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Objective: The present study compared the clinical features of patients with primary Sjögren's syndrome (pSS) with and without nephrolithiasis and/or nephrocalcinosis to determine factors related to renal dysfunction. Methods: The clinical features of 68 patients with anti-Sjogren's syndrome antigen A (SSA)/Ro-antibody–positive pSS with and without nephrolithiasis and/or nephrocalcinosis who underwent abdominal computed tomography and/or ultrasonography were retrospectively analysed. Results: Of the 68 patients with anti-SSA-antibody–positive pSS, 23 (33%) had renal nephrolithiasis and/or nephrocalcinosis, whereas 45 (67%) did not. Fourteen (20%) patients had renal dysfunction at diagnostic imaging. Among five patients who underwent renal biopsy, four patients with renal nephrolithiasis and/or nephrocalcinosis were diagnosed with tubulointerstitial nephritis, and one without nephrolithiasis and/or nephrocalcinosis was diagnosed with minimal change nephrotic syndrome. Estimated glomerular filtration rate at diagnostic imaging was significantly lower in patients with than without nephrolithiasis and/or nephrocalcinosis group (P = 0.010). In addition to nephrolithiasis and/or nephrocalcinosis (odds ratio [OR], 3.467; P = 0.045), the gap between serum sodium and chloride concentrations (OR, 10.400; P = 0.012) and increased urinary β2-microglobulin (OR, 5.444; P = 0.033) were associated with renal dysfunction at the time of diagnostic imaging. Conclusion: Nephrolithiasis and/or nephrocalcinosis, normal anion gap metabolic acidosis, and tubulointerstitial damage are associated with renal dysfunction in patients with pSS. [ABSTRACT FROM AUTHOR]

Objectives: The association of inflammation markers with hypertension (HTN) in primary Sjögren's syndrome (pSS) remains controversial. We aimed to investigate whether inflammation markers are at increased risk of developing HTN in pSS patients. Methods: A retrospective cohort study included pSS patients (n = 380) between May 2011 and May 2020 from the Third People's Hospital of Chengdu. Multivariable Cox regression analyses were used to estimate hazard ratios (HRs) of the potential inflammation markers for pSS-HTN. Subsequently, the dose–response relationships were also used. Results: Out of 380 pSS patients, 171 (45%) developed HTN, and the median follow-up period was 4.16 years. Univariable Cox regression analysis showed that the erythrocyte sedimentation rate (ESR) and neutrophils were significantly associated with the incident HTN (P < 0.05). After adjustment for covariates, this association between ESR (adjusted HR 1.017, 95%CI: 1.005–1.027, P = .003), neutrophils (adjusted HR 1.356, 95%CI: 1.113–1.653, P = .003), and HTN remained significant. The dose–effect relationship was also found between ESR, neutrophils, and HTN (P = .001). Conclusions: Inflammation markers may play an important role in the incident HTN in pSS. [ABSTRACT FROM AUTHOR]

Objective: To investigate whether primary Sjögren's syndrome (pSS) patients with hyperglobulinemia have an increased risk of all-cause mortality. Methods: Patients who registered in the Chinese Rheumatism Data Centre from May 2016 to July 2021 and met the 2002 American European Consensus Group criteria or 2016 American College of Rheumatology /European League Against Rheumatism classification criteria for Sjögren's syndrome were included. Hyperglobulinemia was defined as any elevated serum levels of immunoglobulin G (IgG), immunoglobulin A (IgA), or immunoglobulin M (IgM). The primary outcome was all-cause death. Data for demographic and clinical characteristics, laboratory results, disease activity, damage scores, and treatments were evaluated. Results: A total of 9527 pSS patients were included in the analysis, of whom 4236 (44.5%) had at least one kind of elevated immunoglobulin level among IgG, IgA, and IgM. Patients with hyperglobulinemia had a significantly increased risk of death (crude hazard ratio 2.60; 95% confidence interval 1.91–3.55; adjusted hazard ratio 1.90; 95% confidence interval 1.20–3.01). The risk of death was positively correlated with IgG level (P trend <.001). The 5-, 10-, and 15-year survival rates of patients with hyperglobulinemia were 96.9%, 92.3%, and 87.9%, respectively, and significantly lower than the corresponding rates of 98.8%, 97.9%, and 96.4% in patients without hyperglobulinemia. Conclusions: Hyperglobulinemia is an independent risk factor for increased all-cause mortality in pSS patients. The risk of death is positively correlated with IgG level. [ABSTRACT FROM AUTHOR]

Objective: To establish and validate a nomogram for individualized prediction of renal involvement in pSS patients. Methods: A total of 1293 patients with pSS from the First Affiliated Hospital of Wenzhou Medical University between January 2008 and January 2020 were recruited and analyzed retrospectively. The patients were randomly divided into development set (70%, n = 910) and validation set (30%, n = 383). The univariable and multivariate logistic regression were performed to analyze the risk factors of renal involvement in pSS. Based on the regression β coefficients derived from multivariate logistic analysis, an individualized nomogram prediction model was developed and subsequently evaluated by AUC and calibration plot. Results: Multivariate logistic analysis showed that hypertension, anemia, albumin, uric acid, anti-Ro52, hematuria, and ChisholmMason grade were independent risk factors of renal involvement in pSS. The AUC were 0.797 and 0.750, respectively, in development set and validation set. The calibration plot showed nomogram had a strong concordance performance between the prediction probability and the actual probability. Conclusion: The individualized nomogram for pSS patients those who had renal involvement could be used by clinicians to predict the risk of pSS patients developing into renal involvement and improve early screening and intervention. [ABSTRACT FROM AUTHOR]

Objectives: We aimed to assess the sleep quality of patients with primary Sjögren's syndrome (pSS) and the associated factors. Moreover, we conducted a preliminary exploration of the clinical significance of serum brain-derived neurotrophic factor (BDNF) in pSS patients with sleep disorders., Methods: A self-report survey was administered to 111 pSS patients and 40 healthy individuals using the Pittsburgh Sleep Quality Index for sleep quality. Clinical data, and mental conditions were collected using on-site questionnaires and various scales. A total of 40 healthy controls from the health examination centre of the same hospital, who were age- and sex-matched. Detection of serum BDNF levels by enzyme-linked immunosorbent assay method. Use GraphPad Prism to analyze data and create graphs., Results: Patients with pSS had higher scores on the Pittsburgh Sleep Quality Index than the healthy individuals. Abnormal sweating, high Patient Health Questionnaire-9 and EULAR Sjögren Syndrome Patient Reported Index scores were independent risk factors for sleep disorders. pSS patients had lower serum BDNF than the healthy individuals. The area under the curve of predicting sleep disorder in pSS patients using detection of serum BDNF level was 0.8470, and the sensitivity and specificity were 0.951 and 0.727, which were superior to Patient Health Questionnaire-9 and Generalized Anxiety Disorder-7., Conclusion: Compared with the healthy individuals, pSS patients had a higher prevalence of sleep disorders and lower serum BNDF. Serum BDNF level demonstrated greater predictive advantage for sleep disorder in pSS patients., (© Japan College of Rheumatology 2024. Published by Oxford University Press. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site–for further information please contact journals.permissions@oup.com.)

Objectives: To evaluate the efficacy and safety of iguratimod in patients with early primary Sjögren's syndrome (pSS). Methods: Twenty-seven disease-modifying antirheumatic drug-naive female patients met the revised American-European Consensus Group criteria for pSS were enrolled in this open-label pilot study. Patients were treated with iguratimod 25 mg twice a day for 24 weeks. The disease activity was assessed with European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) at 12 and 24 weeks. Salivary and lacrimal gland function, laboratory, and subjective variables were also assessed. Generalized estimating equations were used to analyze parameters over time. Results: ESSDAI (median, 5 versus 2 versus 2, p < .01), IgG (median, 26.6 versus 22.4 versus 21.4 g/L, p < .01) and rheumatoid factor (median, 119.9 versus 94.1 versus 83.8 lU/mL, p < .01) levels decreased significantly during iguratimod treatment. ESSPRI, salivary and lacrimal gland function, fatigue and health-related quality of life did not change during treatment. One patient experienced thrombocytopenia, and no other serious adverse effects were observed. Conclusion: In this study, iguratimod treatment is safe and effective for improving disease activity and laboratory parameters in early pSS patients. [ABSTRACT FROM AUTHOR]

Objective: We researched the findings of musculoskeletal ultrasound sonography (MSUS) on primary Sjogren's syndrome in childhood (pSS-C) with articular manifestations. The correlation of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody (ACPA) were investigated to evaluate the usefulness of MSUS on their articular prognosis. Method: The objective patients are pSS-C cases who visited our hospital complaining joint pain and/or joint swelling and for whom MSUS was performed. Result: Eight patients included 6 female and 2 male, 5 RF-positive patients and 3 ACPA-positive patients. The mean age of onset was 11.1 ± 3.0 years (352 physical joint findings and 284 MSUS findings. The number of joints found clinical articular manifestations was 58/352 joints, and arthritis detected by MSUS was 30/284 joints). In multivariate analysis, the odds ratio of clinical articular manifestations was significant high in RF-positivity (2.9, 95%CI 1.5-6.2). The odds ratio of arthritis detected by MSUS in ACPA-positivity was significant high (3.7, 95%CI 1.5-11.6), although odds ratio in RF-positivity had no statistical significance and a similar trend was seen in odds ratios of subclinical arthritis (4.9, 95%CI 1.6-18.0). Conclusion: It was indicated that MSUS is useful for pSS-C. ACPA-positive pSS-C patients have arthritis and subclinical arthritis more frequently than ACPA-negative patients. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study is to assess the impact of dryness caused by primary Sjögren's Syndrome (pSS) on smell, taste and sexual function in female patients, and its influence on quality of life. Methods: Electronic databases including MEDLINE via Ovid, Web of Science, SCOUPUS, EMBASE and COCHRANE LIBRARY were searched until April 2016. Studies that assessed the function of smell, taste and sexuality in pSS patients, defined by the American European Consensus Group (AECG) criteria. Standardized mean differences (SMD) for individual studies using randomeffects meta-analysis were feasible. Results: Five studies incorporated 378 participants were included in the quantitative synthesis. The impact of pSS vs. healthy controls was: smell SMD -0.78 (95% CI -1.29 to -0.27); taste SMD -1.01 (95% CI -1.54 to -0.49); total sexual function SMD -0.93 (95% CI -1.22 to -0.64); physical and mental component of the quality of life SMD -1.28 (95% CI -1.65 to -0.90) and SMD -0.83 (95% CI -1.27 to -0.40) respectively; anxiety and depression SMD 0.61 (95% CI 0.02, 1.20) and SMD 0.79 (95% CI 0.43 to 1.15), respectively. Conclusion: pSS has a negative impact on smell, taste, sexual function and quality of life in women. [ABSTRACT FROM AUTHOR]

Objectives: Anticentromere antibody (ACA) is generally considered to be a serological marker for systemic sclerosis (SSc). ACA-positive patients with primary Sjögren's syndrome (pSS) have also been reported. ACA often recognizes centromere proteins (CENPs): CENP-A, CENP-B, and CENP-C, and sometimes reacts to heterochromatin protein 1 (HP1)a. We compared the reactivity against six different epitopes for three ACA-positive clinical subgroups: 29 patients with pSS, 36 SSc patients with sicca symptoms, and 28 SSc patients without sicca symptoms. Methods: We utilized enzyme-linked immunosorbent assays (ELISAs) with recombinant proteins covering six different epitope regions of ACA (the amino terminus (Nt) of CENP-A, CENP-B, and CENP-C, the carboxyl terminus (Ct) of CENP-B and CENP-C, and HP1a). Results: The patients with pSS were found to have IgG-class autoantibodies against CENP-C-Nt and HP1a, and IgA-class autoantibodies against CENP-C-Ct with significantly higher frequencies than the SSc patients with or without sicca symptoms. The positive predictive value and the negative predictive value of the combination of these three autoantibodies for pSS were 73% and 82%, respectively, for pSS. Conclusions: Based on the result that reactivities against CENP-C and HP1a in patients with pSS differ from those in patients with SSc, we propose ACA-positive pSS as a clinical subset of SS that is independent of SSc. [ABSTRACT FROM AUTHOR]

Objectives. To estimate the diagnostic accuracy of anti-alpha-fodrin antibodies for primary Sjögren's syndrome (pSS). Methods. Sixty-four pSS subjects and 108 non-pSS patients were prospectively enrolled in this study. Serum anti-alpha-fodrin IgA and IgG were detected by ELISA in a blind fashion. The diagnostic accuracy of anti-alpha-fodrin antibodies was assessed by receiver operating characteristic (ROC) curve analysis. Logistic regression was used to investigate whether anti-alpha-fodrin antibodies could improve the accuracy of pSS diagnosis if used in addition to anti-SSA and anti-SSB. Results. The areas under the ROC curves for anti-alpha-fodrin IgG and IgA were 0.69 (95% confidence interval (CI): 0.60-0.77) and 0.63 (95% CI: 0.54-0.72), respectively ( P < 0.01 for both). The optimal diagnostic thresholds for anti-fodrin IgG and IgA were 11.75 U/ml and 9.75 U/ml, respectively, with a sensitivity of 0.59 and 0.55, and a specificity of 0.75 and 0.73, respectively. Anti-alpha-fodrin IgG and IgA antibodies were associated with pSS after adjustment for anti-SSA and anti-SSB. Conclusions. Anti-alpha-fodrin IgG and IgA antibodies are useful diagnostic markers which may improve the accuracy of pSS diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of our study was to define the clinical characteristics of anti-centromere antibody and anti-SS-A/Ro antibody (ACA/SS-A) double positive Sjögren's syndrome (SS) and to clarify the clinical impact of these antibodies., Methods: We examined 108 patients (6 males, mean age 57.9 years) with SS who underwent labial salivary gland biopsy. The patients were divided into four groups by ACA and anti-SS-A/Ro antibody positivity. Symptoms, laboratory and pathological data, and scleroderma-related data were compared among the groups., Results: The cohort consisted of 16 ACA/SS-A double positive, 20 ACA single positive, 67 SS-A single positive, and 5 ACA/SS-A double negative SS. ACA/SS-A double positive SS were significantly older than SS-A single positive SS (mean age 71.1 vs. 53.1 years). They had higher EULAR Sjögren's syndrome disease activity index (ESSDAI) at diagnosis (mean 3.81 vs. 0.50) and higher serum IgG (mean 2009 vs. 1389 mg/dL) than ACA single positive SS. No patients developed skin sclerosis during a mean follow-up period of 45.6 months (range: 1-178)., Conclusion: These results demonstrate that ACA/SS-A double positive SS is distinct from ACA single positive and SSA single positive SS. The combination of ACA and anti-SS-A/Ro antibody in SS should deserve greater attention in clinical practice.