenerative rheumatic disease. Because of its chronic nature, it significantly affects the quality of life of those who suffer...
: Sjögren's Syndrome (SS) is a chronic degenerative rheumatic disease. Because of its chronic nature, it significantly affects the quality of life of those who suffer from it. : This qualitative study investigated disease experience among women suffering from SS to understand its impact on their overall well-being. In-depth interviews were conducted with 15 women who suffer from SS. Interviews were analyzed using the Grounded Theory methodology, using open, axial, and selective coding. : Three central phenomena of disease experience were identified: invisibility; uncontrollability; and unpredictability. : SS disease experience has a strong imprint on emotional well-being and sense of self-control among middle-aged women. Understanding SS impacts on women's lives is important to better understand the disease and contribute to recognizing potential areas of management and social support in relevant windows of opportunity within the health-disease continuum.
PubMed: 38892941
DOI: 10.3390/jcm13113228 -
Journal of Clinical Medicine May 2024Depressive disorders are a growing problem worldwide. They are also characterized by high comorbidity, including from the circle of dermatological diseases. Autoimmune... (Review)
Review
Depressive disorders are a growing problem worldwide. They are also characterized by high comorbidity, including from the circle of dermatological diseases. Autoimmune diseases seem to be particularly correlated with depressive comorbidity, raising the question of their possible common pathomechanism. The PubMed database was searched, focusing on results published after 2016. A particular reciprocal correlation of depressive disorders with psoriasis, atopic dermatitis, alopecia areata, impetigo, lupus and systemic scleroderma was found. One possible explanation for the co-occurrence of the above diseases is that the inflammatory theory may be applicable to depression, the various elements of which also apply to autoimmune diseases.
PubMed: 38892934
DOI: 10.3390/jcm13113224 -
International Journal of Molecular... May 2024Elevated oxidative stress can play a pivotal role in autoimmune diseases by exacerbating inflammatory responses and tissue damage. In Sjögren's disease (SjD), the...
Elevated oxidative stress can play a pivotal role in autoimmune diseases by exacerbating inflammatory responses and tissue damage. In Sjögren's disease (SjD), the contribution of oxidative stress in the disease pathogenesis remains unclear. To address this question, we created mice with a tamoxifen-inducible conditional knockout (KO) of a critical antioxidant enzyme, superoxide dismutase 2 (, in the salivary glands (i-sg- KO mice). Following tamoxifen treatment, deletion occurred primarily in the ductal epithelium, and the salivary glands showed a significant downregulation of expression. At twelve weeks post-treatment, salivary glands from the i-sg- KO mice exhibited increased 3-Nitrotyrosine staining. Bulk RNA-seq revealed alterations in gene expression pathways related to ribosome biogenesis, mitochondrial function, and oxidative phosphorylation. Significant changes were noted in genes characteristic of salivary gland ionocytes. The i-sg- KO mice developed reversible glandular hypofunction. However, this functional loss was not accompanied by glandular lymphocytic foci or circulating anti-nuclear antibodies. These data demonstrate that although localized oxidative stress in salivary gland ductal cells was insufficient for SjD development, it induced glandular dysfunction. The i-sg- KO mouse resembles patients classified as non-Sjögren's sicca and will be a valuable model for deciphering oxidative-stress-mediated glandular dysfunction and recovery mechanisms.
Topics: Animals; Superoxide Dismutase; Salivary Glands; Sjogren's Syndrome; Mice; Mice, Knockout; Epithelial Cells; Oxidative Stress; Mitochondria; Disease Models, Animal
PubMed: 38892170
DOI: 10.3390/ijms25115983 -
Reumatologia Clinica May 2024Autoimmune diseases are known to be associated with an elevated risk of cardiovascular diseases; however, there exists a lack of awareness regarding this increased risk...
INTRODUCTION
Autoimmune diseases are known to be associated with an elevated risk of cardiovascular diseases; however, there exists a lack of awareness regarding this increased risk among patients.
OBJECTIVE
This study aimed to assess the prevalence of cardiovascular risk factors and events in various systemic autoimmune diseases, including Systemic Sclerosis (SSc), Systemic Lupus Erythematosus (SLE), Rheumatoid Arthritis (RA), and Sjögren's syndrome (SS), matched by age, sex, and disease duration. Additionally, the study aimed to evaluate the perceived and actual risks of cardiovascular disease among patients.
METHODS
A cross-sectional self-reported survey on the patient's perspective of cardiovascular risk was conducted between January and June 2023. Sociodemographic and clinical data, including disease activity, were collected through medical records and questionnaires. Traditional cardiovascular risk factors and events were assessed, alongside the perceived cardiovascular risk. The SCORE calculation and Charlson Comorbidity Index (CCI) were employed for cardiovascular risk assessment.
RESULTS
Survey responses from 180 patients (45 patients each with SSc, SLE, RA, and SS) with systemic autoimmune diseases revealed that 20% perceived a low risk, 23% perceived neither lower nor higher, and 56% perceived a higher risk of developing cardiovascular diseases in the next ten years. Only 45% agreed that their autoimmune disease could increase the risk of a heart attack, even in the absence of other risk factors, and 46.7% were unaware that NSAIDs pose a cardiovascular risk. An association between cardiovascular risk measured by SCORE, comorbidities, and risk perception was observed in RA, SSc, and SS patients, with no association found in SLE patients (p=0.27). Except for SS patients (p=0.02), no association between CCI and disease activity level was found. Regarding the influence of age, working status, and education in CVD risk perception, an association between CVD risk perception and age was observed (p=0.01), with patients over 40 years exhibiting a higher perception of CVD risk. No differences were found regarding working status (p=0.19) nor education level (p=0.06).
CONCLUSIONS
Patients with SS, RA, and SSc displayed a heightened perception of cardiovascular risk, correlating with their actual risk and preexisting comorbidities. However, patients exhibited unawareness of certain cardiovascular risk behaviors. This underscores the need for tailored education programs on cardiovascular risk for autoimmune disease patients, to be implemented at the time of diagnosis and during follow-up in outpatient clinics.
Topics: Humans; Male; Female; Cross-Sectional Studies; Autoimmune Diseases; Cardiovascular Diseases; Middle Aged; Adult; Aged; Heart Disease Risk Factors; Self Report; Arthritis, Rheumatoid; Lupus Erythematosus, Systemic; Sjogren's Syndrome; Scleroderma, Systemic; Risk Assessment; Prevalence; Self Concept; Risk Factors
PubMed: 38880551
DOI: 10.1016/j.reumae.2024.05.002 -
Biomedicine & Pharmacotherapy =... Jun 2024Sjögren's syndrome (SS) is an autoimmune disease in which the salivary glands (SGs) and the lacrimal glands (LGs) are affected by lymphocytic infiltration and...
Sjögren's syndrome (SS) is an autoimmune disease in which the salivary glands (SGs) and the lacrimal glands (LGs) are affected by lymphocytic infiltration and inflammation. It has been reported that interferon-α (IFN-α) released by plasmacytoid dendritic cells (pDCs) contribute to the pathology of SS, and ART has been shown to effectively ameliorates SS. Despite the current research endeavors, the mechanism of how ART works in the treatment of SS remains to be fully elucidated. Whether ART can treat SS by inhibiting IFN-α remains unclear. This hypothesis was tested both in vivo and in vitro settings during the study. The SS model mice, which were treated with ART, showed amelioration in symptoms related to dryness. RNA-seq analysis revealed strong anti-IFN-α signaling response upon ART treatment. Additional in vitro studies provided further confirmation that the application of ART inhibits the MyD88 protein expression and the nuclear translocation of IRF7. This suggests that the intervention of ART in the TLR-MyD88-IRF7 pathway plays a role in the therapeutic approach for SS. In summary, this study highlighted the therapeutic potential of ART in SS and ART inhibited the IFN-α signaling in pDCs via the TLR-MyD88-IRF7 pathway.
PubMed: 38878633
DOI: 10.1016/j.biopha.2024.116885 -
Medicine Jun 2024This study employs CiteSpace software to analyze the research status, hotspots, and trends of primary Sjogren syndrome (pSS). Relevant publications from 1999 to 2023...
This study employs CiteSpace software to analyze the research status, hotspots, and trends of primary Sjogren syndrome (pSS). Relevant publications from 1999 to 2023 were searched in the Web of Science Core Collection (WoSCC) set, followed by generating a network map using CiteSpace software to identify top authors, institutions, countries, keywords, journals, references, and research trends. A total of 3564 valid articles were included in this study. The People Republic of China had the highest number of articles (n = 524), while the University of Bergen emerged as the institution with the highest publication count (n = 94). Mariette X was identified as the author with the most publications (n = 67), whereas Vitali C received recognition as the most cited author (n = 1706). Annals of Rheumatic Diseases stood out as the journal with the highest citation count (n = 2530). Notably, an article published in the Annals of Rheumatic Diseases in 2017 garnered significant attention by being cited a remarkable 304 times. The bibliometric analysis reveals that key areas of research in pSS encompass investigating pathogenesis; advancing and applying targeted biological agents; and establishing treatment and diagnostic standards.
Topics: Sjogren's Syndrome; Bibliometrics; Humans; Software
PubMed: 38875384
DOI: 10.1097/MD.0000000000038162 -
Frontiers in Immunology 2024The effect of immune cells on autoimmune diseases (ADs) complicated by non-Hodgkin lymphoma (NHL) has been widely recognized, but a causal relationship between...
BACKGROUND
The effect of immune cells on autoimmune diseases (ADs) complicated by non-Hodgkin lymphoma (NHL) has been widely recognized, but a causal relationship between regulatory T cell (Treg) immune traits and ADs complicated by NHL remains debated.
METHODS
Aggregate data for 84 Treg-related immune traits were downloaded from the Genome-Wide Association Study (GWAS) catalog, and GWAS data for diffuse large B-cell lymphoma (DLBCL; n=315243), follicular lymphoma (FL; n=325831), sjögren's syndrome (SS; n=402090), rheumatoid arthritis (RA; n=276465), dermatopolymyositis (DM; n=311640), psoriasis (n=407876), atopic dermatitis (AD; n=382254), ulcerative colitis (UC; n=411317), crohn's disease(CD; n=411973) and systemic lupus erythematosus (SLE; n=307587) were downloaded from the FinnGen database. The inverse variance weighting (IVW) method was mainly used to infer any causal association between Treg-related immune traits and DLBCL, FL, SS, DM, RA, Psoriasis, AD, UC, CD and SLE, supplemented by MR-Egger, weighted median, simple mode, and weighted mode. Moreover, we performed sensitivity analyses to assess the validity of the causal relationships.
RESULTS
There was a potential genetic predisposition association identified between CD39+ CD8br AC, CD39+ CD8br % T cell, and the risk of DLBCL (OR=1.51, p<0.001; OR=1.25, p=0.001) (adjusted FDR<0.1). Genetic prediction revealed potential associations between CD25++ CD8br AC, CD28- CD25++ CD8br % T cell, CD39+ CD8br % CD8br, and the risk of FL (OR=1.13, p=0.022; OR=1.28, p=0.042; OR=0.90, p=0.016) (adjusted FDR>0.1). Furthermore, SLE and CD exhibited a genetically predicted potential association with the CD39+ CD8+ Tregs subset. SS and DM were possibly associated with an increase in the quantity of the CD4+ Tregs subset; RA may have reduced the quantity of the CD39+ CD8+ Tregs subset, although no causal relationship was identified. Sensitivity analyses supported the robustness of our findings.
CONCLUSIONS
There existed a genetically predicted potential association between the CD39+ CD8+ Tregs subset and the risk of DLBCL, while SLE and CD were genetically predicted to be potentially associated with the CD39+ CD8+ Tregs subset. The CD39+ CD8+ Tregs subset potentially aided in the clinical diagnosis and treatment of SLE or CD complicated by DLBCL.
Topics: Humans; T-Lymphocytes, Regulatory; Genome-Wide Association Study; Mendelian Randomization Analysis; Risk Factors; Autoimmune Diseases; Lymphoma, Non-Hodgkin; Genetic Predisposition to Disease; Polymorphism, Single Nucleotide
PubMed: 38863695
DOI: 10.3389/fimmu.2024.1374938 -
Primary biliary cholangitis and Sjogren's syndrome: bi-directional Mendelian randomization analysis.European Review For Medical and... May 2024Observational studies have shown a higher prevalence of Sjogren's syndrome (SjS) in patients with primary biliary cholangitis (PBC) than in the healthy population, but...
OBJECTIVE
Observational studies have shown a higher prevalence of Sjogren's syndrome (SjS) in patients with primary biliary cholangitis (PBC) than in the healthy population, but whether this correlation is causal needs further confirmation. This study aimed to investigate the bidirectional causal relationship between PBC and SjS using Mendelian randomization (MR) analysis.
MATERIALS AND METHODS
We used pooled data from a large-scale genome-wide association study (GWAS) to select mutually independent genetic loci associated with PBC and SjS in people of European ancestry as instrumental variables (IVs). The causal association between PBC and SjS was analyzed by MR analysis using inverse variance weighting (IVW) and weighted median methods, and the ratio of ratios (OR) was used as an evaluation index. In addition, sensitivity analyses, including Cochran's Q test, MR-PRESSO, MR-Egger intercept test, and leave-one-out test, were performed to ensure the stability of the results.
RESULTS
A total of 20 validated IVs were selected for PBC, and the number of IVs for SjS was seven. Positive MR analysis showed that genetically predicted PBC was significantly associated with the risk of SjS (IVW OR=1.174, 95% CI: 1.107-1.246, p<0.001). The weighted median method further confirmed this result (OR=1.146, 95% CI: 1.053-1.247, p=0.016). Inverse MR analysis showed that genetic susceptibility to SjS also increased the risk of PBC (IVW OR=1.737, 95% CI: 1.280-2.357, p<0.001), and this result was also confirmed by the weighted median method (OR=1.398, 95% CI: 1.120-1.746, p=0.003).
CONCLUSIONS
Our study found that genetically predicted SjS increased the risk of PBC and vice versa in a European population. This may shed light on the etiology of PBC and the management of patients with SjS.
Topics: Humans; Mendelian Randomization Analysis; Sjogren's Syndrome; Liver Cirrhosis, Biliary; Genome-Wide Association Study; Genetic Predisposition to Disease; Polymorphism, Single Nucleotide
PubMed: 38856131
DOI: 10.26355/eurrev_202405_36292 -
European Review For Medical and... May 2024This study aimed to evaluate the efficacy and safety of total glucosides of paeony (TGP) in patients with primary Sjögren's syndrome (pSS).
OBJECTIVE
This study aimed to evaluate the efficacy and safety of total glucosides of paeony (TGP) in patients with primary Sjögren's syndrome (pSS).
PATIENTS AND METHODS
This study included 236 patients with pSS, including 118 TGP users and 118 non-users. Propensity score matching and Binary logistic regression analyses were used to minimize confounding factors and determine the association between TGP treatment and clinical variables.
RESULTS
The baseline indexes of TGP users and non-users were basically the same. The median time of follow-up in the two groups was also similar (p < 0.05). Compared with non-users, TGP users showed higher rates of improvement in dry mouth and eyes and musculoskeletal involvement, as well as more significant reductions in serum alanine aminotransferase (ALT) and direct bilirubin (DBIL) levels after treatment. Logistic regression confirmed that the use of TGP was negatively correlated with the increase of ALT and DBIL in pSS patients, and the reduction in these variables was more pronounced after 2 years of treatment. The incidence of adverse reactions in the TGP users was 11.9%, which was compatible with those in non-users.
CONCLUSIONS
TGP is often a safe option for treating pSS patients with musculoskeletal features and abnormal ALT levels. Besides, it can help improve dry mouth and dry eyes and decrease DBIL levels.
Topics: Humans; Sjogren's Syndrome; Paeonia; Glucosides; Middle Aged; Female; Male; Propensity Score; Treatment Outcome; Adult; Plant Extracts; Aged
PubMed: 38856127
DOI: 10.26355/eurrev_202405_36287 -
Journal of Neuroengineering and... Jun 2024Many individuals with neurodegenerative (NDD) and immune-mediated inflammatory disorders (IMID) experience debilitating fatigue. Currently, assessments of fatigue rely...
Evaluation of walking activity and gait to identify physical and mental fatigue in neurodegenerative and immune disorders: preliminary insights from the IDEA-FAST feasibility study.
BACKGROUND
Many individuals with neurodegenerative (NDD) and immune-mediated inflammatory disorders (IMID) experience debilitating fatigue. Currently, assessments of fatigue rely on patient reported outcomes (PROs), which are subjective and prone to recall biases. Wearable devices, however, provide objective and reliable estimates of gait, an essential component of health, and may present objective evidence of fatigue. This study explored the relationships between gait characteristics derived from an inertial measurement unit (IMU) and patient-reported fatigue in the IDEA-FAST feasibility study.
METHODS
Participants with IMIDs and NDDs (Parkinson's disease (PD), Huntington's disease (HD), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), primary Sjogren's syndrome (PSS), and inflammatory bowel disease (IBD)) wore a lower-back IMU continuously for up to 10 days at home. Concurrently, participants completed PROs (physical fatigue (PF) and mental fatigue (MF)) up to four times a day. Macro (volume, variability, pattern, and acceleration vector magnitude) and micro (pace, rhythm, variability, asymmetry, and postural control) gait characteristics were extracted from the accelerometer data. The associations of these measures with the PROs were evaluated using a generalised linear mixed-effects model (GLMM) and binary classification with machine learning.
RESULTS
Data were recorded from 72 participants: PD = 13, HD = 9, RA = 12, SLE = 9, PSS = 14, IBD = 15. For the GLMM, the variability of the non-walking bouts length (in seconds) with PF returned the highest conditional R2, 0.165, and with MF the highest marginal R2, 0.0018. For the machine learning classifiers, the highest accuracy of the current analysis was returned by the micro gait characteristics with an intrasubject cross validation method and MF as 56.90% (precision = 43.9%, recall = 51.4%). Overall, the acceleration vector magnitude, bout length variation, postural control, and gait rhythm were the most interesting characteristics for future analysis.
CONCLUSIONS
Counterintuitively, the outcomes indicate that there is a weak relationship between typical gait measures and abnormal fatigue. However, factors such as the COVID-19 pandemic may have impacted gait behaviours. Therefore, further investigations with a larger cohort are required to fully understand the relationship between gait and abnormal fatigue.
Topics: Humans; Feasibility Studies; Male; Female; Middle Aged; Fatigue; Walking; Aged; Mental Fatigue; Neurodegenerative Diseases; Gait; Wearable Electronic Devices; Immune System Diseases; Adult; Accelerometry
PubMed: 38840208
DOI: 10.1186/s12984-024-01390-1