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This article discusses a case of tumefactive vasculitis in a 74-year-old woman with primary Sjögren syndrome (SS). The patient presented with a headache and experienced reduced consciousness and seizures. Brain magnetic resonance imaging (MRI) revealed an expansive lesion in the right frontal lobe, along with perivascular enhancement in the right basal ganglia. Histopathological analysis confirmed the presence of vasculitis. The patient was treated with immunoglobulin therapy, methylprednisolone, and rituximab, resulting in complete improvement of neurological symptoms. Tumefactive vasculitis should be considered as a differential diagnosis for expansive brain lesions in patients with primary SS. [Extracted from the article]

Anti‐neutrophil cytoplasmic antibody (ANCA) ‐positive vasculitis is a small‐vessel vasculitis that affects multiple body systems. Salivary gland involvement in ANCA‐associated vasculitis is rare. When present, it mimics infection or malignancy, which might lead to misdiagnosis. In this report, we describe a 72‐year‐old man who presented with parotid and submandibular gland pain and swelling in addition to dry mouth and eyes. He had bilateral non‐tender parotid gland lumps and no lymphadenopathies. Laboratory tests were positive for ANCA, hematuria, and proteinuria but negative for Anti‐Ro and ‐La. He was treated with corticosteroids and cyclophosphamide for acute kidney injury. Unfortunately, the patient died a few months later. This case report sheds light on a rare manifestation of salivary gland involvement in ANCA‐associated vasculitis that mimics Sjögren syndrome and the challenges associated with its diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Aim: Sjögren syndrome (SS) is a slowly progressive, inflammatory, autoimmune disease. The aim of this study was to construct the DNA methylation profiles of whole blood of SS patients and healthy controls (HC), and to explore the role of differentially methylated genes in the pathogenesis of the disease. Methods: Whole‐genome bisulfite sequencing was performed on three SS patients and four HC. The biological function of genes associated with differentially methylated regions (DMRs) was investigated using Gene Ontology functional analysis and Kyoto Encyclopedia of Genes and Genomes pathway analysis, using network‐based key driver analysis (KDA) to find KDA genes. In clinical samples of SS patients and controls, the expression levels of KDA genes were validated by quantitative real‐time polymerase chain reaction and immunohistochemical analysis. Moreover, the diagnostic value of KDA genes for SS was confirmed using receiver operating characteristic curves. Results: We identified 322 DMRs, annotated as 162 associated genes. Six genes were selected via the number of networks of KDA genes. Differential expression of genes such as human leukocyte antigen (HLA) class I, ADAR, and OAS2 was observed in patients' peripheral blood mononuclear cells and the minor salivary glands, which can be used as potential diagnostic biomarkers for SS. Conclusion: Clinical sample validation suggested that HLA class I, ADAR, and OAS2 might play a role in the development of SS. Our study shows epigenetic regulatory mechanisms and potential disease markers associated with SS, which in turn will enable us to identify new therapeutic targets. [ABSTRACT FROM AUTHOR]

Aim: To investigate the effects of pelvic floor training on pain, sexual dysfunction and quality of life in female patients with primary Sjögren syndrome (pSS) and determine whether pelvic floor training was superior to an education program. Methods: Forty‐six pSS patients (all women) with an average age of 47.78 ± 9.18 years were included in the study. Patients were divided into two groups as a pelvic floor training group and control group for 8 weeks. Visual Analog Scale (VAS), Pelvic Pain Impact Questionnaire (PPIQ), Female Sexual Function Scale (FSFI), Health Assessment Questionnaire (HAQ), Pelvic Floor Disability Index‐20 (PFDI‐20), and Pelvic Floor Impact Questionnaire (PFIQ‐7) were used to evaluate the outcomes. All evaluations were performed at baseline and at the end of the 8th week. Results: When the groups were compared before training, there was no significant difference (p >.05). In post‐training comparisons, there were significant differences in VAS, PPIQ, FSFI, PFIQ‐7, and PFDI‐20 in the pelvic floor training group (p values between.02 and.00), and in FSFI‐lubrication, PFIQ‐7, and PFDI‐20 (p values between.00 and.03) in the control group. According to Δ values, the pelvic floor training group was found to be superior in terms of FSFI orgasm, pain, and lubrication scores (p =.00) and all sub‐parameters of PFDI‐20 (p =.00). Conclusion: Pelvic floor training has a positive effect on the sexual dysfunction and discomfort caused by pelvic symptoms in patients with pSS. Pelvic floor training should be included in rehabilitation programs to improve sexual function and pelvic floor dysfunctions for patients with pSS. [ABSTRACT FROM AUTHOR]

This article discusses a rare case of a patient with Sjögren syndrome (SS) and psoriasis who was successfully treated with the Janus kinase inhibitor, tofacitinib. The patient presented with symptoms such as xerostomia, xerophthalmia, blurred vision, itching skin rash, scaly plaques, muscle weakness, and weight loss. Laboratory results showed abnormalities in potassium levels, CO2 combining power, blood urea nitrogen, creatinine, and inflammatory markers. Treatment with tofacitinib, along with other medications, resulted in significant improvement in symptoms and laboratory values without any adverse events. The article highlights the potential of targeted synthetic drugs like tofacitinib in the treatment of autoimmune diseases. [Extracted from the article]

Objective: To investigate cardiovascular risk among diabetic patients with Sjögren syndrome. Methods: This study was a nationwide population‐based case‐control study from 1997 to 2013, in which the association between autoimmune diseases and diabetes was investigated. The study population consisted of individuals with newly diagnosed type 2 diabetes with macrovascular or microvascular complications with at least two outpatient visits or one hospitalization as the outcome variables, and the exposure variables included traditional risk factors, medications, and autoimmune diseases. The odds ratio of cardiovascular events among each prevalent autoimmune disease and hydroxychloroquine's effect on cardiovascular risk were analyzed. Results: The study included a total of 7026 individuals with diabetes with microvascular and macrovascular complications and the same number of patients in the control group. Sjögren syndrome was significantly higher in the diabetes complication group than in the non‐complication group (0.8% vs 0.5%, P = 0.036). By using multivariate analysis, we found hypertension, hyperlipidemia, and Sjögren syndrome to be three independent risk factors for diabetes vascular complications (odds ratio [OR] 1.96, 95% confidence interval [CI] 1.82‐2.10; OR 1.53, 95% CI 1.42‐1.64; and OR 1.67, 95% CI 1.06‐2.65; respectively, all P < 0.05). Treatment with traditional statins and aspirin might be able to overcome the increased risk of developing cardiovascular events while comparing between diabetes patients with and without Sjögren syndrome. Conclusion: Sjögren syndrome is an unrecognized independent risk factor for cardiovascular events among diabetes patients, which indicates that patients with diabetes combined with Sjögren syndrome require closer follow up regarding cardiovascular complications in clinical settings. Treatment with hydroxychloroquine might not be enough to lower the cardiovascular risk significantly in diabetes patients with Sjögren syndrome. [ABSTRACT FROM AUTHOR]

Sjögren's syndrome (SS) is a common systemic autoimmune disease. SS usually occurs among middle‐aged women with a peak incidence age of approximately 50 years old. Kidney involvement is relatively uncommon in SS, which is mostly characterized as interstitial nephritis and may result in renal tubular acidosis (RTA). But premature onset of SS seems to be prone to RTA. Here we reported four cases of premature onset SS who developed into RTA at a relatively young age and three of whom suffered from severe osteomalacia. All of them shared a disease onset under age eighteen. Two of them presented hypokalemic periodic paralysis initially, one presented purpura and one endured xerophthalmia at first place. Three of them complicated with osteomalacia under age thirty. All the 4 cases didn't receive proper medical care in time due to a prolonged delay of diagnosis. We aim to raise the alarm over misdiagnosis/underdiagnosis of the disorder among young people. [ABSTRACT FROM AUTHOR]

Hypergammaglobulinemic purpura misdiagnosed as IgA vasculitis in a girl with primary Sjögren syndrome Keywords: hypergammaglobulinemic purpura; IgA vasculitis; Sjögren syndrome EN hypergammaglobulinemic purpura IgA vasculitis Sjögren syndrome 2091 2092 2 10/11/23 20231001 NES 231001 MYSTERY SECTION A 14-year-old girl had been suffering from reddish-brown skin rashes that appeared after prolonged walking or running for 6 months before presentation. Unlike adults, children with primary Sjögren syndrome rarely present glandular symptoms.[1] Hypergammaglobulinemic purpura (HP) is common in adults.[2] Some cases are misdiagnosed as (or complicated by) IgAV, a common disease in children. [Extracted from the article]

Background: Human α‐fucosidase (EC 3.2.1.51) is a hydrolase the importance of which has been increasing in the latest years. However, data about its plasma level in children with autoimmune disorders, particularly Sjögren's syndrome (SS), are lacking. In this study, the plasma activity of L‐α‐fucosidase‐1 (α‐L‐FUCA‐1) was assayed in hospitalized children and adults and its association with SS and other rheumatic disorders further evaluated. Methods: In total 73 Hungarian hospitalized patients, 32 children (2.5‐10 years) and 41 adults (32‐68 years), were enrolled in the study and underwent plasma assay of α‐L‐FUCA1 activity. Linear regression, Durbin‐Watson (DW), and Pearson tests were evaluated to investigate the relationship between α‐L‐FUCA‐1 plasma levels and autoimmune manifestations. Results: α‐L‐FUCA‐1 correlated with SS both in children (2‐sided t test, P = 0.0023) and in adults (2‐sided t test, P = 0.00035). Linear regressions showed that in other rheumatic disorders, α‐L‐FUCA1 did not show any differential distribution related to the particular pathology (r = 0.2042, P = 0.1531, DW test = 2.2139 positive), while this trend was radically opposite for patients with SS (r = 0.1462, P = 0.0032, DW test = 1.3664, negative). Conclusions: Alterations in plasma level of α‐L‐FUCA‐1 were significantly associated with SS. This preliminary result should encourage further research on α‐L‐FUCA‐1 as a possible differential serological marker of SS. [ABSTRACT FROM AUTHOR]

Abstract: Aims: Although rheumatic diseases constitute a leading cause of disability, the environmental risk factors for these diseases are not clarified. In the present study, we aim to systematically appraise the epidemiological credibility of the environmental risk factors for rheumatic diseases. Methods: We systematically searched PubMed to capture meta‐analyses of observational studies on environmental risk factors for the most prevalent rheumatic diseases. For each association, we estimated the summary effect size estimate, the 95% confidence and prediction intervals, and the I2 metric. We further examined the presence of small‐study effects and excess significance bias. Results: Overall, we identified 30 eligible papers describing 42 associations. Thirty‐three associations were statistically significant at P < 0.05, whereas 13 of them were statistically significant at P < 1 × 10−6. Thirty‐two associations had large or very large between‐study heterogeneity. In 12 associations, evidence of small‐study effects and/or excess significance bias was found. Six risk factors (nine associations) presented convincing or highly suggestive evidence of association: smoking and pack‐years of smoking for rheumatoid arthritis; BMI (per 5 kg/m2 increase) for gout and hip osteoarthritis; alcohol consumption for gout; BMI (overweight vs lean, obese vs lean), knee injury and participation in heavy work for knee osteoarthritis. Conclusion: Our umbrella review indicated that a narrow range of risk factors has been examined for rheumatic diseases. Current evidence strongly supports that smoking, obesity, alcohol consumption, knee injury, and work activities are associated with risk for at least one rheumatic disease. [ABSTRACT FROM AUTHOR]