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ID PMID Title PublicationDate abstract
32632715 Focus on Effects of Chinese Medicine on Improving Anxiety-Depression and Quality of Life o 2020 Jul Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease with a long duration of illness, for which there is no cure. Patients often suffer from anxiety and depression due to various reasons, exhibiting a decline in their quality of life. Chinese medicine (CM) has certain advantages in the treatment of pSS, which not only helps relieve clinical symptoms and improve treatment outcomes, but also reduces anxiety and depression and improves the quality of life. Therefore, CM should be considered as early as possible given its effectiveness and synergistic effects in treating pSS.
32533289 Prevalence and factors associated with osteoporosis and fragility fractures in patients wi 2020 Aug This study aimed at determining socio-demographic and clinical factors of primary Sjögren syndrome (pSS) associated with osteoporosis (OP) and fragility fracture. SJOGRENSER is a cross-sectional study of patients with pSS, classified according to American European consensus criteria developed in 33 Spanish rheumatology departments. Epidemiological, clinical, serological and treatment data were collected and a descriptive analysis was conducted. Bivariate and multivariate analyses were performed using a binomial logistic regression to study the factors associated with OP and fragility fracture in pSS. 437 patients were included (95% women, with a median age of 58.6 years). 300 women were menopausal (76.4%). Prevalence of OP was 18.5% [in men (N = 21) this measured 19%]. A total of 37 fragility fractures were recorded. In the multivariate analysis, there was an association between OP and age: in the 51-64 age range (menopausal women), the OR measured 9.993 (95% CI 2301-43,399, p = 0.002); In the age > 64 years group, OR was 20.610 (4.679-90.774, p < 0.001); between OP and disease duration, OR was 1.046 (1.008-1085, p = 0.017); past treatment with corticosteroids, OR 2.548 (1.271-5.105, p = 0.008). Similarly, an association was found between fragility fractures and age: in the 51-64 age group, OR measured 5.068 (1.117-22,995, p = 0.035), age > 64 years, OR was 7.674 (1.675-35,151, p < 0.009); disease duration, OR 1.049 (CI 1.003-1097, p < 0.036) and the ESSDAI index, OR 1.080 (1.029-1134, p = 0.002). Patients with pSS can develop osteoporosis and fragility fractures over the course of the disease. Age, corticosteroids treatment and disease duration were associated with the development of OP. Disease duration and ESSDAI were associated with the development of fractures in patients with pSS.
31237947 Hydroxychloroquine treatment downregulates systemic interferon activation in primary Sjög 2020 Jan 1 OBJECTIVE: HCQ is frequently used to treat primary SS (pSS), but evidence for its efficacy is limited. HCQ blocks IFN activation, which is present in half of the pSS patients. The effect of HCQ treatment on the expression of IFN-stimulated genes (ISGs) was studied in pSS. Furthermore, HCQ-treated patients were stratified based on IFN activation and differences in disease activity and clinical parameters were studied. METHODS: Expression of ISGs and IFN scores was determined in 77 patients, who were previously enrolled in the placebo-controlled JOQUER trial. Patients were treated for 24 weeks with 400 mg/d HCQ or placebo. RESULTS: HCQ treatment reduced IFN scores and expression of ISGs compared with the placebo-treated group. HCQ reduced ESR, IgG and IgM levels independently of the patients' IFN activation status. No differences in EULAR SS disease activity index or EULAR SS patient reported index scores were observed after HCQ treatment, even after IFN stratification. CONCLUSION: Treatment for 24 weeks with HCQ significantly reduced type I IFN scores and ISG-expression compared with the placebo-treated group. HCQ reduced several laboratory parameters, but failed to improve clinical response. This suggests that in pSS, type I IFN is associated to some laboratory parameters abnormalities, but not related to the clinical response.
31207153 Orofacial Motor Functions and Temporomandibular Disorders in Patients With Sjögren's Synd 2020 Aug OBJECTIVE: Sjögren's syndrome (SS) induces difficulty in chewing and swallowing due to low salivary flow. However, these symptoms may be associated with other factors, such as orofacial myofunctional disorders and temporomandibular disorder (TMD), which have not been comprehensively assessed in this population. The aims of this study were to investigate orofacial muscles and functions as well as the presence of TMD in patients with SS compared with a group without SS and to analyze whether the patients' experience of limitations in orofacial functioning is associated with the orofacial functional status and muscle pain related to TMD. METHODS: Women with SS based on the 2002 American-European Consensus Group criteria and volunteers paired by age and sex were compared. The examinations included the orofacial myofunctional evaluation with scores (OMES) protocol, tongue and lip strength measures, and electromyography of the masticatory muscles. TMD investigations included clinical examination, self-report of symptoms, and assessment according to the Jaw Functional Limitation Scale. RESULTS: Patients with SS present with impaired muscle and orofacial functions based on lower scores of all categories of OMES (P < 0.0001), tongue strength (P = 0.0003-0.0004), and masticatory muscle activity (P = 0.0002-0.007), as well as worse TMD signs and symptoms (P < 0.05) and jaw functional limitation (P < 0.0001-0.0003). CONCLUSION: Patients' experiences with limitation in mastication and swallowing were associated with orofacial myofunctional status and muscle pain related to TMD. Those disorders should be monitored along with disease control and must be addressed in the clinical evaluation to prevent nutritional and metabolic comorbidities in patients with SS.
33613559 Epstein Barr Virus and Autoimmune Responses in Systemic Lupus Erythematosus. 2020 Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease. Infections or infectious reactivation are potential triggers for initiation of autoimmunity and for SLE flares. Epstein-Barr virus (EBV) is gamma herpes virus that has been associated with several autoimmune diseases such as SLE, multiple sclerosis, Sjogren's syndrome, and systemic sclerosis. In this review, we will discuss the recent advances regarding how EBV may contribute to immune dysregulation, and how these mechanisms may relate to SLE disease progression.
31873754 Epidemiological profile and north-south gradient driving baseline systemic involvement of 2020 Sep 1 OBJECTIVE: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. METHODS: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. RESULTS: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). CONCLUSION: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.
31814226 Patient-related outcomes in Sjögren syndrome treated with stimulants of salivary secretio 2020 Mar OBJECTIVES: To investigate the impact of gustatory stimulants of salivary secretion (GSSS) on Sjögren's syndrome patients' self-perception of xerostomia, oral health-related quality of life (OHRQoL) and salivary secretion. METHODS: A total of 110 Sjögren's syndrome patients were randomly allocated to be treated with either a malic acid lozenge or a citric acid mouthwash and then crossed over. Before and after the interventions, the Xerostomia Inventory 5 (SXI-5-PL) and the Oral Health Impact Profile (OHIP-14-PT) questionnaires (both in the Portuguese language) were administered to patients. Unstimulated, mechanical and gustatory-stimulated salivary flows were determined. Repeated measures and between-subject analyses were performed. Statistical significance was set at 5%. RESULTS: After the intervention and within each group, both GSSS elicited a reduction in the SXI-5-PL and OHIP-14-PT scores and an increase in salivary output, significant in the malic acid lozenge group. The malic acid treatment resulted in a greater effect size and percentage improvement than citric acid mouthwash. The malic acid lozenge also produced a significant greater salivary output than the citric acid rising solution. CONCLUSIONS: In Sjögren's syndrome patients, lozenges containing malic acid increased saliva production and xerostomia relief, resulting in improved quality of life.
33133025 Intrinsic Restriction of TNF-Mediated Inflammatory Osteoclastogenesis and Bone Resorption. 2020 TNF (Tumor necrosis factor) is a pleiotropic cytokine that plays an important role in immunity and inflammatory bone destruction. Homeostatic osteoclastogenesis is effectively induced by RANKL (Receptor activator of nuclear factor kappa-B ligand). In contrast, TNF often acts on cell types other than osteoclasts, or synergically with RANKL to indirectly promote osteoclastogenesis and bone resorption. TNF and RANKL are members of the TNF superfamily. However, the direct osteoclastogenic capacity of TNF is much weaker than that of RANKL. Recent studies have uncovered key intrinsic mechanisms by which TNF acts on osteoclast precursors to restrain osteoclastogenesis, including the mechanisms mediated by RBP-J signaling, RBP-J and ITAM (Immunoreceptor tyrosine-based activation motif) crosstalk, RBP-J mediated regulatory network, NF-κB p100, IRF8, and Def6. Some of these mechanisms, such as RBP-J and its mediated regulatory network, uniquely and predominantly limit osteoclastogenesis mediated by TNF but not by RANKL. As a consequence, targeting RBP-J activities suppresses inflammatory bone destruction but does not significantly impact normal bone remodeling or inflammation. Hence, discovery of these intrinsic inhibitory mechanisms addresses why TNF has a weak osteoclastogenic potential, explains a significant difference between RANKL and TNF signaling, and provides potentially new or complementary therapeutic strategies to selectively treat inflammatory bone resorption, without undesirable effects on normal bone remodeling or immune response in disease settings.
33118847 Efficacy and safety of iguratimod on patients with primary Sjögren's syndrome: a randomiz 2021 Mar Objective: To evaluate the clinical efficacy and safety of iguratimod for the treatment of primary Sjögren's syndrome (pSS) and explore its possible mechanism of action.Method: We conducted a randomized, placebo-controlled clinical trial in 66 pSS patients. Patients were randomized in a 2:1 ratio to receive oral iguratimod for 24 weeks or matching placebo. The primary endpoint was the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI). Secondary endpoints included mental discomfort visual analogue scale (VAS) score, patient global assessment (PGA), EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI), Schirmer's test values, unstimulated whole salivary flow, erythrocyte sedimentation rate (ESR), and immunoglobulin G (IgG). The proportions of B cells in peripheral blood and levels of serum B-cell activating factor (BAFF) were measured at baseline and week 24 in the iguratimod group. All adverse events were recorded during the trial period.Results:ESSPRI improved more in the iguratimod than in the placebo group (p = 0.016). Mental discomfort VAS score, PGA, Schirmer's test, ESR, and IgG also improved more in the iguratimod than in the placebo group (all p < 0.05). Adverse events were reported 13.6% of the iguratimod group. Levels of BAFF and proportions of plasma cells in patients decreased significantly after iguratimod treatment. The proportions of peripheral plasma cells had positive correlations with both serum IgG and BAFF.Conclusion: Iguratimod improved some dryness symptoms and disease activity in pSS patients, and reduced the level of BAFF and percentage of plasma cells over 24 weeks. Iguratimod seems to be an effective and safe treatment for pSS.
32841507 Risk of malignancy in Korean patients with primary Sjögren's syndrome. 2020 Aug OBJECTIVES: To evaluate the risks of overall and site-specific malignancies in Korean patients with primary Sjögren's syndrome (pSS). METHODS: Using the Korean nationwide healthcare claims database, we constructed a retrospective cohort for prevalent pSS aged over 50 years. After enrollment between January 2012 and December 2014, patients were followed until the development of any malignancy, or until December 2015. Crude incidence rates of malignancies of pSS patients were calculated, and their standardized incidence ratios (SIRs) for malignancies were calculated compared to those in knee osteoarthritis (OA) patients. RESULTS: A total of 6,359 pSS and 5,476,302 knee OA patients were included in this study. During follow-up (19,474 person-years [PYs]), 310 cases of solid malignancy (158.8/10,000 PYs) and 47 cases of hematologic malignancies (23.5/10,000 PYs) were observed in pSS patients. The risks of overall (SIR 1.30, 95% CI 1.16-1.43), solid (SIR 1.16, 95% CI 1.03-1.29), and hematologic malignancies (SIR 4.80, 95% CI 3.43-6.17) were increased in pSS patients. There was an elevated risk of site-specific malignancy in non-Hodgkin's lymphoma (NHL, SIR 6.45, 95% CI 4.05-8.83), multiple myeloma (SIR 4.88, 95% CI 2.00-7.76), and oropharynx (SIR 4.16, 95% CI 1.90-6.42). The risk of lung cancer was increased only in male pSS patients (2.50, 95% CI 1.02-3.99), while the risk of thyroid cancer was increased in female patients (1.44, 95% CI 1.04, 1.84). CONCLUSION: In patients with pSS over age 50, the risk of solid cancers such as oropharynx, thyroid, and lung cancers is also increased in addition to NHL.
32062697 Serum immunoglobulin G4 in Sjögren's syndrome: a pilot study. 2020 Apr Immunoglobulin IgG4 plays a role in the pathogenesis of the Mikulicz disease previously considered a form of primary Sjögren's syndrome (pSS). We investigated serum levels of IgG4, total IgG, C3, and C4 serum complementary components in patients suspected of Sjögren's syndrome. Basic laboratory and immunological tests, including IgG4 and IgG concentration, were performed on 20 healthy and 68 suspected of pSS individuals. We distinguished: group I: 48 pSS patients; group II (sicca): 20 patients with dryness without pSS. We revealed: statistical differences between groups I and II concerning hypergammaglobulinemia, ESR, RF, ANA, Ro, and La antibodies; lower IgG4 levels and IgG4/IgG ratio in group I compared to healthy individuals (p < 0.0435; 0.0035, respectively); no significant differences in the concentrations of IgG4 and IgG4/IgG ratio between sicca and control groups. significantly lower (p < 0.0002) C4 levels in group I compared to other groups; significant differences in C4 concentration and IgG4/IgG ratio between three groups (p = 0.0002 and p = 0.0090, respectively); a weak negative correlation between C4 and IgG (r =- 0.274) in the whole database; weak positive correlation between C4 and IgG4/IgG ratio (r = 0.237); a negative correlation of IgG4, IgG4/Ig ratio and C4 with focus score (r = - 0.281; r = - 0.327; r = - 0.406, respectively). IgG4 serum levels were significantly decreased compared to healthy subjects. IgG4 and C4 levels correlated with infiltrations in minor salivary glands. Hypergammaglobulinemia and decreased serum C4 component levels are typical for pSS.
31925325 TGFβ1-Smad canonical and -Erk noncanonical pathways participate in interleukin-17-induced 2020 Jun Interleukin-17 (IL-17) is a pleiotropic cytokine that plays a primary role in triggering epithelial-mesenchymal transition (EMT) in many chronic inflammatory diseases. EMT plays a critical role in the progression of salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS). This study focused on the activation of the canonical TGF-β1/Smad2/3 and noncanonical TGF-β1/Erk1/2 pathways in IL-17-dependent TGFβ1-induced EMT in human SG epithelial cells (SGEC) derived from healthy subjects. The expression of phosphorylated Smad2/3 and Erk1/2 during IL-17 treatment-stimulated EMT was evaluated in healthy SGEC. Cotreatment with IL-17 and specific TGFβ receptor type I kinase inhibitor SB431542, or Erk 1/2 inhibitor U0126, abrogates the corresponding morphological changes and EMT phenotypic markers expression in healthy SGEC. Interestingly, inhibition of canonical TGFβ1/Smad2/3 signal transduction had no effect on activation of the noncanonical TGFβ1/Erk1/2/EMT pathway, suggesting that the two pathways act independently in activating IL-17-dependent EMT in SGEC.
31644053 Psoriasis Agents. 2012 Psoriasis is a chronic inflammatory skin disease that affects up to 2.5% of the United States population. Psoriasis varies greatly in severity, from an intermittent condition with a few localized patches of skin involvement, to a widespread serious skin disease with severe pruritus, extensive skin involvement, complications and disability. Psoriasis is associated with an inflammatory arthritis in at least 5% of cases. The typical psoriatic skin lesion is a raised, erythematous and sharply demarcated papule or plaque, often with a silvery crust. They are often pruritic. Histology shows acanthosis and inflammation with neutrophils and lymphocytes, which are rich in activated T cells. The etiology of psoriasis is not well defined, but it appears to be an autoimmune condition or a disease of immune dysregulation. The therapy of psoriasis ranges from topical ointments and oral therapies to intravenously or subcutaneously administered biologics. Milder cases can be managed by topical ointments, corticosteroids and vitamin D and retinoid derivatives. Systemic therapy is used for more severe disease or extensive skin involvement. Agents used include psoralen with ultraviolet light, methotrexate, acitretin, phosphodiesterase type 4 inhibitors (apremilast), cyclosporine or other immunomodulatory agents and, most recently, antitumor necrosis factor agents and monoclonal antibodies directed at activated T cells or their proinflammatory cytokines (secukinumab, ustekinumab). Psoriatic arthritis is typically treated similarly as rheumatoid arthritis. Most of the agents used to treat severe psoriasis have other major uses, such as in cancer chemotherapy (methotrexate), organ transplantation (cyclosporine), and autoimmune diseases (antitumor necrosis factor agents, secukinumab, ustekinumab). Antipsoriatic medications that have been linked to cases of hepatotoxicity include methotrexate, acitretin and the tumor necrosis factor antagonists.
32161274 Correlation between TM joint disease and rheumatic diseases detected on bone scintigraphy 2020 Mar 11 The aim of this study was to evaluate the effect of rheumatic disease as a risk factor for temporomandibular disease (TMD). A total of 143 outpatients reporting symptoms indicating rheumatic disease at their first visit to the rheumatology clinic were included. We evaluated the temporomandibular joint (TMJ) with scintigraphic images, and standard questionnaires were administered for the symptomatic assessment for all patients. The patients were classified into 'healthy controls' or as per their diagnosis into 'osteoarthritis', 'axial spondyloarthritis', 'peripheral spondyloarthritis', 'rheumatoid arthritis', or 'other rheumatic diseases' groups. The patients were also differentiated depending on the presence or absence of axial involvement. The relation between the rheumatic disease type and findings at the TMJ were evaluated using statistical analyses. Axial spondyloarthritis, peripheral spondyloarthritis, and rheumatic arthritis patients showed significantly higher scintigraphic uptake at the TMJ compared with those in the control and osteoarthritis groups (axial spondyloarthritis: 4.5, peripheral spondyloarthritis: 4.5, rheumatoid arthritis: 4.09, control: 3.5, osteoarthritis: 3.4, p < 0.0001). Compared with patients without axial involvement, patients with axial involvement also showed significantly higher TMJ scintigraphic uptake (axial involvement: 4.24, without axial involvement: 3.50, p < 0.0001) with elevated symptomatic rates in TMD (axial involvement: 17.82, without axial involvement: 9.97, p < 0.005).
33383289 Outcome of refractory to conventional and/or biologic treatment adult Still's disease foll 2021 Feb OBJECTIVE: To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. METHODS: In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150-300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3-84) months. RESULTS: Α complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. CONCLUSION: High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab.
33095148 Pathogenesis of primary Sjögren's syndrome beyond B lymphocytes. 2020 Jul Primary Sjögren's syndrome (pSS) is a chronic autoimmune disorder affecting exocrine glands of the body, prevalently lacrimal and salivary glands. The pSS pathogenesis has been thought to be B-cell-centric and several clinical trials have been carried out in order to clarify the therapeutic role of B-cell depletion in patients with pSS. Unfortunately, however, B-cell depletion with rituximab has failed in demonstrating any significant results in pSS patients. Besides the contribution of B cells in the pathogenesis of pSS, effector Tfh, Th17 and Th22 cells, follicular dendritic cells (DCs), innate cells (ICs) and several cytokines, chemokines and miRNA have been proved to participate to the development of this systemic disease. Understanding these molecular processes may help guide research into resistant diseases and highly targeted therapeutic strategies. This review aims to discuss important pathogenetic mechanisms involved in the initiation and perpetuation of pSS behind the established role of B cells.
33025892 The use of digital image analysis in the histological assessment of Sjögren's syndrome sa 2020 Jul OBJECTIVES: To assess whether the use of digital image analysis (DIA) in primary Sjögren's syndrome (pSS) for the calculation of the total area of the salivary gland (SG), focus score (FS) and SG area occupied by the inflammatory infiltrate (area fraction, AF), was able to generate reproducible readings among different raters, reducing disagreement. METHODS: Haematoxylin and Eosin digital slides from pSS and non-specific chronic sialadenitis (NSCS) patients were analysed blindly by 4 independent raters among 3 centres. Using an open-source software (QuPath) raters were asked to provide the total area of the gland i) using a grid-based method and ii) a software-based area-calculation tool, iii) the number of inflammatory foci and iv) the total area of the inflammatory infiltrate. Collected data was used to calculate the inter-rater agreement. RESULTS: For the calculation of the total SG area, DIA generated higher agreement among raters than grid-based calculation (inter-class correlation coefficient ICC=0.85 vs 0.98). Agreement for calculated total area of the inflammatory infiltrate (ICC=0.94) and for AF (ICC=0.94) was higher than infiltrates count number (ICC=0.54) and FS (ICC=0.56). AF achieved a 30% improvement over the FS at generating consensus among raters when used as a diagnostic cut-off. CONCLUSIONS: A digital approach achieved a far superior inter-rater agreement when calculating the total area compared to a grid-based approach. The calculation of AF proved superior to FS in correctly classifying pSS vs NSCS biopsies. We suggest that digitally calculated AF should be used alongside FS for large multi-centre studies to improve data harmonisation.
32445100 Epigenetics in Primary Sjögren's Syndrome. 2020 Primary Sjögren's syndrome (SjS) is a chronic and systemic autoimmune epithelitis with predominant female incidence, which is characterized by exocrine gland dysfunction. Incompletely understood, the etiology of SjS is multi-factorial and evidence is growing to consider that epigenetic factors are playing a crucial role in its development. Independent from DNA sequence mutations, epigenetics is described as inheritable and reversible processes that modify gene expression. Epigenetic modifications reported in minor salivary gland and lymphocytes from SjS patients are related to (i) an abnormal DNA methylation process inducing in turn defective control of normally repressed genes involving such matters as autoantigens, retrotransposons, and the X chromosome in women; (ii) altered nucleosome positioning associated with autoantibody production; and (iii) altered control of microRNA. Results from epigenome-wide association studies have further revealed the importance of the interferon pathway in disease progression, the calcium signaling pathway for controlling fluid secretions, and a cell-specific cross talk with risk factors associated with SjS. Importantly, epigenetic modifications are reversible thus opening opportunities for therapeutic procedures in this currently incurable disease.
32315513 Assessment of left atrial volume and function in patients with Sjögren's syndrome using t 2020 May OBJECTIVE: We used real time, three-dimensional transthoracic echocardiography (3DTTE) to evaluate left atrial (LA) volume and mechanical function in patients with primary Sjögren's syndrome (SS). METHODS: We prospectively included 42 consecutive patients with primary SS and 42 controls who were similar in terms of basal characteristics. 3DTTE was used to assess LA function. RESULTS: Maximum LA volume, minimum LA volume, pre-atrial contraction LA volume, LA Active Stroke Volume (ASV), LA Total Stroke Volume (TSV), maximal left atrial volume index (LAVImax), Left atrial pre-contraction volume index, and Left atrial minimum volume index, ASV index, and TSV index were significantly higher in the SS group, and the LA Total Emptying Fraction, LA Expansion Index, and LA Passive Emptying Fraction were significantly lower. Although the active emptying fraction was higher in the SS group, the difference was not statistically significant. LAVImax was positive correlated with disease duration (r = .753). CONCLUSION: Left atrial function is impaired in SS patients and serves as an early marker of subclinical cardiac involvement.
33229783 Acquired Bartter Syndrome in Primary Sjögren Syndrome. 2020 Sep Renal tubular involvement in Sjögren's syndrome (SS) often described with renal tubular acidosis, nephrogenic diabetes insipidus, or rarely with Fanconi syndrome. SS presenting with clinical features of Bartter's syndrome or Gitelman's syndrome is rare. We report a case of a female patient who presented an acquired Bartter syndrome with a primary SS.