2,918 results on '"sjogren syndrome"'
Search Results
2. Efficacy of Cevimeline on Xerostomia in Sjögren's Syndrome Patients: A Systematic Review and Meta-Analysis of Randomized Clinical Trials
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Karimi, Mehdi, Hajikolaei, Fatemeh Ahmadi, Hoseinpour, Fahime, Hashemi, Seyed-Ali, Fatehi, Anita, Pakmehr, Seyed-Abbas, Deravi, Niloofar, Naziri, Mahdyieh, Belbasi, Mohaddeseh, Khoshravesh, Sahar, and Vaezzadeh, Seyed Hossein
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- 2025
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3. Sjögren syndrome (Sjögren disease)
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Dumusc, Alexandre and Bowman, Simon J
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- 2024
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4. Association between oral microbiome dysbiosis and Sjogren Syndrome. A systematic review of clinical studies
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Nagi, Ravleen, Kumar, Sanjana Santhosh, Sheth, Megha, Deshpande, Ashwini, and Khan, Junad
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- 2025
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5. Characteristics and treatment responses of immune thrombocytopenia in patients with primary Sjögren syndrome
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Sun, Feng, Tang, Qi, Cheng, Wei, Xie, Xi, Li, Fen, and Chen, Jiali
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- 2023
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6. Chapter 5 - Dry Eye
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- 2025
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7. Genetic insight into dissecting the immunophenotypes and inflammatory profiles in the pathogenesis of Sjogren syndrome.
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Xu, Jingyi, Si, Shucheng, Han, Yijun, Zeng, Lin, and Zhao, Jinxia
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SJOGREN'S syndrome , *MENDELIAN randomization , *MEDICAL sciences , *GENOME-wide association studies , *B cells - Abstract
Background: Sjogren syndrome (SS) is a chronic systemic autoimmune disease and its pathogenesis often involves the participation of numerous immune cells and inflammatory factors. Despite increased researches and studies recently focusing on this area, it remains to be fully elucidated. We decide to incorporate genetic insight into investigation of the causal link between various immune cells, inflammatory factors and pathogenesis of Sjogren syndrome (SS). Methods: Our study leveraged the genetic variants of multi-omics statistics extracted from genome-wide association study (GWAS), the University of Bristol and the FinnGen study. We performed a bidirectional Mendelian randomization and mediation study based on randomly allocated instrumental variables to infer causality, followed by external validation with UK Biobank data and Bayesian colocalization. Results: We demonstrated that an elevated level of CD27 on IgD + CD24 + B cell, a subset of B cells expressing both IgD and CD24, was associated with a higher risk of SS (OR = 1.119, 95% CI: 1.061–1.179, P < 0.001), while CD3 on CD45RA + CD4 + Treg was a protective factor (OR = 0.917, 95%CI: 0.877–0.959, P < 0.001). Results of meta-analysis and colocalization further supported the significant results identified in the primary analysis. A total of 4 inflammatory cytokines and 7 circulating proteins exhibited potential causal relationships with SS despite no significant result achieved after FDR correction. Finally, results of mediation analysis indicated that CD40L receptor levels had significant mediating effects (β = 0.0314, 95% CI: 0.0004–0.0624, P = 0.0471) at a mediation proportion of 28% (95% CI: 0.364%-55.6%) in causal relationship between CD27 on IgD + CD24 + B cell and SS. Conclusions: By providing a novel genetic insight into unveiling the roles of autoimmunity and inflammation in Sjogren syndrome, our findings may potentially lead to identifying new clinical biomarkers for disease monitoring and therapeutic targets that offer more effective alternatives for treating this condition. Therefore, our study may provide valuable evidence for future clinical intervention and targeted immunotherapy. [ABSTRACT FROM AUTHOR]
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- 2025
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8. The analysis of the pulmonary domain involvement in Sjögren's disease.
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Madej, Marta, Proc, Krzysztof, Wawryka, Piotr, Morgiel, Ewa, Sebastian, Maciej, Wiland, Piotr, and Sebastian, Agata
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SJOGREN'S syndrome ,CHRONIC cough ,INTERSTITIAL lung diseases ,SMOKING ,RHEUMATOID factor ,ADOLESCENT smoking - Abstract
Background: The EULAR Sjögren's syndrome (SS) disease activity index (ESSDAI) pulmonary domain is used to assess the activity of respiratory system involvement in Sjögren's disease (SjD). The most unfavorable form of respiratory involvement in SjD, after lymphomas, is interstitial lung disease (ILD). Objectives: The aim of the study was to assess the involvement of the respiratory system in SjD patients and the occurrence of ILD in high-resolution computed tomography (HRCT), depending on immunological markers, the influence of cigarette smoking, and the age of the patients. Design: Single-center, registry, cohort study. Methods: Among all SjD patients, a group with involvement in the pulmonary domain was distinguished. This group was later subjected to a detailed analysis of immunological and serological markers and chest imaging tests. Results: In all, 64 patients out of 299 with SjD had involvement in the pulmonary domain defined according to the ESSDAI definition. The most frequently reported clinical symptoms of respiratory system involvement included dryness and chronic cough (over 80% of patients), followed by shortness of breath. Nine percent of patients with changes in lungs were asymptomatic. Patients with pulmonary involvement were older (54 vs 48 years, p < 0.05). In the subpopulation of patients with SjD and pulmonary involvement, the presence of rheumatoid factor (73% vs 60%, p < 0.05), and hematological domain involvement according to ESSDAI (54% vs 37%, p < 0.05) were more common. In the group of 64 patients with a positive pulmonary domain, 34 (53%) had ILD on HRCT. A higher incidence of comorbidities was found in the population of patients with ILD. No correlation was found between the type of lung involvement and the immunological profile, inflammatory markers, age, and smoking habit. Conclusion: Involvement of the pulmonary domain is common in patients with SjD. However, the clinical picture is very heterogeneous, which determines the subsequent personalization of treatment. [ABSTRACT FROM AUTHOR]
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- 2024
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9. Labial salivary gland biopsy: a crucial method for confirming seronegative Sjogren's syndrome—a case report.
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Romala, Satyasai S. G., Moturi, Kishore, Manyam, Ravikanth, Vadupu, Meghana, Lakshmi, P. Divya Naga, Budumuru, Anil, Shivajiraju, U., Hyma, J. S. D. N. Sai, and Ramachandrudu, R. V. S. N. S.
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SJOGREN'S syndrome , *MEDICAL specialties & specialists , *MEDICAL sciences , *EXOCRINE glands , *SWEAT glands , *SALIVARY glands - Abstract
Background: Sjogren's syndrome is an autoimmune disease that affects exocrine glands like sweat glands, salivary glands, and lacrimal glands. Dentists frequently encounter these patients and have an important role in establishing the diagnosis. It is mainly diagnosed by clinical features like xerostomia and xerophthalmia, along with anti-Sjögren's syndrome-related antigen A (anti-SSA/also called Ro antigen) and anti-Sjögren's syndrome-related antigen B (anti-SSB/ also called La antigen) antibodies in the patients. However, there is a subset of the population where autonuclear antibodies cannot be detected but are still present with all clinical features of Sjogren's syndrome. Such patients usually remain undiagnosed, and only symptomatic treatment is given in spite of repeated consultation with various medical specialists. Only after establishing a proper diagnosis can we initiate definitive treatment. Case presentation: Here, we report a case of seronegative Sjogren's syndrome in a patient with severe xerostomia without anti-SSA and anti-SSB antibodies. The focus score from the labial biopsy, co-relating with clinical presentation, helped us in establishing the diagnosis. After a definitive diagnosis, our patient is put on cholinergic therapy, which greatly reduced the symptoms of xerostomia. Conclusions: Physicians and dentists should refer such patients to an oral and maxillofacial surgeon for a labial biopsy to consider "seronegative Sjogren's syndrome." The "focus score" evaluation by the pathologist helps in establishing the diagnosis of "seronegative Sjogren's syndrome". [ABSTRACT FROM AUTHOR]
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- 2024
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10. The Relationship Between Systemic Immune Inflammatory Level and Dry Eye in Patients with Sjögren's Syndrome.
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Uzeli, Ülkem Şen, Doğan, Ayşe Gülşen, and Şahin, Tayfun
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SJOGREN'S syndrome , *DRY eye syndromes , *DISEASE progression , *INFLAMMATION , *WOMEN patients - Abstract
Background and objectives: Sjögren Syndrome (SS) is a chronic, systemic, and progressive autoimmune disease in which inflammatory processes play a role. Dry eyes or mouth are present in approximately 95–98% of patients with pSS. This study aimed to evaluate the relationship between SII level and disease activity as well as dry eye involvement in patients with pSS. Materials and methods: A cross-sectional design was employed, and a total of 28 female patients who were aged 18–65 years and were diagnosed with pSS were involved. The Sjögren Syndrome Disease Activity Index (ESSDAI) was calculated in patients. The Schirmer test was applied to all patients. The relationship between SII level and disease activity as well as dry eye involvement in pSS patients was evaluated. Results: In our study, a strong positive correlation was found between the SII value and pSS disease activity, while a negative correlation was found between the Schirmer test, which shows dry eye findings, and eye drying time, and a positive correlation was found with the OSDI. Conclusions: this study reported a correlation between hematological parameters and the development of dry eye in pSS. NLR, PLR, and SII showed statistically significant changes in pSS patients. [ABSTRACT FROM AUTHOR]
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- 2024
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11. Rare connective tissue diseases in patients with C1-inhibitor deficiency hereditary angioedema: first evidence on prevalence and distribution from a large Italian cohort study.
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Triggianese, P., Senter, R., Perego, F., Gidaro, A., Petraroli, A., Arcoleo, F., Brussino, L., Giardino, F., Rossi, O., Bignardi, D., Quattrocchi, P., Brancaccio, R., Marcelli, A. Cesoni, Accardo, P. A., Lo Sardo, L., Cataudella, E., Guarino, M. D., Firinu, D., Bergamini, A., and Spadaro, G.
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SJOGREN'S syndrome ,CONNECTIVE tissue diseases ,SYSTEMIC lupus erythematosus ,SYSTEMIC scleroderma ,HUMORAL immunity ,ANTIPHOSPHOLIPID syndrome - Abstract
Introduction: In patients with Hereditary Angioedema (HAE) related to primary C1 inhibitor deficiency (C1INH), the defective clearance of immune complexes and apoptotic materials along with impairment of normal humoral response potentially leads to autoimmunity. Few studies report evidence on autoimmune diseases in C1INH-HAE, but no large population studies focus on rare connective tissue diseases (RCTDs). We aim at evaluating for the first time prevalence and distribution of RCTDs - Systemic Lupus Erytematosus (SLE), primary Sjogren Syndrome (SjS), primary antiphospholipid syndrome (APS), Systemic Sclerosis (SSc), and mixed connective tissue diseases (MCTD) in a large Italian cohort of C1INH-HAE patients. Methods: A multicenter observational study includes C1INH-HAE patients from ITACA Centers throughout Italy (time frame Sept 2023-March 2024). Inclusion criteria are i. a defined diagnosis of type I or type II C1INH-HAE; ii. age =15 years (puberty already occurred); iii. enrollment in the ITACA Registry. The diagnosis of SLE, primary SjS, primary APS, SSc, and MCTD are made in accordance with international classification criteria. Results: Data are collected from a total of 855 C1INH-HAE patients referring to 15 ITACA Centers. Patients with concomitant RCTDs were 18/855 (2.1%) with F:M ratio 3.5 and a prevalent type I C1INH-HAE diagnosis (87.2%). A diagnosis of SLE results in 44.5% of cases (n=8) while the remaining diagnoses are primary SjS (22.2%, n=4), primary APS (16.6%, n=3), SSc (11.2%, n=2), and a single case of MCTD (5.5%). The female gender is prevalent in all the RCTDs. Patients on long term prophylaxis (LTP) are significantly prevalent in RCTDs group than in the whole C1INH-HAE population (p<0.01). Conclusions: A relevant prevalence of RCTDs is documented in C1INH-HAE patients, mainly SLE. Patients with RCTDs are on LTP in a significant proportion supporting the idea of a bidirectional link between C1INH-HAE and autoimmunity. [ABSTRACT FROM AUTHOR]
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- 2024
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12. Endoplasmic reticulum stress in the salivary glands of patients with primary Sjögren’s syndrome, associated Sjögren’s syndrome, and non-Sjögren’s sicca syndrome: a comparative analysis and the influence of chloroquine
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Graziela Vieira Cavalcanti, Fabiola Reis de Oliveira, Rafael Ferraz Bannitz, Natalia Aparecida de Paula, Ana Carolina Fragoso Motta, Eduardo Melani Rocha, John Chiorini, Hilton Marcos Alves Ricz, Denny Marcos Garcia, Maria Cristina Foss-Freitas, and Luiz Carlos Conti de Freitas
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Sjögren syndrome ,Gene expression ,Cross-sectional studies ,Endoplasmic reticulum stress ,Unfolded protein response ,Diseases of the musculoskeletal system ,RC925-935 ,Immunologic diseases. Allergy ,RC581-607 - Abstract
Abstract Background Endoplasmic reticulum stress (ERS) and the unfolded protein response (UPR) are adaptive mechanisms for conditions of high protein demand, marked by an accumulation of misfolded proteins in the endoplasmic reticulum (ER). Rheumatic autoimmune diseases (RAD) are known to be associated with chronic inflammation and an ERS state. However, the activation of UPR signaling pathways is not completely understood in Sjögren’s disease (SD). This study evaluated the expression of ERS-related genes in glandular tissue of patients with primary SD (pSD), associated SD (aSD) with other autoimmune diseases, and non-Sjögren sicca syndrome (NSS). Methods In a cross-sectional study, minor salivary gland biopsies were obtained from 44 patients with suspected SD and 13 healthy controls (HC). Patients were classified as pSD, aSD, or NSS based on clinical, serological, and histological assessment. Histopathological analysis and mRNA expression analysis of genes associated with ERS and UPR (PERK, XBP1, ATF-6, ATF-4, CANX, CALR, CHOP, and BIP) were performed on the samples. Differences between groups (pSD, aSD, NSS, and HC) were assessed. The influence of chloroquine (CQ) on the ER was also investigated. Results Twenty-eight SD patients showed increased expression of PERK (p = 0.0117) and XBP1 (p = 0.0346), and reduced expression of ATF-6 (p = 0.0003) and CHOP (p = 0.0003), compared to the HC group. Increased expression of BIP (p
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- 2025
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13. A Review of a Breakdown in the Barrier: Tight Junction Dysfunction in Dental Diseases
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Do TT, Nguyen VT, Nguyen NTN, Duong KTT, Nguyen TTM, Le DNT, and Nguyen TH
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epithelial barrier ,gingival inflammation ,sjogren syndrome ,oral squamous cell carcinoma ,dental diseases ,Dentistry ,RK1-715 - Abstract
Thao Thi Do,1,* Vy Thuy Nguyen,1,* Ngoc Tran Nhu Nguyen,1 Kim Tran Thien Duong,1 Tri Ta Minh Nguyen,1 Duong Nguyen Thuy Le,1 Tin Hoang Nguyen2 1Oral Diagnosis and Periodontology Department, Faculty of Odonto-Stomatology, Can Tho University of Medicine and Pharmacy, Can Tho City, 900000, Vietnam; 2Department of Physiology, Faculty of Medicine, Can Tho University of Medicine and Pharmacy, Can Tho City, 900000, Vietnam*These authors contributed equally to this workCorrespondence: Tin Hoang Nguyen, Department of Physiology, Faculty of Medicine, Can Tho University of Medicine and Pharmacy, 179 Nguyen Van Cu Street, An Khanh Ward, Ninh Kieu District, Can Tho City, 900000, Vietnam, Tel +84344895522, Email nhtin@ctump.edu.vnAbstract: The tight junction (TJ), a type of cell-cell junction, regulates the permeability of solutes across epithelial and endothelial cellular sheets and is believed to maintain cell polarity. However, recent studies have provided conflicting views on the roles of TJs in epithelial polarity. Membrane proteins, including occludin, claudin, and the junction adhesion molecule, have been identified as TJ components. TJs are predominantly found at the stratum granulosum and stratum corneum. Although it remains unclear whether the disruption of TJs is the cause or consequence of certain dental diseases, evidence suggests that TJ dysfunction may be a crucial factor in gingival epithelial barrier impairment and the progression of oral diseases. Bacterial infection is among the most specific factors we found that may contribute to the breakdown of the epithelial barrier formed by TJs in dental diseases. Bacteria and their products may weaken the epithelial barrier by directly destroying intercellular junctions or altering the expression of junctional proteins. Additionally, they may induce the production of inflammatory cytokines, which could lead to the downregulation of TJ proteins and, consequently, impair the epithelial barrier. This review introduces a novel perspective by exploring, for the first time, the role of TJs dysfunction in the breakdown of the oral epithelial barrier and its potential link to the progression of dental diseases such as gingivitis, periodontitis, Sjӧgren syndrome, and oral squamous cell carcinoma.Keywords: epithelial barrier, gingival inflammation, Sjӧgren syndrome, oral squamous cell carcinoma, periodontitis
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- 2024
14. Sjögren syndrome from a dermatological perspective: A retrospective study of 185 SSA‐Ro positive patients
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Fiona André, Martha Bujak, Nina Frischhut, Andreas Kronbichler, Norbert Sepp, Gudrun Ratzinger, and Barbara Böckle
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internal organ involvement ,Sjögren syndrome ,skin manifestations ,Dermatology ,RL1-803 ,Diseases of the genitourinary system. Urology ,RC870-923 - Abstract
Abstract Background Sjögren syndrome (SS) is a systemic autoimmune disease characterised by oral and ocular mucosal dryness. Objectives Our study focused on skin manifestations of SS. The differences between primary SS (pSS) and secondary SS (sSS) were investigated. Furthermore, we elucidated factors predicting internal organ involvement. Methods This was a single centre retrospective cohort study. We analysed clinical and laboratory data from 185 Ro‐SSA positive SS patients between January 2000 and December 2016. Results Skin manifestations were present in 74.9% (n = 137) of SS patients. Dermatitis and Lupus erythematosus (LE)‐specific skin manifestations were present in 34.1% (n = 63) and 21.6% (n = 40) of SS patients respectively. 65.9% (n = 122) of SS patients had internal organ involvement, with 53% (n = 98) presenting neuropsychiatric manifestations. Several risk factors for internal organ involvement were identified: presence of myalgia/arthralgia (odds ratio [OR] = 3.05, confidence interval [CI] 1.29−7.22), fatigue (OR = 2.53, CI 1.11−5.73) and age older than 50 years (OR = 2.23, CI 1.01−4.93). Conclusions Internal organ involvement is frequent in SS, however there is no significant difference between pSS and sSS concerning its occurrence.
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- 2024
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15. Parotid gland MALT lymphoma with amyloid deposition, challenges in preoperative diagnosis: A case report
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Yuriko Watanabe, MD, Hiroyuki Fujii, MD, PhD, Saki Yamamoto, MD, Sota Masuoka, MD, Ryoma Kobayashi, MD, Nana Fujii, MD, Akihiro Nakamata, MD, Takeharu Kanazawa, MD, PhD, Mitsuru Matsuki, MD, PhD, and Harushi Mori, MD, PhD
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MALT lymphoma ,Amyloidosis ,MRI ,Sjögren syndrome ,Parotid gland ,Medical physics. Medical radiology. Nuclear medicine ,R895-920 - Abstract
Mucosa-associated lymphoid tissue (MALT) lymphoma commonly arises from chronic inflammation or autoimmune diseases, such as Sjögren syndrome (SjS). Although rare, amyloid deposition in MALT lymphoma has been reported. We present a rare case of parotid gland MALT lymphoma in a 49-year-old woman, in whom preoperative diagnosis was challenging due to atypical imaging findings resulting from amyloid deposits. MRI showed T2-hypointense and T1-iso- to slightly hyperintense masses in the left parotid gland and right sublingual gland, with predominant marginal contrast enhancement and no significant diffusion restriction. Additionally, atrophy and fatty replacement of the parenchyma were noted in bilateral parotid glands, suggesting SjS. Left superficial parotidectomy was performed and pathological findings confirmed MALT lymphoma with extensive amyloid deposition. Histopathological findings of the resected parotid gland parenchyma also suggested SjS. MALT lymphoma should be considered in the differential diagnosis of multiple salivary gland masses in patients with suspected SjS. If MRI reveals atypical imaging findings for malignant lymphoma, particularly T2-hypointensity with no significant diffusion restriction, the possibility of amyloid deposition in MALT lymphoma should be considered.
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- 2024
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16. USP18-mediated protein stabilization of NOTCH1 is associated with altered Th17/Treg cell ratios and B cell-mediated autoantibody secretion in Sjögren syndrome.
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Jin, Xiaorong, Bai, Yunjing, Xu, Xiaohua, Wu, Fan, Long, Xiaoyu, and Yao, Yajuan
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Sjögren Syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of exocrine glands. This study, based on bioinformatics predictions, investigates the biological functions of ubiquitin specific peptidase 18 (USP18) and notch receptor 1 (NOTCH1) in T helper 17 (Th17) and regulatory T (Treg) cell imbalance and B cell activity in SS. USP18 and NOTCH1 were highly expressed in peripheral blood mononuclear cells (PBMCs) of SS patients and the PBMCs of NOD mice compared to the controls. Adenovirus-mediated knockdown of USP18 significantly enhanced the salivary flow rate of NOD mice while reducing lymphocyte infiltration in mouse salivary ligand tissues. In addition, it decreased the proportions of Th17 cells while increasing the proportions of Treg cells. USP18 enhanced NOTCH1 protein stability through de-ubiquitination modification. In the presence of USP18 knockdown, the NOTCH1 upregulation restored the predominance of Th17 cells in mice. In B cells isolated from PBMCs, the production of B cell autoantibodies was decreased by USP18 silencing but enhanced by NOTCH1 upregulation. In summary, this study demonstrates that USP18-mediated protein stabilization of NOTCH1 is correlated with Th17/Treg cell imbalance and B cell activity during SS development. [ABSTRACT FROM AUTHOR]
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- 2025
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17. Adipose derived or bone-marrow derived mesenchymal stem cell treatment for hyposalivation: protocol for a systematic review and network meta-analysis
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Joachim Hansen, Amanda-Louise Fenger Carlander, Kathrine Kronberg Jakobsen, Christian Grønhøj, and Christian von Buchwald
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Mesenchymal stem cell ,Adipose derived ,Bone marrow derived ,Xerostomia ,Radiotherapy ,Sjogren syndrome ,Medicine - Abstract
Abstract Background Salivary hypofunction leads to debilitating oral symptoms and has major complications for overall quality of life. Two of the most frequent causes of xerostomia are radiotherapy in the head and neck and Sjögren’s syndrome. Only symptomatic treatment is available today. An increasing number of both preclinical and clinical studies have suggested that mesenchymal stem cell (MSC) transplantation treatment can increase the salivary flow rate and ameliorate symptoms of xerostomia. However, both adipose-derived and bone marrow–derived MSCs are used, although they differ in important ways. The primary objective of this study is an indirect comparison of the change in the unstimulated salivary flow rate after intervention between patients treated with adipose-derived or bone marrow–derived MSCs. Methods This systematic review and network meta-analysis will search for eligible studies in the MEDLINE, EMBASE, and Cochrane CENTRAL register of Controlled Trials. Eligible studies are as follows: clinical studies including human patients with salivary hypofunction due to either radiotherapy or Sjogren’s syndrome who were subsequently treated with either adipose-derived MSCs or bone marrow–derived MSCs. Studies with no control group will be excluded. The search phrase has been peer-reviewed following the PRESS guidelines. The primary outcome is the change in the unstimulated salivary flow rate after treatment with either adipose-derived or bone marrow–derived MSCs. Secondary outcomes are as follows: change in patient reported outcomes, methods of intervention administration, number of injected MSCs, and safety. Data from included studies will be pooled and compared with a fixed-effects or random effects model dependent on signs of heterogeneity, presented with a forest plot, and indirectly compared with a meta-regression in a network meta-analysis. Risk of bias will be assessed with the tools ROBINS-I or RoB-2 depending on type of study. Discussion Both adipose-derived and bone marrow–derived MSCs are used today for experimental treatment of salivary hypofunction in humans as no direct or indirect comparisons have been made. Therefore, an evaluation of the effect of adipose-derived vs bone marrow–derived MSC treatment is needed to support future decision-making on the type of MSC used in a clinical trial. Systematic review registration PROSPERO ID CRD42024527183.
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- 2024
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18. 25-Hydroxyvitamin-D levels in Sjögren’s syndrome: is it the right time to dismiss the case or not?
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Karabulut Gonca, Yilmaz Zevcet, Yildirim Simsir Ilgin, Barutcuoglu Burcu, and Tanigor Goksel
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25 oh-d3 levels ,disease activity ,sjögren syndrome ,vitamin d ,Biochemistry ,QD415-436 - Abstract
This study aimed to investigate whether patients with primary Sjögren syndrome (SjS) have different levels of 25 OH-D3 (vitamin D) when compared to healthy populations and whether differences in 25 OH-D3 correlated with disease activity or markers.
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- 2024
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19. A case report on Sjögren Syndrome: it‘s more than just autoimmune epithelitis
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Dhanush Balaji S., Abinaya Srinivasa Rangan, M. M. Kavitha, S. Prasanna Karthik, Karpaka Vinayakam Gopalakrishnan, Selva Balaji, and Raghunathan E.G.
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pah ,sjögren syndrome ,epithelitis ,autoimmune disorders ,connective tissue disorder ,Medicine ,Immunologic diseases. Allergy ,RC581-607 - Abstract
Background. Primary Sjögren’s syndrome (pSS) is an autoimmune epithelitis associated with various pulmonary manifestations, including PAH (pulmonary arterial hypertension), a severe complication often found in collagen tissue disorders. Case presentation. A 26-year-old female having chest pain and exertional dyspnea diagnosed with severe PAH associated with pSS is presented in this case study. Clinical, laboratory, and imaging findings supported the diagnosis, and treatment involved immunosuppressive therapy and standard PAH medications. Discussion and conclusion. The report highlights the complexities of diagnosing and treating PAH associated with pSS, emphasizing the importance of early intervention for improved long-term outcomes. The prognosis for PAH in connective tissue diseases, encompassing pSS, remains challenging, underscoring the need for accurate diagnosis and timely management with immunosuppressants and PAH-specific therapies.
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- 2024
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20. Associations between Sjogren syndrome and psychiatric disorders in European populations: a 2-sample bidirectional Mendelian randomization study.
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Lingai Pan, Guangpeng Zhou, Guocui Wei, Qian Zhao, Yanping Wang, Qianlan Chen, Qing Xiao, Yujie Song, Xiangui Liang, Zhili Zou, Xiuxia Li, and Xuan Xiong
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GENETIC disorders ,SJOGREN'S syndrome ,GENOME-wide association studies ,MENTAL illness ,BIPOLAR disorder - Abstract
Background: Psychiatric disorders, such as major depressive disorder (MDD), anxiety disorder (AD), bipolar disorder (BD), and schizophrenia (SCZ), are disturbances in brain activity that lead to disorders of cognition, behavior, and emotion regulation. Among Sjogren syndrome (SS) patients, psychiatric disorders are more prevalent than in the general population. Identifying associated risk factors can provide new evidence for clinical diagnosis and treatment. Methods: We selected genetic instruments based on published genome-wide association studies (GWASs) to determine predisposition. Then, we conducted a 2-sample bidirectional Mendelian randomization (MR) analysis to explore the potential causal associations between SS and four major psychiatric disorders. The primary analysis was performed using MR with the inverse-variance weighted method. Confirmation was achieved through Steiger filtering and testing to determine the causal direction. Sensitivity analyses were conducted using MR-Egger, MR-PRESSO, and "leave-one-out" method methods. Results: Our study showed that SS was linked to BD and SCZ, indicating that individuals with SS may have a reduced risk of developing BD (IVW: OR = 0.940, P=0.014) and SCZ (IVW: OR = 0.854, P=1.47*10-4), while there was no causal relationship between SS and MDD or AD. MR-Egger regression shows no evidence of pleiotropy (BD: intercept = 0.007, p = 0.774; SCZ: intercept = 0.051, p = 0.209). The same as the MR-PRESSO analysis (BD: global test p = 1.000; SCZ: global test p = 0.160). However, the results from the leave-one-out analysis demonstrated instability. Specifically, after excluding SNP rs3117581, the effects on BD and SCZ were found to be non-significant, suggesting the potential influence of unrecognized confounding factors. The results of the reverse MR show that four major psychiatric disorders had no causal effects on SS. Conclusions: Our research findings demonstrate a causal relationship between SS and SCZ, as well as between SS and BD. There are no causal effects between the four major psychiatric disorders and SS. These findings suggest that SS may have the potential to reduce the risk of both psychiatric disorders. This study provides new insight for their prevention and treatment. [ABSTRACT FROM AUTHOR]
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- 2024
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21. Sjögren syndrome induced by anti PDL-1 treatment for TNBC: case report and review of literature.
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Pellegrino, Caterina, D'Antonio, Chiara, Ierinò, Debora, Onesti, Concetta Elisa, Aschelter, Anna Maria, Santini, Daniele, and Mazzuca, Federica
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TRIPLE-negative breast cancer ,SJOGREN'S syndrome ,METASTATIC breast cancer ,IMMUNE checkpoint inhibitors ,ADVERSE health care events - Abstract
Background: Rheumatological toxicity associated with immunotherapy, particularly Sjögren's syndrome (SjS), has been observed with variable incidence in patients treated with immune checkpoint inhibitors (ICIs). Although SjS is a well-known autoimmune disease, its occurrence as an immune-related adverse event (irAE) during cancer treatment is less well understood. Current literature documents a range of incidence rates and clinical manifestations of SjS in patients undergoing ICI therapy, highlighting the need for early diagnosis and multidisciplinary management. Case presentation: A 40-year-old woman underwent mammography, which revealed a 43 mm mass in the left breast. Core biopsy confirmed grade 3 infiltrating triple negative ductal carcinoma with high MIB-1. She received neoadjuvant chemotherapy, followed by surgery and radiotherapy. A CT scan in September 2022 showed lung nodules and lymph node involvement. A lung biopsy confirmed breast cancer metastasis. She started treatment with atezolizumab and nab-paclitaxel with evidence of a partial response. Nabpaclitaxel was discontinued due to side effects and atezolizumab was continued as maintenance therapy. After four cycles, the patient developed symptoms consistent with Sjögren's syndrome (SjS), which were confirmed by diagnostic tests. Treatment wi th prednisone, pilocarpine and hydroxychloroquine was initiated alongside ongoing immunotherapy. The patient continues to receive atezolizumab with stable disease and good quality of life. Conclusion: This case highlights the importance of recognizing SjS as a potential irAE in patients treated with ICIs, particularly those with TNBC. Multidisciplinary collaboration is essential for the prompt diagnosis and effective management of SjS to maintain both cancer control and patient quality of life. Given the recent emergence of these events and the lack of specific guidelines, our case report may provide valuable insights into the management of a little-known adverse event and pave the way for further real-world data collection on the management of these rare but significant toxicities that impact on patient quality of life. Further research is needed to optimize treatment protocols and outcomes for patients experiencing rheumatological irAEs during cancer immunotherapy. [ABSTRACT FROM AUTHOR]
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- 2024
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22. Adipose derived or bone-marrow derived mesenchymal stem cell treatment for hyposalivation: protocol for a systematic review and network meta-analysis.
- Author
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Hansen, Joachim, Carlander, Amanda-Louise Fenger, Jakobsen, Kathrine Kronberg, Grønhøj, Christian, and von Buchwald, Christian
- Subjects
SJOGREN'S syndrome ,STEM cell treatment ,MESENCHYMAL stem cells ,RANDOM effects model ,PATIENT reported outcome measures ,XEROSTOMIA - Abstract
Background: Salivary hypofunction leads to debilitating oral symptoms and has major complications for overall quality of life. Two of the most frequent causes of xerostomia are radiotherapy in the head and neck and Sjögren's syndrome. Only symptomatic treatment is available today. An increasing number of both preclinical and clinical studies have suggested that mesenchymal stem cell (MSC) transplantation treatment can increase the salivary flow rate and ameliorate symptoms of xerostomia. However, both adipose-derived and bone marrow–derived MSCs are used, although they differ in important ways. The primary objective of this study is an indirect comparison of the change in the unstimulated salivary flow rate after intervention between patients treated with adipose-derived or bone marrow–derived MSCs. Methods: This systematic review and network meta-analysis will search for eligible studies in the MEDLINE, EMBASE, and Cochrane CENTRAL register of Controlled Trials. Eligible studies are as follows: clinical studies including human patients with salivary hypofunction due to either radiotherapy or Sjogren's syndrome who were subsequently treated with either adipose-derived MSCs or bone marrow–derived MSCs. Studies with no control group will be excluded. The search phrase has been peer-reviewed following the PRESS guidelines. The primary outcome is the change in the unstimulated salivary flow rate after treatment with either adipose-derived or bone marrow–derived MSCs. Secondary outcomes are as follows: change in patient reported outcomes, methods of intervention administration, number of injected MSCs, and safety. Data from included studies will be pooled and compared with a fixed-effects or random effects model dependent on signs of heterogeneity, presented with a forest plot, and indirectly compared with a meta-regression in a network meta-analysis. Risk of bias will be assessed with the tools ROBINS-I or RoB-2 depending on type of study. Discussion: Both adipose-derived and bone marrow–derived MSCs are used today for experimental treatment of salivary hypofunction in humans as no direct or indirect comparisons have been made. Therefore, an evaluation of the effect of adipose-derived vs bone marrow–derived MSC treatment is needed to support future decision-making on the type of MSC used in a clinical trial. Systematic review registration: PROSPERO ID CRD42024527183. [ABSTRACT FROM AUTHOR]
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- 2024
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23. A Rare Case of Sjogren's Syndrome with Polymyositis: A Case Report.
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Gohil, Namra, Patel, Apurva, Gohil, Aasvi, and Solanki, Dipak
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THERAPEUTIC use of vitamin D ,PATIENT compliance ,PHYSICAL diagnosis ,BIOPSY ,RARE diseases ,PREDNISOLONE ,PILOCARPINE ,FLUORESCENT antibody technique ,INTRAVENOUS therapy ,ELECTROMYOGRAPHY ,POLYMYOSITIS ,VITAMIN B6 ,SJOGREN'S syndrome - Abstract
Introduction: Sjogren's syndrome (SS) is an autoimmune chronic inflammatory disorder affecting women in their fourth to sixth decade, affecting gastrointestinal, and musculoskeletal systems. A 35-year-old female with SS with polymyositis (PM) presented with symptoms of weakness in all four limbs, difficulty in sitting, rising, swallowing solid foods, vomiting, and difficulty in climbing stairs. She was diagnosed with SS in 2018 and was treated with prednisolone, Vitamin D, calcium, pyridoxine, methylcobalamin, artificial tears, pilocarpine, and painkillers. However, a muscle biopsy was never done. Methods: The data were collected from the patient's file along with her consent when she came for follow-up. Results: A muscle biopsy was done at our center to confirm the diagnosis of PM. The patient was prescribed IV prednisolone and other symptomatic treatment until symptoms resolved and was discharged with oral drugs when they were manageable. Conclusion: The diagnosis of primary SS along with PM is a rare occurrence. Although it does not change the treatment plan much, its diagnosis is very important for managing any complications that may arise from it. Patients' noncompliance and loss of follow-up can create issues in the treatment. Such cases help in forming the guidelines for the future and restructuring the classification of autoimmune conditions. [ABSTRACT FROM AUTHOR]
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- 2024
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24. Sicca syndrome/Sjögren's disease associated with cancer immunotherapy: a narrative review on clinical presentation, biomarkers, and management.
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Pasoto, Sandra Gofinet, Franco, André Silva, Silva, Clovis Artur, and Bonfa, Eloisa
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SJOGREN'S syndrome ,IMMUNE checkpoint inhibitors ,DRY eye syndromes ,SALIVARY glands ,B cells - Abstract
Introduction: Almost one-quarter of immune checkpoint inhibitor (ICI) recipients experience sicca syndrome, while Sjögren's disease (SjD) is estimated at 0.3–2.5%, possibly underreported. Areas covered: This narrative review (Medline/Embase until January/31/2024) addresses the pathophysiology, incidence, demographic/clinical features, biomarkers, labial salivary gland biopsy (LSGB), fulfillment of the idiopathic SjD (iSjD) classificatory criteria, differential diagnosis, and management of sicca syndrome/SjD associated with ICIs. Expert opinion: SjD associated with ICIs is underdiagnosed, since studies that performed the mandatory SjD investigation identified that 40–60% of patients with sicca syndrome associated with ICIs meet the iSjD classificatory criteria. LSGB played a fundamental role in recognizing these cases, as most of them had negative anti-Ro/SS-A antibody. Despite the finding of focal lymphocytic sialoadenitis in LSGB samples mimicking iSjD, immunohistochemical analysis provided novel evidence of a distinct pattern for sicca syndrome/SjD associated with ICIs compared to iSjD. The former has scarcity of B lymphocytes, which are a hallmark of iSjD. Additionally, patients with sicca syndrome/SjD associated with ICIs have demographical/clinical/serological and treatment response dissimilarities compared to iSjD. Dryness symptoms are more acute in the former than in iSjD, with predominance of xerostomia over xerophthalmia, and partial/complete response to glucocorticoids. Dryness symptoms in ICI-treated patients warrant prompt SjD investigation. [ABSTRACT FROM AUTHOR]
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- 2024
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25. Prevalence of celiac disease in systemic lupus erythematosus, sjogren syndrome and systemic sclerosis: A systematic review and meta-analysis.
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Beas, Renato, Altamirano-Farfan, Euler, Izquierdo-Veraza, Diego, Norwood, Dalton A., Riva-Moscoso, Adrian, Godoy, Ambar, Montalvan-Sanchez, Eleazar E., Ramirez, Mirian, Guifarro, Daniel A., Kitchin, Emily, Fischer, Monika, and Kurada, Satya
- Abstract
Celiac disease (CeD) is an immune-mediated disorder affecting the small bowel, associated with genetic factors and increasing global prevalence. This study explores the association between CeD, Systemic Lupus Erythematosus (SLE), primary Sjogren syndrome (pSS), and Systemic Sclerosis (SSc). A systematic review and meta-analysis were conducted following PRISMA guidelines. Searches across multiple databases yielded 2728 articles, with 15 studies selected. Data extraction included study characteristics, prevalence of CeD and CeD antibodies in SLE, pSS, and SSc. Quality assessment utilized the Newcastle-Ottawa Scale. The meta-analysis revealed a pooled prevalence of biopsy-proven CeD in SLE, pSS, and SSc of approximately 3%. Seroprevalence of any CeD antibody in SLE, pSS, and SSc ranged from 3% to 10%. Notably, pSS exhibited the highest prevalence at 5.59%. High heterogeneity was observed in seroprevalence across autoimmune conditions. Quality assessment indicated robust methodological quality in the selected studies. This study highlights a significantly higher prevalence of CeD, especially pSS, compared to the general population. The findings underscore the importance of recognizing elevated CeD antibodies in patients with SLE, pSS and SSc emphasizing the need for early detection and comprehensive care for gastrointestinal symptoms in these conditions. [ABSTRACT FROM AUTHOR]
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- 2024
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26. Confocal Microscopy of the Cornea in Aqueous-Deficient Dry Eye Disease—A Literature Review.
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Bucsan, Radu, Coroleucă, Ruxandra, Garhöfer, Gerhard, Popa-Cherecheanu, Alina, Schmetterer, Leopold, and Iancu, Raluca
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- *
SJOGREN'S syndrome , *DRY eye syndromes , *LITERATURE reviews , *CONFOCAL microscopy , *DENDRITIC cells - Abstract
Background: In vivo confocal microscopy (IVCM) is a vital tool in studying dry eye disease (DED), providing insights into morphological changes at ocular surface unit levels. This review presents the main differences in corneal structure between aqueous-deficient dry eye disease (AD-DED) and normal eyes. Methods: A comprehensive search of PubMed, Web of Science, Embase, and MEDLINE databases from January 2000 to December 2023 was conducted. The study selection process, as well as data selection and examination, were independently performed by two members of the review team. Results: The review reveals a consistent decrease in corneal surface epithelial cell density in AD-DED cases compared to a control group, but conflicting data on basal epithelial cell density. Notably, the abnormal hyperreflectivity of keratocytes in patients with Sjogren's syndrome was recorded, and there was a significant keratocyte density in AD-DED subjects compared to evaporative DED and control groups. Studies also found a decrease in sub-basal nerve density, increased tortuosity, and the fragmentation of nerve fibers. Dendritic cell density and dendritic cell dendrites increase in AD-DED patients compared to healthy subjects. Conclusions: IVCM is a powerful tool for enhancing our understanding of the pathophysiological mechanisms underlying DED. However, the review underscores the urgent need to standardize the terminology, analysis, and units used for accurate interpretation, a crucial step in advancing our knowledge of DED. [ABSTRACT FROM AUTHOR]
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- 2024
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27. Neurological and Psychiatric Clinical Manifestations of Sjögren Syndrome.
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Popescu, Alexandra, Hickernell, John, Paulson, Anisha, and Aouhab, Zineb
- Abstract
Purpose of Review: Sjögren Syndrome is a systemic autoimmune disorder that presents mainly with sicca symptoms, but frequently affects other body systems which can lead to a wide variety of manifestations. Understanding the neurological and psychiatric manifestations of Sjögren Syndrome can help with an earlier diagnosis of this disease and leads to better clinical outcomes. Recent Findings: We provide an updated overview of the central neurological manifestations, peripheral neurological manifestations and psychiatric manifestations and their diagnosis when associated with primary Sjögren Syndrome. The epidemiology and clinical features of the neurological and psychiatric manifestations are derived from different cohort studies and review articles that were selected from PubMed searches conducted between January 2024 and March 2024. Summary: The absence of diagnostic criteria and the scarcity of large, robust studies makes the recognition of the neurological and psychiatric manifestations of Sjögren Syndrome more difficult. Maintaining a high index of suspicion in clinical practice and a close collaboration between the Neurologist and the Rheumatologist will facilitate the diagnosis and management of these patients. [ABSTRACT FROM AUTHOR]
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- 2024
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28. Sjogren's Syndrome in the Mask of Hypokalemia: A Case Report
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Krishna Bahadur Sodari, Pragyan Basnet, and Krishna Adhikari
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case report ,hypokalemia ,renal tubular acidosis ,sjogren syndrome ,Medicine (General) ,R5-920 - Abstract
Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in xerostomia and dry eyes affecting entire body. A 40-year-old woman presented with generalized weakness and shortness of breath. She had reduced muscle power (2/5) with reduced tone, and absent reflexes in four limbs. Further investigation revealed severe hypokalemia with metabolic acidosis. She was treated with intravenous potassium chloride and diagnosed with Sjogren's syndrome based on her serology profile. After treatment, her condition improved. Hypokalemia occurs due to increased renal excretions of potassium. Distal Renal Tubular Acidosis is a major cause of potassium loss in urine. Sjogren's Syndrome is an important cause of Distal Renal Tubular Acidosis, which can present as severe hypokalemia. Although it commonly presents with symptoms like dry eyes and dry mouth, it can rarely present as Distal Renal Tubular Acidosis and hypokalemia as the primary presentation. Sjogren's syndrome should be suspected in patients with hypokalemia with features of Distal Renal Tubular Acidosis.
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- 2024
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29. Sialendoscopy enhances saliva production of parotid glands in primary Sjögren syndrome patients.
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Hagai, Aharon, Mohana, Aladdin, Shalabi, Akram, Adawi, Mohamed, Ben Amy, Dalit Porat, and El Naaj, Imad Abu
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PAROTID gland physiology ,ENDOSCOPIC surgery ,SALIVA ,ORAL health ,TREATMENT effectiveness ,COMPARATIVE studies ,QUALITY of life ,DESCRIPTIVE statistics ,SJOGREN'S syndrome ,ENDOSCOPY ,DISEASE complications ,EVALUATION - Abstract
Objective: Primary Sjögren syndrome (pSS) is a chronic systemic autoimmune disorder that harms exocrine glands located mainly in the oral and ocular regions. pSS patients often complain about pain and mouth dryness. The aim of this retrospective study was to evaluate the influence of parotid glands’ sialendoscopy on salivary flow in pSS patients and to assess the tissue characteristics of the parotid glands during the sialendoscopy procedure. Method and materials: Twenty-six pSS patients (52 glands) treated with sialendoscopy for their parotid glands between the years 2017 and 2019 were included. The unstimulated whole saliva (UWS) flow rate was obtained 2 weeks before intervention (T1) and 3 months post intervention (T2). For 20 patients, UWS was measured 6 months post intervention (T3); and for 11 patients, UWS was measured 12 months post intervention (T4). Patients were asked about their oral quality of life before and after sialendoscopy. Results: UWS was significantly higher at T2, T3, and T4 compared to T1 (P = .002, P = .01, and P = .04 respectively). In total, 22 patients (84.6%) reported substantial improvement of their oral quality of life at T2. Of the 52 glands, 33 (63.5%) exhibited avascularity in the walls of the Stensen duct, 33 (63.5%) had strictures, and in 8 (15.4%) mucus plugs were present. No major complications were observed in this study except for one patient who suffered from duct perforation, which during follow-up was spontaneously healed. Conclusion: This study indicates a positive effect of sialendoscopy on salivary flow rate and oral quality of life in pSS patients. Sialendoscopy should be considered as a vital tool in treating pSS patients. [ABSTRACT FROM AUTHOR]
- Published
- 2023
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30. A scientometric and comparative study of Sjogren's syndrome research by rheumatologists and stomatologists
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Zuohao Zhang, Hanyi Zhu, Zhenhu Ren, Huan Shi, and Wei Liu
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Bibliometric ,Oral complications ,Rheumatology ,Sjögren syndrome ,Stomatology ,Systemic comorbidities ,Dentistry ,RK1-715 - Abstract
Background/purpose: The diagnosis and treatment of Sjogren's syndrome (SS) are commonly conducted by rheumatologists and stomatologists. The purpose of this study was to compare the scientometric characteristics of SS publications by rheumatologists and stomatologists. Materials and methods: All the papers on cheilitis were comprehensively retrieved from the Scopus database, and divided into rheumatologists and stomatologists groups. Results: There were 3245 and 1209 papers on SS were published by rheumatologists and stomatologists, respectively. For the most-cited top-200 papers, the total citation count was 29,764 and the h index was 108 for SS publications by rheumatologists; whereas the count is 19,891 and h index is 81 for publications by stomatologists. Interestingly, we observed that accumulated citations of the publications by stomatologists cooperated with rheumatologists were larger than those by stomatologists alone during 2012–2022. The more common keywords such as saliva, salivation, minor salivary glands, parotid gland, submandibular gland, sialography, lip, dental caries, and hyposalivation were reported by stomatologists. The more frequent keywords such as rheumatoid factor, fatigue, lymphoma, interstitial lung disease, arthralgia, Raynaud phenomenon, lymphadenopathy, and vasculitis were reported by rheumatologists. Conclusion: This study firstly reports the scientometric characteristics of SS publications by rheumatologists and stomatologists. The scale and citations of rheumatologists' publications greatly outweigh those of stomatologists, suggesting stomatologists can cooperate more with rheumatologists regarding SS research.
- Published
- 2024
- Full Text
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31. RENAL TUBULAR ACIDOSIS DUE TO SJOGREN'S SYNDROME PRESENTING AS HYPOKALEMIC QUADRIPARESIS: A CASE REPORT.
- Author
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Islam, Asif and Khurshid, Fatima
- Abstract
Sjögren's syndrome is characterized by dry mouth and eyes symptoms caused by lymphocyte infiltration of the exocrine glands as it is an autoimmune condition. Nonetheless, this condition can lead to symptoms and various illnesses. Renal tubular acidosis (RTA) occurs when the kidney tubules are unable to regulate acid-base balance. This study tracked a 40-year-old female who initially showed symptoms of hypokalemic stroke and normal anion gap acidosis and was later determined to have Sjögren's syndrome. The patient complains of weakness in the legs, vomiting, abdominal pain and dry cough. Like other Sjögren's symptoms, dry eyes and mouth have been present for years. Electrolyte abnormalities and lymphocytic infiltration in the lymph nodes can be confirmed by physical examination and laboratory tests. Positive antibodies, Schirmer test, and histology results indicate the diagnosis. Treatments include potassium replenishment, artificial tears, and the use of coconut oil. Early diagnosis and treatment can help prevent the condition from worsening and improve patient outcomes. It also emphasizes the importance of completing a comprehensive evaluation that includes organ system and specific markers of disease. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
32. Assessment of hydroxychloroquine blood levels in Sjögren's disease patients: drug adherence and clinical associations.
- Author
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Pasoto, Sandra Gofinet, Villamarín, Lorena Elizabeth Betancourt, de Vinci Kanda Kupa, Léonard, Deveza, Giordano Bruno Henriques, Ribeiro, Carolina Torres, Emi Aikawa, Nádia, Leon, Elaine Pires, de Oliveira Martins, Victor Adriano, Silva, Clovis Artur, and Bonfa, Eloisa
- Subjects
- *
PATIENT compliance , *LIQUID chromatography-mass spectrometry , *SJOGREN'S syndrome , *HYDROXYCHLOROQUINE , *HIGH performance liquid chromatography , *XEROSTOMIA - Abstract
Hydroxychloroquine (HCQ) has been used to treat Sjögren's disease (SjD) patients. However, there are no studies evaluating drug adherence through HCQ blood levels, pharmacy refill (PR) and medication adherence questionnaires. The relationship of HCQ blood levels with glandular/extraglandular disease parameters was also poorly assessed. This cross-sectional observational study included 74 adult SjD patients, who were receiving a stable HCQ dose (4-5.5 mg/kg/day, actual weight) for at least 3 months before study inclusion. HCQ blood levels were quantified by high-performance liquid chromatography coupled to mass spectrometry. Adherence was assessed by PR and Medida de Adesão aos Tratamentos (MAT) questionnaire. The following parameters were evaluated: Xerostomia Inventory, Ocular Surface Disease Index, EULAR (European League Against Rheumatism) Sjögren's Syndrome Disease Activity Index, EULAR Sjögren's Syndrome Patient Reported Index, Schirmer's I test and non-stimulated/stimulated salivary flow rates. HCQ blood levels were 775.3(25.0–2,568.6)ng/mL. Eleven patients (14.9%) had HCQ blood levels < 200ng/mL (non-adherent group); 11(14.9%), 200-499ng/mL (sub-therapeutic levels group); and 52(70.2%), ≥ 500ng/mL (adherent group). PR classified incorrectly all non-adherent/sub-therapeutic patients and 2/52(3.9%) adherent patients. Using MAT, the overall misclassification was 24/52(46.2%) in the adherent group, and were correctly identified 9/11(81.8%) patients in non-adherent and 7/11(63.6%) in sub-therapeutic groups. MAT sensitivity and specificity to identify non-adherent/sub-therapeutic patients were 72.7% and 53.9%, respectively. The three groups were comparable regarding glandular/extraglandular disease parameters (p > 0.05). The assessment of HCQ blood levels is a promising tool for evaluating drug adherence in SjD. This is particularly crucial as one-third of patients exhibited non-adherence/sub-therapeutic levels, and neither PR nor MAT reliably identified these patients. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
33. A scientometric and comparative study of Sjogren's syndrome research by rheumatologists and stomatologists.
- Author
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Zhang, Zuohao, Zhu, Hanyi, Ren, Zhenhu, Shi, Huan, and Liu, Wei
- Subjects
SJOGREN'S syndrome ,RHEUMATOLOGISTS ,DENTAL caries ,RHEUMATOID factor ,INTERSTITIAL lung diseases ,SUBMANDIBULAR gland - Abstract
The diagnosis and treatment of Sjogren's syndrome (SS) are commonly conducted by rheumatologists and stomatologists. The purpose of this study was to compare the scientometric characteristics of SS publications by rheumatologists and stomatologists. All the papers on cheilitis were comprehensively retrieved from the Scopus database, and divided into rheumatologists and stomatologists groups. There were 3245 and 1209 papers on SS were published by rheumatologists and stomatologists, respectively. For the most-cited top-200 papers, the total citation count was 29,764 and the h index was 108 for SS publications by rheumatologists; whereas the count is 19,891 and h index is 81 for publications by stomatologists. Interestingly, we observed that accumulated citations of the publications by stomatologists cooperated with rheumatologists were larger than those by stomatologists alone during 2012–2022. The more common keywords such as saliva, salivation, minor salivary glands, parotid gland, submandibular gland, sialography, lip, dental caries, and hyposalivation were reported by stomatologists. The more frequent keywords such as rheumatoid factor, fatigue, lymphoma, interstitial lung disease, arthralgia, Raynaud phenomenon, lymphadenopathy, and vasculitis were reported by rheumatologists. This study firstly reports the scientometric characteristics of SS publications by rheumatologists and stomatologists. The scale and citations of rheumatologists' publications greatly outweigh those of stomatologists, suggesting stomatologists can cooperate more with rheumatologists regarding SS research. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
34. Increased Interferon Signaling in Vaginal Tissue of Patients With Primary Sjögren Syndrome.
- Author
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Visser, Annie, van Nimwegen, Jolien F., Wilbrink, Rick, Liefers, Silvia C., van der Tuuk, Karin, Mourits, Marian J. E., Diercks, Gilles F. H., Bart, Joost, van der Vegt, Bert, van Kempen, Léon C., Bootsma, Hendrika, Kroese, Frans G. M., and Verstappen, Gwenny M.
- Subjects
SJOGREN'S syndrome ,VAGINAL dryness ,INTERFERONS ,DENDRITIC cells ,TISSUES ,TRANSFORMING growth factors - Abstract
Objective. Vaginal dryness is an important factor influencing sexual function in women with primary Sjögren syndrome (pSS). Previous studies showed a higher degree of inflammation in vaginal biopsies from patients with pSS compared to non-pSS controls. However, the molecular pathways that drive this inflammation remain unclear. Therefore, the aim of this study was to investigate inflammatory pathway activity in the vaginal tissue of patients with pSS. Methods. Vaginal biopsies of 8 premenopausal patients with pSS experiencing vaginal dryness and 7 age-matched non-pSS controls were included. Expression of genes involved in inflammation and tissue homeostasis was measured using NanoString technology and validated using TaqMan Real-Time PCR. Vaginal tissue sections were stained by immunohistochemistry for myxovirus resistance protein 1 (MxA) and CD123 (plasmacytoid dendritic cells [pDCs]). Results. The most enriched pathway in vaginal biopsies from patients with pSS compared to non-pSS controls was the interferon (IFN) signaling pathway (P < 0.01). Pathway scores for Janus kinase and signal transducer and activator of transcription (JAK-STAT) and Notch signaling were also higher (P < 0.01 for both pathways). Conversely, transforming growth factor-β signaling and angiogenesis pathway scores were lower in pSS (P = 0.02 and P = 0.04, respectively). Differences in IFN signaling between patients with pSS and non-pSS controls were confirmed by PCR and MxA tissue staining. No CD123+ pDCs were detected in vaginal biopsies. IFN-stimulated gene expression levels correlated positively with CD45+ cell numbers in vaginal biopsies and serum anti-SSA/Ro positivity. Conclusion. Upregulation of IFN signaling in vaginal tissue of women with pSS, along with its association with tissue pathology, suggests that IFNs contribute to inflammation of the vaginal wall and potentially also to clinical symptomatology (ie, vaginal dryness). [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
35. Invisible, Uncontrollable, Unpredictable: Illness Experiences in Women with Sjögren Syndrome.
- Author
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Herrera, Andrea, Leiva, Loreto, Espinoza, Iris, Ríos-Erazo, Matías, Shakhtur, Nailah, Wurmann, Pamela, and Rojas-Alcayaga, Gonzalo
- Subjects
- *
SJOGREN'S syndrome , *MIDDLE-aged women , *RHEUMATISM , *SOCIAL support , *QUALITY of life - Abstract
Background/Objectives: Sjögren's Syndrome (SS) is a chronic degenerative rheumatic disease. Because of its chronic nature, it significantly affects the quality of life of those who suffer from it. Methods: This qualitative study investigated disease experience among women suffering from SS to understand its impact on their overall well-being. In-depth interviews were conducted with 15 women who suffer from SS. Interviews were analyzed using the Grounded Theory methodology, using open, axial, and selective coding. Results: Three central phenomena of disease experience were identified: invisibility; uncontrollability; and unpredictability. Conclusions: SS disease experience has a strong imprint on emotional well-being and sense of self-control among middle-aged women. Understanding SS impacts on women's lives is important to better understand the disease and contribute to recognizing potential areas of management and social support in relevant windows of opportunity within the health-disease continuum. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
36. Diagnosis, management and outcomes of primary hypokalemic periodic paralysis during pregnancy.
- Author
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Jha, Nivedita, Balachandran, Divya Mecheril, Thabah, Molly Mary, and Jha, Ajay Kumar
- Subjects
- *
MEDICAL history taking , *POTASSIUM , *HYPOKALEMIC periodic paralysis , *TREATMENT effectiveness , *HYPOKALEMIA , *POTASSIUM chloride , *PREGNANCY - Abstract
Primary hypokalaemic periodic paralysis during pregnancy has been rarely reported. Four pregnant women with the acute onset of flaccid paralysis presented between January 2018 and December 2021. Focussed history and physical examination helped an appropriate radiological and laboratory investigation plan to be made. All women recovered within 4–7 days of potassium supplementation. Supplemental potassium continued until delivery. A pain management plan with continuous epidural infusion helped in avoiding stress-induced hypokalaemia. None of the women developed an episode of muscle weakness during the intervening period. In conclusion, a focussed history and targeted laboratory investigation are needed to diagnose primary hypokalaemic periodic paralysis. Early administration of oral or intravenous potassium is crucial in improving fetomaternal outcomes. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
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37. Exploring Sjögren's syndrome through interdisciplinary perspectives: a concise review.
- Author
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Rathore, Tanisha and Dattatri, Mayur
- Subjects
- *
SJOGREN'S syndrome , *MOUTH , *RHEUMATOID arthritis , *AUTOIMMUNE diseases , *DISEASE prevalence , *XEROSTOMIA - Abstract
Dr. Henrik Sjögren after whom Sjögren's Syndrome is named, was a Swedish ophthalmologist who identified the syndrome which had three main symptoms namely, dry eyes, dry mouth, and arthritis. His contributions also highlighted the systemic complications of the syndrome which made our understanding of this disease better. Since then, there have been several studies on Sjögren's Syndrome (SS) of which two of them have changed the perception of the disease's prevalence. The first was a British study in the late 1990s which indicated this syndrome was no more a rare condition. The second is a 2008 study in the US which placed the syndrome as the second most prevalent autoimmune disease after rheumatoid arthritis (RA). Being one of the most prevalent autoimmune disease, there is a pressing need for a more profound and comprehensive understanding of the syndrome. This review endeavors to offer a comprehensive overview of the disease, encompassing its prevalence, manifestations, mechanisms, genetic factors, diagnostic methods, and treatment options. This review additionally offers the āyurvedic viewpoint on SS and its symptoms. This supplementary insight has the potential to contribute to the development of an integrated and holistic approach to managing the condition. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
38. Rheumatology
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Brown, Amanda G. and Naga, Osama, editor
- Published
- 2024
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39. Cutaneous Connective Tissue Diseases
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Prohic, Asja and Prohic, Asja
- Published
- 2024
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40. Sjögren syndrome induced by anti PDL-1 treatment for TNBC: case report and review of literature
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Caterina Pellegrino, Chiara D’Antonio, Debora Ierinò, Concetta Elisa Onesti, Anna Maria Aschelter, Daniele Santini, and Federica Mazzuca
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immunorelated adverse events ,Sjogren syndrome ,triple negative breast cancer ,immune checkpoint inhibitors ,case report ,Immunologic diseases. Allergy ,RC581-607 - Abstract
BackgroundRheumatological toxicity associated with immunotherapy, particularly Sjögren’s syndrome (SjS), has been observed with variable incidence in patients treated with immune checkpoint inhibitors (ICIs). Although SjS is a well-known autoimmune disease, its occurrence as an immune-related adverse event (irAE) during cancer treatment is less well understood. Current literature documents a range of incidence rates and clinical manifestations of SjS in patients undergoing ICI therapy, highlighting the need for early diagnosis and multidisciplinary management.Case presentationA 40-year-old woman underwent mammography, which revealed a 43 mm mass in the left breast. Core biopsy confirmed grade 3 infiltrating triple negative ductal carcinoma with high MIB-1. She received neoadjuvant chemotherapy, followed by surgery and radiotherapy. A CT scan in September 2022 showed lung nodules and lymph node involvement. A lung biopsy confirmed breast cancer metastasis. She started treatment with atezolizumab and nab-paclitaxel with evidence of a partial response. Nab-paclitaxel was discontinued due to side effects and atezolizumab was continued as maintenance therapy. After four cycles, the patient developed symptoms consistent with Sjögren’s syndrome (SjS), which were confirmed by diagnostic tests. Treatment with prednisone, pilocarpine and hydroxychloroquine was initiated alongside ongoing immunotherapy. The patient continues to receive atezolizumab with stable disease and good quality of life.ConclusionThis case highlights the importance of recognizing SjS as a potential irAE in patients treated with ICIs, particularly those with TNBC. Multidisciplinary collaboration is essential for the prompt diagnosis and effective management of SjS to maintain both cancer control and patient quality of life. Given the recent emergence of these events and the lack of specific guidelines, our case report may provide valuable insights into the management of a little-known adverse event and pave the way for further real-world data collection on the management of these rare but significant toxicities that impact on patient quality of life. Further research is needed to optimize treatment protocols and outcomes for patients experiencing rheumatological irAEs during cancer immunotherapy.
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- 2024
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41. A case of mixed hereditary gelsolin amyloidosis and hydroxychloroquine induced myopathy
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Mak, Gloria, Tarnopolsky, Mark, and Lu, Jian-Qiang
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- 2024
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42. B cell receptor repertoire analysis in primary Sjogren’s syndrome salivary glands identifies repertoire features associated with clinical activity
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Ling Chang, Zihan Zheng, Yiwen Zhou, Kun Liu, Yinong Li, Bing Zhong, Zihua Zhao, Chengshun Chen, Can Qian, Qingshan Ni, Qinghua Zou, Yuzhang Wu, Jingyi Li, and Liyun Zou
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Sjogren syndrome ,pSS ,B cell ,BCR ,IgA2 ,Diseases of the musculoskeletal system ,RC925-935 - Abstract
Abstract Background Primary Sjogren’s syndrome (pSS) is a complex autoimmune disease featuring damage to salivary and lacrimal glands, with the possibility of manifestations across multiple organs. Antibody-producing B cells have long been appreciated to play a significant role in pSS pathogenesis, with a number of autoreactive antibody species having been identified to be elevated in pSS patients. While several studies have attempted to characterize the BCR repertoires of peripheral blood B cells in pSS patients, much remains unknown about the repertoire characteristics of gland-infiltrating B cells. Methods Through paired scRNAseq and scBCRseq, we profiled the BCR repertoires of both infiltrating and circulating B cells in a small cohort of patients. We further utilize receptor reconstruction analyses to further investigate repertoire characteristics in a wider cohort of pSS patients previously profiled through RNAseq. Results Via integrated BCR and transcriptome analysis of B cell clones, we generate a trajectory progression pattern for infiltrated memory B cells in pSS. We observe significant differences in BCR repertoires between the peripheral blood and labial gland B cells of pSS patients in terms of relative expansion, isotype usage, and BCR clustering. We further observe significant decreases in IgA2 isotype usage among pSS patient labial and parotid gland B cells these analyses relative to controls as well as a positive correlation between kappa/lambda light chain usage and clinical disease activity. Conclusions Through BCR repertoire analysis of pSS patient salivary glands, we identify a number of novel repertoire characteristics that may serve as useful indicators of clinical disease and disease activity. By collecting these BCR repertoires into an accessible database, we hope to also enable comparative analysis of patient repertoires in pSS and potentially other autoimmune disorders.
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- 2024
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43. Overlap Syndrome of Diffuse Systemic Sclerosis, Sjögren Syndrome, and ANCA-Associated Renal-Limited Vasculitis: Three Entities in One Patient – Case Report
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Angela Maria Cordoba-Hurtado, Laura Fuentes-Mendez, Lucia Monserrat Perez-Navarro, Virgilia Soto-Abraham, and Rafael Valdez-Ortiz
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overlap syndrome ,diffuse systemic sclerosis ,sjögren syndrome ,anca vasculitis ,glomerulopathies ,case report ,Diseases of the genitourinary system. Urology ,RC870-923 - Abstract
Introduction: The presence of three different entities in a single patient is usually of clinical interest and mostly anecdotal. The overlap of systemic sclerosis (SSc), Sjögren syndrome (SS), and ANCA-associated renal-limited vasculitis has been reported only once previously. Case Presentation: A 61-year-old female was evaluated at consultation with 2 years of symptomatology, presenting cardboard-like skin, sclerodactyly, limited oral opening, and dry skin and eyes. She was admitted for progressive renal failure (serum creatinine, 5.5 mg/dL). Her serology work-up showed positive anti-SCL-70, anti-Ro, anti-La, anti-MPO, and antinuclear antibodies. Renal biopsy was performed and confirmed histological findings for SSc, SS, and ANCA-associated vasculitis with active extracapillary glomerulonephritis with fibrous predominance (EUVAS-Berden sclerotic class), active tubulointerstitial nephritis, focal tubular injury, and moderate chronic arteriolopathy. Treatment with 6 monthly doses of methylprednisolone and cyclophosphamide was established. At the last follow-up, the patient maintained a stable serum creatinine level of 2.6 mg/dL and had decreased proteinuria, no erythrocyturia, and no requirement for renal replacement therapy. Conclusion: Systemic sclerosis is a rare autoimmune disease; nevertheless, overlap with Sjögren syndrome is relatively common, although its association with ANCA vasculitis is anecdotal. Diagnostic integration presents a challenge for nephrologists to define the prognosis and a specific treatment.
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- 2024
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44. Brain tumefactive vasculitis in primary Sjögren syndrome.
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Corrêa, Diogo Goulart, da Hygino da Cruz, Luiz Celso, dos Santos, Roberto Queiroz, Marcondes, Jorge, and de Abreu, Mirhelen Mendes
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SJOGREN'S syndrome , *LACRIMAL apparatus , *MAGNETIC resonance imaging , *LITERATURE reviews , *CENTRAL nervous system - Abstract
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]
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- 2024
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45. Primary Localized Labial Amyloidosis Associated with Sjögren Syndrome.
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Koutlas, Ioannis G. and Ziegler, Erik
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- 2024
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46. Case of successful treatment with glucocorticoid for isolated anti-centromere antibody-positive acute interstitial nephritis
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Takata, Chisa, Kuma, Akihiro, Suwabe, Atsuko, Iwasaki, Takahide, and Kuragano, Takahiro
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- 2024
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47. Rapamycin can alleviate the submandibular gland pathology of Sjögren's syndrome by limiting the activation of cGAS–STING signaling pathway.
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Zhu, Wen, Wang, Yabei, Guan, Yin, Lu, Yun, Li, Yehui, sun, Lixia, and Wang, Yue
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Background: Sjögren's Syndrome (SS) is also known as autoimmune exocrine gland disease. Previous studies have confirmed that adaptive immunity plays an important role in the development of this disease. But less is known about the role of the innate immune system. Methods: To identify the core pathways, and local infiltrated immune cells in the local immune microenvironment of SS. We verified the activation of these core genes and core signaling pathways in SS model mice by in vivo experiment and transcriptome sequencing. Results: Finally, we identified 6 core genes EPSTI1, IFI44L, MX1, CXCL10, IFIT3, and IFI44. All the 6 genes had good diagnostic value. Based on multi-omics sequencing results and experimental studies, we found that cGAS–STING signaling pathway is most relevant to the pathogenesis of SS. By in vivo experiments, we verified that autophagy is the key brake to limit the activation of cGAS–STING signaling pathway. Conclusions: Maladaptive activation of autophagy and cGAS–STING signaling pathway are central contributors to the SG pathogenesis of pSS patient. Regulating autophagy by rapamycin may be a possible treatment for Sjögren's syndrome in the future. [ABSTRACT FROM AUTHOR]
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- 2024
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48. Association between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis: a Taiwanese population-based study.
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Ren-Long Jan, Chung-Han Ho, Cheng-Hao Sung, Jhi-Joung Wang, Han-Yi Jan, Wei-Yu Chen, and Yuh-Shin Chang
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SJOGREN'S syndrome ,OPTIC neuritis ,OPTIC nerve diseases ,SOCIODEMOGRAPHIC factors ,NOSOLOGY ,SYSTEMIC lupus erythematosus ,ANKYLOSING spondylitis - Abstract
Purpose: Our study aimed to explore the correlation between Sjögren syndrome, sociodemographic factors, comorbid conditions, and optic neuritis. Methods: This retrospective, nationwide, population-based, matched case-control investigation involved 33,190 individuals diagnosed with optic neuritis, identified using the International Classification of Diseases, Ninth Revision, Clinical Modification codes 377.30 for optic neuritis or 377.32 for retrobulbar neuritis. Patient data were extracted from the Taiwan National Health Insurance Research Database. Demographic characteristics, the presence of Sjögren syndrome, and pre-existing comorbid conditions were analyzed using univariate logistic regression. Continuous variables were assessed with a paired t-test. Adjusted logistic regression was employed to compare the prognosis odds ratio (OR) of patients with optic neuritis to controls. Results: After adjusting for confounding variables, individuals with Sjögren syndrome exhibited a significantly higher likelihood of developing optic neuritis compared to controls (adjusted OR, 9.79; 95% confidence interval [CI], 7.28-12.98; p < 0.0001). Other conditions associated with increased odds of optic neuritis included rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, systemic lupus erythematosus, and granulomatous vasculitis (adjusted OR: 1.57, 95% CI: 1.33-1.86; adjusted OR: 2.02, 95% CI: 1.65-2.48; adjusted OR: 140.77, 95% CI: 35.02-565.85; adjusted OR: 2.38, 95% CI: 1.71-3.30; adjusted OR: 18.28, 95% CI: 2.21-151.45, respectively), as well as systemic infections such as human herpes viral infection and tuberculosis infection (adjusted OR: 1.50, 95% CI: 1.35-1.66; adjusted OR: 4.60, 95% CI: 3.81-5.56, respectively). Discussion: Our findings strongly support the existence of an association between Sjögren syndrome, rheumatoid arthritis, ankylosing spondylitis, multiple sclerosis, systemic lupus erythematosus, granulomatous vasculitis, human herpes viral infection, tuberculosis, and optic neuritis. [ABSTRACT FROM AUTHOR]
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- 2024
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49. Monitoring of Women with Anti-Ro/SSA and Anti-La/SSB Antibodies in Germany—Status Quo and Intensified Monitoring Concepts.
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Bedei, Ivonne Alexandra, Kniess, David, Keil, Corinna, Wolter, Aline, Schenk, Johanna, Sachs, Ulrich J., and Axt-Fliedner, Roland
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HEART block , *PREGNANT women , *MEDICAL specialties & specialists , *FETAL monitoring , *IMMUNOGLOBULINS - Abstract
Background: The fetuses of pregnant women affected by anti-Ro/anti-La antibodies are at risk of developing complete atrioventricular heart block (CAVB) and other potentially life-threatening cardiac affections. CAVB can develop in less than 24 h. Treatment with anti-inflammatory drugs and immunoglobulins (IVIG) can restore the normal rhythm if applied in the transition period. Routine weekly echocardiography, as often recommended, will rarely detect emergent AVB. The surveillance of these pregnancies is controversial. Home-monitoring using a hand-held Doppler is a promising new approach. Methods: To obtain an overview of the current practice in Germany, we developed a web-based survey sent by the DEGUM (German Society of Ultrasound in Medicine) to ultrasound specialists. With the intention to evaluate practicability of home-monitoring, we instructed at-risk pregnant women to use a hand-held Doppler in the vulnerable period between 18 and 26 weeks at our university center. Results: There are trends but no clear consensus on surveillance, prophylaxis, and treatment of anti-Ro/La positive pregnant between specialists in Germany. Currently most experts do not offer home-monitoring but have a positive attitude towards its prospective use. Intensified fetal monitoring using a hand-held Doppler is feasible for pregnant women at risk and does not lead to frequent and unnecessary contact with the center. Conclusion: Evidence-based guidelines are needed to optimize the care of anti-Ro/La-positive pregnant women. Individual risk stratification could help pregnancy care of women at risk and is welcmed by most experts. Hand-held doppler monitoring is accepted by patients and prenatal medicine specialists as an option for intensified monitoring and can be included in an algorithm for surveillance. [ABSTRACT FROM AUTHOR]
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- 2024
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50. Claudin-10 Decrease in the Submandibular Gland Contributes to Xerostomia.
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He, L., Yuan, S.Z., Mao, X.D., Zhao, Y.W., He, Q.H., Zhang, Y., Su, J.Z., Wu, L.L., Yu, G.Y., and Cong, X.
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SUBMANDIBULAR gland ,XEROSTOMIA ,SJOGREN'S syndrome ,TIGHT junctions ,SALIVARY glands ,MUSCARINIC acetylcholine receptors - Abstract
Tight junction proteins play a crucial role in paracellular transport in salivary gland epithelia. It is clear that severe xerostomia in patients with HELIX syndrome is caused by mutations in the claudin-10 gene. However, little is known about the expression pattern and role of claudin-10 in saliva secretion in physical and disease conditions. In the present study, we found that only claudin-10b transcript was expressed in human and mouse submandibular gland (SMG) tissues, and claudin-10 protein was dominantly distributed at the apicolateral membranes of acini in human, rat, and mouse SMGs. Overexpression of claudin-10 significantly reduced transepithelial electrical resistance and increased paracellular transport of dextran and Na
+ in SMG-C6 cells. In C57BL/6 mice, pilocarpine stimulation promoted secretion and cation concentration in saliva in a dose-dependent increase. Assembly of claudin-10 to the most apicolateral portions in acini of SMGs was observed in the lower pilocarpine (1 mg/kg)–treated group, and this phenomenon was much obvious in the higher pilocarpine (10 mg/kg)–treated group. Furthermore, 7-, 14-, and 21-wk-old nonobese diabetic (NOD) and BALB/c mice were used to mimic the progression of hyposalivation in Sjögren syndrome. Intensity of claudin-10 protein was obviously lower in SMGs of 14- and 21-wk-old NOD mice compared with that of age-matched BALB/c mice. In the cultured mouse SMG tissues, interferon-γ (IFN-γ) downregulated claudin-10 expression. In claudin-10–overexpressed SMG-C6 cells, paracellular permeability was decreased. Furthermore, IFN-γ stimulation increased p-STAT1 level, whereas pretreatment with JAK/STAT1 antagonist significantly alleviated the IFN-γ–induced claudin-10 downregulation. These results indicate that claudin-10 functions as a pore-forming component in acinar epithelia of SMGs, assembly of claudin-10 is required for saliva secretion, and downregulation of claudin-10 induces hyposecretion. These findings may provide new clues to novel therapeutic targets on hyposalivation. [ABSTRACT FROM AUTHOR]- Published
- 2024
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