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Clinical and Experimental Rheumatology Dec 2023The link between immune cell function and cell metabolic reprogramming is currently known under the term "immunometabolism". Similarly to the Warburg's effect described... (Review)
Review
The link between immune cell function and cell metabolic reprogramming is currently known under the term "immunometabolism". Similarly to the Warburg's effect described in cancer cells, in activated immune cells an up-regulation of specific metabolic pathways has been described and seems to be pathogenic in different inflammatory conditions.Sjӧgren's syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands and is characterised by a progressive loss of secretory function. Despite the increasing amount of evidence on the ability of metabolism in regulating cell behaviour in inflammatory or tumoral conditions, the field of metabolism in SS is still for the most part unexplored.The aim of this review is to summarise currently available studies evaluating cell metabolism in SS with a particular focus on the possible pathogenic role of metabolic changes in immune and non-immune cells in this condition.
Topics: Humans; Sjogren's Syndrome
PubMed: 38149514
DOI: 10.55563/clinexprheumatol/hhbqej -
Clinical and Experimental Rheumatology Dec 2023Primary Sjögren's syndrome (pSS) is a chronic, systemic, inflammatory autoimmune disease characterised by lymphocyte proliferation and progressive damage to exocrine... (Review)
Review
Primary Sjögren's syndrome (pSS) is a chronic, systemic, inflammatory autoimmune disease characterised by lymphocyte proliferation and progressive damage to exocrine glands. Salivary gland histopathology based on salivary gland biopsy is relevant for the diagnosis of pSS and therefore broadly applied in clinical practice. Tissue can be obtained from labial salivary glands (LSG) biopsy or from major salivary glands (MSG) biopsy, namely the parotid; in this latter scenario, the procedure can be either an open surgical biopsy or a US guided core needle biopsy.In this review we will: i) present the histopathological findings that may be encountered by pathologists on biopsies from pSS patients; ii) discuss the advantages and disadvantages of the surgical and/or imaging guided procedures to obtain tissues from LSG or MSG; iii) describe the histopathological features of lymphoma of MSG in pSS patients.
Topics: Humans; Sjogren's Syndrome; Salivary Glands; Parotid Gland; Salivary Glands, Minor; Lymphoma; Biopsy
PubMed: 38079343
DOI: 10.55563/clinexprheumatol/fcjoqx -
Heliyon Nov 2023Patients with primary Sjögren's syndrome (pSS) take a higher risk of developing lymphoma, which is the most frequent cause of death in pSS. Based on this situation, the... (Review)
Review
BACKGROUND
Patients with primary Sjögren's syndrome (pSS) take a higher risk of developing lymphoma, which is the most frequent cause of death in pSS. Based on this situation, the number of publications focusing on pSS-associated lymphoma has been growing. Nevertheless, the extent, range, and nature of available research in this field have not been systematically summarized. This study aimed to map the literature available on pSS-associated lymphoma and identify global hotspots and trends.
METHODS
Papers on pSS and lymphoma published from 1991 to 2022 were searched from the Web of Science Core Collection. Microsoft Excel, SPSS Statistics, VOSviewer, and CiteSpace software were used to analyze and visualize the quantity and citations of publications, and the global research hotspots and trends of pSS-associated lymphoma.
RESULTS
629 publications from 50 countries/regions and 538 institutions were included in this study. From 1991 to 2022, the cumulative publications steadily increased. The USA ranked first in the number of publications (n = 118, 18.76 %), followed by Italy (n = 94, 14.94 %) and France (n = 73, 11.61 %). Udine University (n = 29) and Salvatore De Vita (n = 39) were the most prolific affiliation and author, respectively. Claudio Vitali was the most frequently cited author (n = 335). In total, the most frequently occurring keywords were clustered into four well-defined groups. The first group of keywords pointed to the clinical assessment and treatment of pSS-associated lymphoma. The second group highlighted the pathogenesis. The third group identified the predictors and prognosis of pSS-associated Lymphoma, while the fourth group focused on interstitial lung disease and pulmonary lymphoma in patients with pSS. Currently, the hot keywords include consensus, disease activity, and pathogenesis. Ultrasonography, mucosa-associated lymphoid tissue (MALT) lymphoma, and epidemiology are the emerging research trends in pSS-associated lymphoma.
CONCLUSION
Research on pSS-associated lymphoma is burgeoning. Despite clinical assessment, treatment and pathogenesis, researchers also showed great interest in the predictors, prognosis, and pulmonary manifestations of pSS-associated lymphoma. Current research of pSS-associated lymphoma mainly focuses on consensus, disease activity, and pathogenesis, while the emerging research trends in pSS are pointing to ultrasonography, MALT lymphoma and epidemiology.
PubMed: 37964859
DOI: 10.1016/j.heliyon.2023.e21337 -
Medicine Aug 2023A clear bidirectional relationship exists between malignant cancers including breast cancer and different autoimmune diseases. However, none of the studies have assessed...
A clear bidirectional relationship exists between malignant cancers including breast cancer and different autoimmune diseases. However, none of the studies have assessed whether this association is causal and none have used Mendelian randomization for determining this relationship. This study therefore investigated the causal relationship between autoimmune diseases and breast cancer using the Mendelian randomization approach. Summary statistical data were obtained from genome-wide association studies to investigate the causal relationship between different autoimmune diseases including Graves' disease, Sjögren's syndrome, Crohn disease, systemic sclerosis, and psoriasis and breast cancer risk. The results revealed no strong evidence to support the causal relationship between Graves disease (odds ratio [OR] = 0.9958; 95% confidence interval [CI]: 0.9982-1.0035), Sjögren's syndrome (OR = 1.0018; 95% CI: 0.9950-1.0087), Crohn disease (OR = 2.1195, 95% CI: 0.1872-23.9978), systemic sclerosis (OR = 1.0024; 95% CI: 0.997-1.0079), and psoriasis (OR = 1.0016; 95% CI: 0.9913-1.0121) to breast cancer risk. A similar result was obtained in the reverse Mendelian randomization analysis. Our study provides evidence that autoimmune diseases might not have a causal relationship with breast cancer risk in the European population and concludes that shared genetic effects or environmental confounders.
Topics: Humans; Female; Breast Neoplasms; Sjogren's Syndrome; Crohn Disease; Genome-Wide Association Study; Mendelian Randomization Analysis; Autoimmune Diseases; Graves Disease; Scleroderma, Systemic; Psoriasis
PubMed: 37653788
DOI: 10.1097/MD.0000000000034612 -
Scientific Reports Nov 2023We compared the efficacy and safety of autologous-serum (AS) and platelet-rich plasma (PRP) eye drops for dry eye (DE) treatment in primary Sjögren's syndrome (SS).... (Randomized Controlled Trial)
Randomized Controlled Trial
We compared the efficacy and safety of autologous-serum (AS) and platelet-rich plasma (PRP) eye drops for dry eye (DE) treatment in primary Sjögren's syndrome (SS). This prospective, randomized, double-blinded clinical study included patients diagnosed with primary SS DE. Thirty-eight participants were randomly assigned to the AS or PRP groups. Corneal and conjunctival staining scores, Schirmer I test, tear film break-up time (TBUT), and ocular surface disease index (OSDI) scores were evaluated at 4 and 12 weeks. Conjunctival impression cytology (CIC) metaplasia grade and goblet cell density grade at 12 weeks were compared with those at baseline. Corneal and conjunctival staining scores and TBUT significantly improved at 4 and 12 weeks in both groups (all p < 0.005). No significant difference between the AS and PRP groups was observed at 4 and 12 weeks. The Schirmer I values, OSDI scores, CIC metaplasia grade, and goblet cell density grade did not significantly change at 4 and 12 weeks in either group. Both AS and PRP eye drops are effective for primary SS DE without a significant difference. Considering that the preparation time of PRP is shorter than that of AS, PRP can be a good alternative treatment for primary SS DE.
Topics: Humans; Dry Eye Syndromes; Metaplasia; Ophthalmic Solutions; Platelet-Rich Plasma; Prospective Studies; Sjogren's Syndrome; Tears
PubMed: 37935760
DOI: 10.1038/s41598-023-46671-2 -
Journal of Clinical Rheumatology :... Aug 2023The aim of this study was to study clinical and biological differences between men and women with primary Sjögren syndrome (pSS) in China and perform a literature... (Review)
Review
OBJECTIVES
The aim of this study was to study clinical and biological differences between men and women with primary Sjögren syndrome (pSS) in China and perform a literature review to confirm if the clinical phenotypes are affected by sex in patients with pSS.
METHODS
Data from 961 patients with pSS treated at a tertiary hospital in China between January 2013 and March 2022 were analyzed based on medical records. Clinical characteristics, including disease manifestations and serological parameters of the disease, were compared between men and women with pSS using the Mann-Whitney U test and χ 2 test.
RESULTS
This study included 140 (14.6%) men and 821 (85.4%) women with pSS. Women with pSS demonstrated a higher prevalence of dry mouth, dry eyes, arthralgia, and dental caries ( p < 0.05); higher erythrocyte sedimentation rate and immunoglobulin M levels ( p < 0.05); higher prevalence of leukopenia, neutropenia, anemia, low complement 3, and low complement 4 ( p < 0.05); and higher titers of antinuclear antibody, anti-Sjögren syndrome A, anti-Ro52, and rheumatoid factor positivity ( p < 0.05) than men, whereas men with pSS had a higher prevalence of parotid enlargement and interstitial lung disease ( p < 0.05).
CONCLUSIONS
Women with pSS are associated with more dryness, cytopenia, hypocomplementemia, and autoantibody positivity. Although men with pSS probably have lighter sicca symptoms and lower immunoactivity and serologic responses, regular monitoring of interstitial lung disease in men is vital.
Topics: Humans; Male; Female; Sex Characteristics; Dental Caries; Sjogren's Syndrome; Lung Diseases, Interstitial; Medical Records
PubMed: 37068269
DOI: 10.1097/RHU.0000000000001962 -
Mediterranean Journal of Rheumatology Sep 2023Dehydroepiandrosterone (DHEA) is an adrenal hormone used to treat rheumatic conditions such as systemic lupus erythematosus (SLE), Sjogren's syndrome (SS), rheumatoid...
BACKGROUND
Dehydroepiandrosterone (DHEA) is an adrenal hormone used to treat rheumatic conditions such as systemic lupus erythematosus (SLE), Sjogren's syndrome (SS), rheumatoid arthritis (RA) with controversial results.
AIM
To review the results of DHEA use in rheumatic diseases.
METHODS
PubMed, Scielo, Scopus, and Embase databases were systematically searched for articles on the treatment of rheumatic diseases with DHEA between 1966 and April 2023.
RESULTS
Twenty-one studies were identified: 13 in SLE, 5 in SS, 2 in RA, and 1 in fibromyalgia. DHEA use in SLE has shown a mild to moderate effect on disease activity, a positive effect on bone mineral density (BMD), and improved fatigue. The studies on SS showed a decrease in symptoms of dry mouth, but its performance did not differ from placebo in disease activity. In RA, a questionable effect on disease activity was noted. The only study on fibromyalgia failed to show any improvement. The drug was well tolerated; mild androgenic effects were the most common complaints.
CONCLUSION
DHEA seems to have a place in SLE treatment, where it improves BMD and disease activity. The use in RA, SS, and FM is questionable.
PubMed: 37941864
DOI: 10.31138/mjr.20230825.dd -
International Journal of Molecular... May 2024Sjögren's disease (SjD) is a heterogeneous autoimmune disease characterized by severe dryness of mucosal surfaces, particularly the mouth and eyes; fatigue; and chronic... (Review)
Review
Sjögren's disease (SjD) is a heterogeneous autoimmune disease characterized by severe dryness of mucosal surfaces, particularly the mouth and eyes; fatigue; and chronic pain. Chronic inflammation of the salivary and lacrimal glands, auto-antibody formation, and extra-glandular manifestations occur in subsets of patients with SjD. An aberrant expression of long, non-coding RNAs (lncRNAs) has been described in many autoimmune diseases, including SjD. Here, we review the current literature on lncRNAs in SjD and their role in regulating X chromosome inactivation, immune modulatory functions, and their potential as biomarkers.
Topics: Humans; Sjogren's Syndrome; RNA, Long Noncoding; Biomarkers; Animals; X Chromosome Inactivation; Gene Expression Regulation
PubMed: 38791207
DOI: 10.3390/ijms25105162 -
Frontiers in Immunology 2023Primary Sjögren's syndrome (pSS) is a progressive inflammatory autoimmune disease. Immune cell infiltration into glandular lobules and ducts and glandular destruction...
BACKGROUND
Primary Sjögren's syndrome (pSS) is a progressive inflammatory autoimmune disease. Immune cell infiltration into glandular lobules and ducts and glandular destruction are the pathophysiological hallmarks of pSS. Macrophages are one of the most important cells involved in the induction and regulation of an inflammatory microenvironment. Although studies have reported that an abnormal tissue microenvironment alters the metabolic reprogramming and polarisation status of macrophages, the mechanisms driving macrophage infiltration and polarisation in pSS remain unclear.
METHODS
Immune cell subsets were characterised using the single-cell RNA sequencing (scRNA-seq) data of peripheral blood mononuclear cells (PBMCs) from patients with pSS (n = 5) and healthy individuals (n = 5) in a public dataset. To evaluate macrophage infiltration and polarisation in target tissues, labial salivary gland biopsy tissues were subjected to histological staining and bulk RNA-seq (pSS samples, n = 24; non-pSS samples, n = 12). RNA-seq data were analysed for the construction of macrophage co-expression modules, enrichment of biological processes and deconvolution-based screening of immune cell types.
RESULTS
Detailed mapping of PBMCs using scRNA-seq revealed five major immune cell subsets in pSS, namely, T cells, B cells, natural killer (NK) cells, dendritic cells (DCs) and monocyte-macrophages. The monocyte-macrophage subset was large and had strong inflammatory gene signatures. This subset was found to play an important role in the generation of reactive oxygen species and communicate with other innate and adaptive immune cells. Histological staining revealed that the number of tissue-resident macrophages was high in damaged glandular tissues, with the cells persistently surrounding the tissues. Analysis of RNA-seq data using multiple algorithms demonstrated that the high abundance of pro-inflammatory M1 macrophages was accompanied by the high abundance of other infiltrating immune cells, senescence-associated secretory phenotype and evident metabolic reprogramming.
CONCLUSION
Macrophages are among the most abundant innate immune cells in PBMCs and glandular tissues in patients with pSS. A bidirectional relationship exists between macrophage polarisation and the inflammatory microenvironment, which may serve as a therapeutic target for pSS.
Topics: Humans; Salivary Glands; Sjogren's Syndrome; Transcriptome; Leukocytes, Mononuclear; Macrophages
PubMed: 38022546
DOI: 10.3389/fimmu.2023.1292146 -
BMC Oral Health Mar 2024Observational studies indicated a controversial relationship between periodontitis (PD) and Sjogren's syndrome (SS). To overcome restrictions in conventional...
OBJECTIVES
Observational studies indicated a controversial relationship between periodontitis (PD) and Sjogren's syndrome (SS). To overcome restrictions in conventional observational studies, we conducted a two-sample Mendelian randomization (MR) analysis to assess the potential bidirectional relationship between PD and SS.
METHODS
We utilized the largest available genome-wide association study (GWAS) of European ancestry on both PD (17,353 cases-28,210 controls) and SS (2495 cases-365,533 controls) for MR genetic instrument selection. The random-effect inverse-variance weighted (IVW) method complemented by Causal Analysis Using Summary Effect (CAUSE), weighted median, weighted mode, simple mode, MR-Egger regression, and MR-pleiotropy residual sum and outlier (MR-PRESSO) was used for MR analysis. Subsequent pleiotropy and heterogeneity tests were conducted.
RESULTS
IVW analysis exhibited neither an effect of PD on SS (OR = 0.939, 95%CI = 0.525-1.677, P = 0.8304) nor that of SS on PD (OR = 1.007, 95%CI = 0.977-1.038, P = 0.6440). The other five complementary methods further recognized the null association with an effect size close to one. No significant pleiotropy was detected in the relationship between PD and SS (P > 0.05). Heterogeneity existed in the effect of PD on SS but not vice versa.
CONCLUSIONS
No genetic causality between PD and SS or vice versa was supported by our results under MR assumptions and limitations. The study results provided new insights into the relationship between periodontal status and sjogren's syndrome, highlighting the need for a more prudent medical intervention.
Topics: Humans; Sjogren's Syndrome; Genome-Wide Association Study; Mendelian Randomization Analysis; Periodontitis
PubMed: 38528503
DOI: 10.1186/s12903-024-04151-7