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Sjogren's syndrome (SS) is a multifaceted clinical condition characterized by various features, including ocular dryness (OD), dry mouth, arthralgia, and fatigue, which plays a substantial role in shaping the clinical presentation of the disease and has detrimental effects on quality of life. Recent research has acknowledged the advantages of the Mediterranean diet (MD) for its positive impact on various autoimmune diseases. This study aims to investigate the effect of Mediterranean diet on Sjogren disease using two validated scores: the Ocular Surface Disease Index (OSDI) score and the Eular Sjogren's Syndrome Patient Reported Index (ESSPRI). This was a cross-sectional observational study of previously diagnosed patients of Sjogren Syndrome using the ACR EULAR 2016 criteria. Patients were recruited from the archives of a tertiary university hospital. Data were collected through a telephone questionnaire, including demographic and disease data, the Ocular Surface Disease Index (OSDI) score to evaluate the OD severity, the Mediterranean Diet Adherence Screener (MEDAS) score to determine adherence to the MD and the Eular Sjogren's Syndrome Patient Reported Index (ESSPRI). The primary outcome of the study, the association between OSDI/ESSPRI and MEDAS scores, was evaluated using Spearman's correlation coefficient. The study comprised 75 patients with a mean age of 50.1 ± 13.3 years. There were 88% women among the patients. The median scores were 5.0 for the ESSPRI (IQR 3.0-6.7), 25.0 for the OSDI (IQR 10.4-40.6), and 8.0 for the MEDAS (IQR 4.5-11.0). The Spearman correlation analysis revealed a significant negative correlation between MEDAS and OSDI (ρ = -0.78, < 0.001). None of the covariates were statistically significant in the ESSPRI model, whereas treatment exhibited 2 a positive correlation with OSDI (β = +6.44, 95% CI 0.84 to 12.03; = 0.025), likely indicating confounding by indication. The study results suggest that higher adherence to a Mediterranean dietary pattern was consistently associated with lower symptom burden. This approach may serve as a complementary strategy with multiple health benefits, alongside conventional treatment options.

Sjögren's disease (SjD) is a chronic systemic autoimmune disease characterized by lymphocytic infiltration and progressive destruction of the lacrimal and salivary glands, leading to ocular and oral dryness as hallmark symptoms. Despite low systemic activity, these sicca symptoms significantly impair the quality of life, particularly in patients with severe dryness. Currently, no disease-modifying therapy is approved for SjD. Efgartigimod PH20, the neonatal Fc receptor (FcRn) blocker, has shown promising efficacy and safety in patients with moderate-to-severe systemic SjD. However, its efficacy for dryness in patients with SjD remains unknown. This is a phase 2, multicenter, randomized, double-blind, placebo-controlled, investigator-initiated trial conducted in Japan. Approximately 45 adult patients with SjD and moderate-to-severe dryness will be randomized in a 1:1:1 ratio to receive subcutaneous efgartigimod PH20 at 1,000 mg weekly (QW), 1,000 mg every other week (Q2W), or placebo for 24 weeks. The primary endpoint is the change in EULAR Sjögren's Syndrome Patient-Reported Index (ESSPRI)-dryness score from baseline to week 24. Key secondary endpoints include changes in ESSPRI-total, ESSPRI-fatigue, Diary of Sjögren's Symptoms Assessment (DiSSA) scores, and the proportion of responders in ESSPRI and Sjögren's Tool for Assessing Response (STAR) assessments. After the blinded period, all participants will be offered an open-label extension treatment to assess long-term safety and efficacy. This trial specifically targets patients with SjD who have prominent sicca symptoms. By using FcRn blockade to reduce pathogenic IgG autoantibodies, this study aims to explore the potential of efgartigimod PH20 as a novel therapeutic approach for dryness-predominant SjD. The findings are expected to provide future treatment strategies to address the major unmet need regarding dryness-related burden in this patient population. Japan Registry of Clinical Trials (jRCT2071250042); registered on 08 July 2025, https://jrct.mhlw.go.jp/en-latest-detail/jRCT2071250042.

The EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI), EULAR Sjogren’s Syndrome Patient Reported Index (ESSPRI), and other patient-reported outcomes (PROs), such as the visual analog scale (VAS) for symptoms and EULAR sicca score (ESS), are used to assess the disease activity of primary Sjögren’s syndrome (pSS). Recently, Clinical ESSDAI (ClinESSDAI) and Clinical Trials ESSDAI (ClinTrialsESSDAI) were developed for objective clinical disease activity indexes. However, the relationship of ClinESSDAI and ClinTrialsESSDAI with PROs as well as that between ESSPRI and other PROs and the objective parameters of glandular function in pSS have not been established. Herein, we investigated the correlation of ESSPRI and other PROs with the objective parameters of glandular function and the relationship of PROs with ClinESSDAI and ClinTrialsESSDAI in 66 patients with pSS. Correlations were calculated with Spearman’s correlation coefficient. ClinTrialsESSDAI was correlated with ESSPRI, dryness (ESSPRI-Dryness), fatigue, and pain domains of ESSPRI, VAS for oral dryness (oral-VAS), and patient’s global assessment. Although ESSPRI did not correlate with the objective parameters of glandular function, ESSPRI-Dryness, ESS, and oral- and ocular-VAS did. These results suggest that ESSPRI-Dryness, ESS, and VAS for symptoms, but not ESSPRI, reflect the glandular dysfunction and that ClinTrialsESSDAI correlates with PROs for dryness in pSS.

Background: Primary Sjögren’s syndrome (pSS) is an autoimmune disease that can cause fatigue and extraglandular manifestations (EGMs). pSS is associated with cytokine network dysregulation, which may be related to the immune-mediated destruction of exocrine glands. Objective: We determined cytokine levels and their relationship to EGMs, the European League Against Rheumatism (EULAR) Sjögren’s syndrome disease activity index (ESSDAI), and fatigue in Saudi patients with pSS. Methods: This study was a cross-sectional, single-center study. We included forty-one patients and 71 controls. Serum samples were collected from random healthy people and pSS patients who were followed in the rheumatology and pulmonary clinics of King Saud University Medical City in Riyadh, Saudi Arabia. Levels of the frequently studied cytokines were measured using Luminex xMAP technology. Each ESSDAI score and EGM were recorded, and the Arabic version of the fatigue severity scale (FSS) was applied to assess fatigue. The main outcome measures were cytokine levels in pSS Saudi patients using/not using immune-suppressive medications (ISMs). Results: Thirty-six (87.8%) patients had one or more EGMs, and the mean ESSDAI score was 9.95 ± 7.73. There was a significant decrease in TNFα and IL-21 levels in the pSS group compared to those in the control group (p = 0.034 and p < 0.001, respectively), whereas IL-12 levels were significantly elevated in the pSS group (p = 0.002). Cytokine levels in patients who used ISMs were the same as those in patients who did not use medications. Decreased IL-1β (p = 0.014), IL-2 (p = 0.035), IL-6 (p = 0.014), and IL-35 (p = 0.010) levels were observed in patients who had EGMs. Patients who had low disease activity exhibited low IL-10 (p = 0.018) and high IFN-α (p = 0.049), IFN-β (p = 0.049), IL-1β (p = 0.006), and IL-35 (p = 0.032) levels compared to patients with high disease activity. A negative association between a positive fatigue score and IL-1β (p = 0.010), IL-2 (p = 0.037), IFN-α (p = 0.025), TNFα (p = 0.030), IL-17 (p = 0.029), IL-12 (p = 0.046), and IL-21 (p = 0.005) levels was found. Conclusions: Cytokine profiles correlate with EGMs, ESSDAI, and fatigue. Patients with controlled disease activity have a normal cytokine profile that is similar to that of controls.

Xerostomia is a subjective measure of dry mouth, while hyposalivation is an objective measure of reduced saliva flow rate. In this study, we aim to assess the association between commonly used xerostomia scoring systems, with different hyposalivation measures among Sjogren Syndrome (SS) patients. In a cohort of SS patients, we assessed xerostomia using Xerostomia index, clinical oral dryness scale (CODS), and the European League Against Rheumatism SS Patient-Reported Index (ESSPRI), and we assessed hyposalivation using unstimulated whole saliva flow (UWS), stimulated whole saliva flow (SWS), and stimulated parotid flow (SPF). We analyzed the association between xerostomia and hyposalivation using association tests in SPSS. We included a total of 49 patients in this study, of which 34 (68%) had primary SS, and 15 (32%) had secondary. CODS was significantly correlated with SWS ( =0.048), with a negative correlation coefficient of 0.216, and with SPF ( =0.009), with a negative correlation coefficient of 0.291. The dryness domain of ESSPRI was significantly correlated with UWS ( =0.031) with a negative correlation coefficient of 0.233. CODS is the scoring system with the highest correlation with hyposalivation, particularly SWS and SPF, followed by ESSPRI dry domain, which is correlated with UWS. Xerostomia index is not correlated with hyposalivation.

Xerostomia and keratoconjunctivitis sicca are the main symptoms of Sjögren’s syndrome. Often patients also suffer from laryngeal complaints, but there is a lack of specific treatment options. The aim of this study was to evaluate the effect of a liposomal inhalation therapy. Patients with Sjögren’s syndrome were included and received a two-month period of liposomal inhalation therapy. The effect was evaluated by standardized questionnaires (patient-reported indices) and measurement of unstimulated whole salivary flow and glandular stiffness. Forty-five patients were included in this study. A comparison of baseline and therapeutic values demonstrated a significant improvement of the EULAR Sjögren’s syndrome patient reported index (ESSPRI) with a baseline of 5.0 ± 2.1 and a therapeutic value of 4.1 ± 2.4 (p = 0.012). This improvement was mainly based on the item on dryness within this score. Overall, the therapy was well tolerated. In conclusion, an inhalative application of liposomes had a beneficial effect on the reported dryness in patients with Sjögren’s syndrome. A first insight into the effect of inhalation therapy on laryngeal symptoms could thus be obtained and at the same time the basis was created on which case calculations can be carried out in the future.

IntroductionEuropean Alliance of Associations for Rheumatology (EULAR) Sjögren’s Syndrome Disease Activity Index (ESSDAI) is a clinician-reported outcome (ClinRO) instrument, assessing Sjögren’s disease activity from the physician perspective. EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) is a patient-reported outcome (PRO) instrument, assessing patient-defined Sjögren’s symptom severity. Both instruments are commonly used as clinical trial endpoints and have been psychometrically validated. However, qualitative evidence supporting content validity and what constitutes a meaningful change is limited. Qualitative evidence supporting Physician/Patient Global Assessment of disease activity and symptom severity (PhGA/PaGA) items used within anchor-based analyses for ESSDAI/ESSPRI is also lacking.MethodsQualitative, semi-structured, telephone/video interviews were conducted with patients with Sjögren’s (n = 12) and physicians who specialise in Sjögren’s (n = 10). Interviews explored: appropriateness of ESSDAI domain weights and meaningful improvements on domain/total scores from the physician perspective, appropriateness of ESSPRI’s 2-week recall period from the patient/physician perspective, patients’ perspectives on meaningful improvements in ESSPRI total scores, and patients’/physicians’ interpretation of PhGA/PaGA items.ResultsMost ESSDAI domain weights were considered clinically appropriate. Generally, a one-category improvement in domain-level scores and a 3-point improvement in total ESSDAI scores were considered clinically meaningful. Most patients/physicians considered ESSPRI’s 2-week recall period appropriate, and patients considered a 1-to-2-point ESSPRI total score improvement meaningful. PhGA/PaGA items developed for use as ESSDAI/ESSPRI anchors were consistently interpreted.ConclusionsThe findings support use of ESSDAI and ESSPRI as Sjögren’s clinical trials endpoints, as well as in clinical practice and other research settings. Qualitative data exploring meaningful change supports existing minimal clinically important improvement (MCII) thresholds.Plain Language SummaryEuropean Alliance of Associations for Rheumatology (EULAR) Sjögren’s Syndrome Disease Activity Index (ESSDAI) is an assessment used by physicians to measure how active Sjögren’s is in individuals with the condition. EULAR Sjögren’s Syndrome Patient Reported Index (ESSPRI) is a questionnaire completed by individuals with Sjögren’s to assess the severity of their symptoms. It is important to show that ESSDAI and ESSPRI are considered appropriate by physicians and individuals with Sjögren’s, respectively, and that ESSPRI is well understood by individuals with Sjögren’s completing the questionnaire. Therefore, interviews were conducted with physicians who specialise in Sjögren’s to explore the appropriateness of ESSDAI, the level of improvement on the assessment that would be important to individuals with Sjögren’s, and the appropriateness of the ESSPRI recall period (i.e. whether it is acceptable to ask individuals to remember their symptoms over the past 2 weeks). Interviews were also conducted with individuals with Sjögren’s to explore their understanding and relevance of ESSPRI (including the 2-week recall period) and the level of improvement on the questionnaire that would be important to them. Most physicians and patients considered ESSDAI and ESSPRI appropriate, supporting their use in a range of settings including Sjögren’s clinical trials, clinical practice and other research settings. Most physicians reported that a 3-point improvement in ESSDAI total score would be meaningful to individuals with Sjögren’s. Individuals with Sjögren’s reported that a 1-to-2-point improvement in ESSPRI total score would be meaningful.

Aims: The aim of the this study is to evaluate beta-2-microglobulin (B2M) levels in patients with primary Sjogren's syndrome (pSS) and to investigate their correlation with serum biomarkers and disease activity indexes commonly used in daily clinical practice. Methods: Eighty-one patients with pSS were included in this retrospective and cross-sectional study. Demographic data, clinical characteristics, B2M, immunoglobulin (Ig) A, IgG, IgM, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), serum complement (C) 3 and C4 levels, anti-nuclear antibody, rheumatoid factor (RF), anti-SSA and anti-SSB antibodies were obtained from the medical records. Disease activity was evaluated by the European League Against Rheumatism (EULAR) SS Disease Activity Index (ESSDAI) and EULAR SS Patients Reported Index (ESSPRI). Results: Serum B2M level was significantly higher in patients with anti-SSA and anti-SSB antibody (median: 2.64 mg/dL, minimum: 2.02 mg/dL, maximum: 10.10 mg/dL) than in only anti-SSA positive patients (median: 2.31 mg/dL, minimum: 1.33 mg/dL, maximum: 4.26 mg/ dL, p=0.010) and both antibody negative patients (median: 1.80 mg/dL, minimum: 1.20 mg/ dL, maximum: 2.65 mg/dL, p<0.001). Also, patients with anti-SSA antibody have significantly higher serum B2M levels than anti-SSA and anti-SSB antibodies negative patients (p=0.009). Serum B2M level was significantly correlated with ESSDAI (r=0.482, p=0.001), serum IgG level (r=0.374, p=0.001), IgA level (r=0.341, p=0.002), RF levels (r=0.412, p=0.021) and ESR (r=0.239, p:0.031). There was no correlation between ESSPRI, IgM, CRP and serum B2M levels. ESSDAI was not correlated with C3 (r=0.044, p=0.697), C4 (r=-0.053, p=0.640), ESR (r=0.111, p=0.326), CRP (r=0.111, p=0.324) and IgG (r=0.154, p=0.169). Conclusions: Although serum B2M levels were higher in autoantibody positive patients, it has a weak correlation with clinical disease activity. Keywords: Primary Sjogren's syndrome, beta-2-microglobulin, ESSDAI, ESSPRI, biomarkers

This study was set to investigate whether serum markers of lymphocytic activity are associated with patient-reported outcomes in Sjögren’s syndrome (SS). Forty-six patients with SS were included in this cross-sectional study. Patients with monoclonal gammopathy, history of malignant lymphoma, or with secondary SS were excluded. Serum levels of IgG, β2-microglobulin (β2M), soluble interleukin-2 receptor (sIL2-R), and free light chains (FLC) were assessed. Systemic disease activity was measured by the EULAR SS disease activity index (ESSDAI). Patient-reported symptoms were recorded by visual analogue scales (VAS) of pain, fatigue, and dryness, as compiled in the EULAR SS patient-reported index (ESSPRI). Depressive symptoms were determined by the Patient Health Questionnaire 9 (PHQ-9). Serum concentrations of κFLC (r = 0.491, p = 0.001), λFLC (r = 0.326, p = 0.027), and β2M (r = 0.421, p = 0.004) correlated with the ESSDAI, whereas sIL-2R and IgG did not. No correlations between serum markers of lymphocytic activity and the ESSPRI, or single VAS measures of pain, dryness, or fatigue, were found. In patients with VAS fatigue scores in the upper quartile, sIL-2R serum levels were even decreased (p = 0.019). Only depressive symptoms as determined by PHQ-9 were positively correlated with fatigue (r = 0.536, p < 0.001). In this well-defined cohort of patients with SS, serological lymphocytic activity was not correlated with patient-reported outcomes and sIL-2R levels were even decreased in patients with high fatigue scores. Only depressive symptoms were correlated with fatigue. This highlights the need to further understand the link between inflammation and disease characteristics in SS.

Background Primary Sjögren’s syndrome (pSS) is a systemic rheumatic disease in which gastrointestinal (GI) symptoms are common. Faecal calprotectin (FC) is a non-invasive biomarker that has been suggested to discriminate organic intestinal disease from functional disorders. The purpose of this study was to explore the usefulness of FC testing in patients with pSS. Methods In total, 56 consecutive patients with pSS and 29 healthy control subjects were included in this cross-sectional study. FC was measured with a commercially available enzyme-linked immunosorbent assay kit. GI symptoms were evaluated with the Rome III questionnaire and the Visual Analogue Scale for Irritable Bowel Syndrome. In patients with pSS, disease activity was estimated using the European League Against Rheumatism (EULAR) Sjögren’s Syndrome Disease Activity Index (ESSDAI), and patient-reported outcomes were evaluated with the EULAR Sjögren’s Syndrome Patient-Reported Index. Results Patients with pSS had higher levels of FC than healthy control subjects (median 54 μg/g, interquartile range [IQR 20–128]; vs. 20 μg/g [20–43]; p  = 0.002). Concomitant organic GI disease was found in 14 patients with pSS and included inflammatory bowel disease ( n  = 3), colonic adenoma ( n  = 2) and GI lymphoma ( n  = 1). Patients with organic GI disease had higher FC levels than the other patients with pSS (median 274 μg/g [IQR 61–363] vs. median 34 μg/g [IQR 20–76]; p  < 0.001). Although patients with pSS reported abdominal discomfort more frequently than healthy control subjects did, such symptoms were not associated with organic GI disease or elevated FC levels. FC correlated moderately with ESSDAI. Excluding patients with organic GI disease, we did not identify any significant association between ESSDAI and FC levels. Conclusions GI symptoms are frequent in pSS. Contrary to patient-reported outcomes, elevated FC levels in pSS indicate possible organic GI disease that warrants further investigation.