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Sjogren syndrome

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https://www.readbyqxmd.com/read/29034869/further-symptoms-of-sj%C3%A3-gren-s-syndrome
#1
Holger Kiesewetter
No abstract text is available yet for this article.
September 29, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/29033144/impaired-anti-inflammatory-activity-of-ppar%C3%AE-in-the-salivary-epithelia-of-sj%C3%A3-gren-s-syndrome-patients-imposed-by-intrinsic-nf-%C3%AE%C2%BAb-activation
#2
Aigli G Vakrakou, Alexandros Polyzos, Efstathia K Kapsogeorgou, Dimitris Thanos, Menelaos N Manoussakis
Sjögren's syndrome (SS) patients manifest inflammation in the salivary glands (SG) and evidence of persistent intrinsic activation of ductal SG epithelial cells (SGEC), demonstrable in non-neoplastic SGEC lines derived from patients (SS-SGEC). The peroxisome-proliferator-activated receptor-γ (PPARγ) mediates important anti-inflammatory activities in epithelial cells. Herein, the comparative analysis of SG biopsies and SGEC lines obtained from SS patients and controls had revealed constitutively reduced PPARγ expression, transcriptional activity and anti-inflammatory function in the ductal epithelia of SS patients that were associated with cell-autonomously activated NF-κB and IL-1β pathways...
October 12, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29032462/association-of-tnfsf4-polymorphisms-with-neuromyelitis-optica-spectrum-disorders-in-a-chinese-population
#3
Zhiyun Lian, Ju Liu, Ziyan Shi, Hongxi Chen, Qin Zhang, Huiru Feng, Qin Du, Xiaohui Miao, Hongyu Zhou
The tumor necrosis factor ligand superfamily member 4 (TNFSF4) gene encodes a vital co-stimulatory molecule of the immune system and has been identified as a susceptibility locus for systemic lupus erythematosus, systemic sclerosis, and primary Sjögren's syndrome. However, the association of TNFSF4 polymorphisms with neuromyelitis optica spectrum disorders (NMOSD), an inflammatory, demyelinating autoimmune disease of the central nervous system, has not yet been investigated. To evaluate whether TNFSF4 polymorphisms contribute to risk of NMOSD, four single-nucleotide polymorphisms (SNPs) (rs1234315, rs2205960, rs704840, and rs844648) were selected and genotyped in a cohort of 312 patients with NMOSD and 487 healthy controls...
October 14, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/29032100/-severity-of-illness-index-in-autoimmune-diseases-have-there-any-usefulness-in-medical-practice
#4
A Saunier, M-A Vandenhende, P Morlat, F Bonnet
INTRODUCTION: Assessing disease activity in patients suffering from autoimmune diseases is complex. Symptoms are multiple, often subjective and there are no reliable biomarkers. Many activity scores have been implemented to compare treatment efficacy in clinical trials. Their use in clinical practice is largely unknown. We performed a practical survey to analyze the use of activity scores in clinical practice to consider treatment response and to assess the determinants of their use. METHODS: A sample of French internists answered a questionnaire about activity scores of systemic lupus erythematosus, Sjögren's syndrome, autoimmune myositis and necrotizing vasculitis of small vessels...
October 11, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29027763/primary-sj%C3%A3-gren-s-syndrome-in-asia-yin-and-yang
#5
EDITORIAL
Pulukool Sandhya, Debashish Danda
No abstract text is available yet for this article.
October 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29019884/utility-of-osteosclerotic-lesion-biopsy-in-diagnosis-of-poems-syndrome-a-case-report
#6
Daisuke Hara, Hisanao Akiyama, Saki Nukui, Takahiro Shimizu, Masahiro Hoshikawa, Yasuhiro Hasegawa
RATIONALE: We report a case of successful diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome based on monoclonality that was confirmed by an osteosclerotic lesion biopsy in a patient without pathognomonic symptoms or monoclonal gammopathy, probably because of comorbidities, which included systemic lupus erythematosus, rheumatoid arthritis, and Sjögren syndrome. PATIENT CONCERNS: A 57-year-old woman presented with an approximately 2-year history of numbness in the toes that had gradually spread, along with muscle weakness in both arms and legs...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29018565/health-related-quality-of-life-and-depression-among-participants-in-the-sj%C3%A3-gren-s-international-collaborative-clinical-alliance-registry
#7
Annie Chou, John A Gonzales, Troy E Daniels, Lindsey A Criswell, Stephen C Shiboski, Caroline H Shiboski
OBJECTIVE: To examine health-related quality of life (HRQoL) and depression among participants in an international Sjögren's syndrome (SS) registry, comparing those with and without SS. METHODS: Cross-sectional study of participants in the Sjögren's International Collaborative Clinical Alliance (SICCA) registry. The 2016 American College of Rheumatology/European League Against Rheumatism SS classification criteria were used to determine disease status. HRQoL was assessed using the Short Form 12, version 2 Health Survey to derive scores for physical component summary (PCS) and mental component summary (MCS)...
2017: RMD Open
https://www.readbyqxmd.com/read/28994016/cardiopulmonary-manifestations-of-collagen-vascular-diseases
#8
REVIEW
Hamza Jawad, Sebastian R McWilliams, Sanjeev Bhalla
PURPOSE OF REVIEW: The study aimed to illustrate the cardiopulmonary findings of the following collagen vascular diseases on cross-sectional imaging: rheumatoid arthritis, scleroderma (progressive systemic sclerosis), systemic lupus erythematosus, the inflammatory myopathies (polymyositis/dermatomyositis), and Sjögren's syndrome. RECENT FINDINGS: Although collagen vascular diseases can affect any part of the body, interstitial lung disease and pulmonary hypertension are the two most important cardiopulmonary complications and are responsible for the majority of morbidity and mortality in this patient population...
October 9, 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28990057/id3-may-protect-mice-from-anti%C3%A2-gbm-glomerulonephritis-by-regulating-the-differentiation-of-th17-and-treg-cells
#9
Huan Zhou, Le Wang, Qing Xu, Qingquan Liu, Hui Liu, Wenhui Qiu, Tingyang Hu, Yongman Lv, Qian Zhang
Anti‑glomerular basement membrane glomerulonephritis (anti‑GBM GN) is an autoimmune disease that leads to severe and rapidly progressive renal injury. Inhibition of DNA‑binding factor 3 (ID3) serves a key role in autoimmune diseases, such as asthma and Sjögren's syndrome, and in experimental allergic encephalitis models. However, the role of ID3 in the progression of anti‑GBM GN remains unknown. In the present study, ID3 mRNA expression increased between 3‑ and 20‑fold in the renal tissues of anti‑GBM GN mice compared with the Control group, with a peak at day 14 post‑induction...
October 4, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28988489/fatty-infiltration-of-the-minor-salivary-glands-is-a-selective-feature-of-aging-but-not-sj%C3%A3-gren-s-syndrome
#10
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, C Erick Kaufman, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVE: Determine the presence and assess the extent of fatty infiltration of the minor salivary glands (SG) of primary SS patients (pSS) as compared to those with non-SS sicca (nSS). METHODS: Minor SG biopsy samples from 134 subjects with pSS (n = 72) or nSS (n = 62) were imaged. Total area and fatty replacement area for each glandular cross-section (n = 4-6 cross-sections per subject) were measured using Image J (National Institutes of Health, Bethesda, MD)...
October 8, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28988418/clinical-utility-of-18-f-fluorodeoxyglucose-positron-emission-tomography-in-diagnosis-of-immunoglobulin-g4-related-sclerosing-sialadenitis
#11
Kenichi Takano, Ryoto Yajima, Ryuta Kamekura, Motohisa Yamamoto, Hiroki Takahashi, Naoya Yama, Masamitsu Hatakenaka, Tetsuo Himi
OBJECTIVES/HYPOTHESIS: The aim of this study was to evaluate the utility of (18) F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) for accurately diagnosing immunoglobulin G4-related sclerosing sialadenitis (IgG4-SS). STUDY DESIGN: Retrospective cohort study. METHODS: We reviewed the records of 64 patients with IgG4-SS (35 male and 29 female patients) and 10 patients with clinically suspected IgG4-SS. Pathological diagnoses of patients clinically suspected with IgG4-SS included four cases of malignant lymphoma, one case of multicentric Castleman disease, one case of Sjögren's syndrome, and four cases of sialadenitis...
October 8, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28987046/-igg4-related-disease-a-case-report
#12
Justyna Milczarek-Banach, Kinga Brodzińska, Anna Jankowska, Urszula Ambroziak, Benedykt Szczepankiewicz, Jolanta Nałęcz-Janik, Piotr Miśkiewicz
Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. A CASE REPORT: 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years...
September 29, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28983418/erosive-cervical-spine-involvement-in-primary-sj%C3%A3-gren-s-syndrome
#13
Marco Di Carlo, Marina Carotti, Francesco Sessa, Daniele Roia, Marika Tardella, Fausto Salaffi
No abstract text is available yet for this article.
September 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28982917/gastric-spindle-cell-neuroendocrine-tumor-mimicking-gastrointestinal-stromal-tumor-unique-morphology-and-diagnostic-pitfall
#14
Alexandria A Lee, Nishant Poddar, Muhammad B Hammami, Jula Veerapong, Dengfeng Cao, Jin-Ping Lai
Gastric neuroendocrine tumors (GNETs) with spindle cell morphology are extremely rare. We present a case of a 49-year-old female patient with a history of systemic lupus erythematosus, Sjogren's syndrome, and gastroesophageal reflux disease. She was initially thought to have a spindle cell gastrointestinal stromal tumor per histological studies of the fundic polypectomy samples. Immunohistochemically, the tumor cells were negative for CD117, and CD34, but positive for chromogranin, synaptophysin, and CD56 with a 6% Ki-67 index, consistent with a spindle cell-type well differentiated neuroendocrine tumor, World Health Organization (WHO) Grade 2...
October 2017: Anticancer Research
https://www.readbyqxmd.com/read/28978133/in-depth-phenotyping-of-lymphoblastoid-cells-suggests-selective-cellular-vulnerability-in-marinesco-sj%C3%A3-gren-syndrome
#15
Laxmikanth Kollipara, Stephan Buchkremer, José Andrés González Coraspe, Denisa Hathazi, Jan Senderek, Joachim Weis, René P Zahedi, Andreas Roos
SIL1 is a ubiquitous protein of the Endoplasmic Reticulum (ER) acting as a co-chaperone for the ER-resident chaperone, BiP. Recessive mutations of the corresponding gene lead to vulnerability of skeletal muscle and central nervous system in man (Marinesco-Sjögren syndrome; MSS) and mouse. However, it is still unclear how loss of ubiquitous SIL1 leads to selective vulnerability of the nervous system and skeletal muscle whereas other cells and organs are protected from clinical manifestations. In this study we aimed to disentangle proteins participating in selective vulnerability of SIL1-deficient cells and tissues: morphological examination of MSS patient-derived lymphoblastoid cells revealed altered organelle structures (ER, nucleus and mitochondria) thus showing subclinical vulnerability...
September 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28975700/hydroxychloroquine-decreases-human-msc-derived-osteoblast-differentiation-and-mineralization-in%C3%A2-vitro
#16
Tim Both, H Jeroen van de Peppel, M Carola Zillikens, Marijke Koedam, Johannes P T M van Leeuwen, P Martin van Hagen, Paul L A van Daele, Bram C J van der Eerden
We recently showed that patients with primary Sjögren Syndrome (pSS) have significantly higher bone mineral density (BMD) compared to healthy controls. The majority of those patients (69%) was using hydroxychloroquine (HCQ), which may have favourable effects on BMD. To study the direct effects of HCQ on human MSC-derived osteoblast activity. Osteoblasts were cultured from human mesenchymal stromal cells (hMSCs). Cultures were treated with different HCQ doses (control, 1 and 5 µg/ml). Alkaline phosphatase activity and calcium measurements were performed to evaluate osteoblast differentiation and activity, respectively...
October 3, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28971741/prognostic-value-of-cryoglobulins-protein-electrophoresis-and-serum-immunoglobulins-for-lymphoma-development-in-patients-with-sj%C3%A3-gren-s-syndrome-a-retrospective-cohort-study
#17
Jesse Kimman, Xavier Bossuyt, Daniel Blockmans
BACKGROUND: Patients with Sjögren's syndrome (SS) have a considerable higher risk of lymphoma development. OBJECTIVES: To determine the incidence of lymphoma and the value of biomarkers to predict lymphoma development in patients with SS. METHODS: Clinical files of all patients with a presumed diagnosis of SS between 1991 and 2016 were retrospectively reviewed for the development of lymphoma. Biochemical data were plotted as a function of the relative time before and after the lymphoma diagnosis (for patients who developed lymphoma) or before the last available blood test (for patients who did not develop lymphoma)...
October 3, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/28971643/novel-therapeutic-strategies-in-primary-sj%C3%A3-gren-s-syndrome
#18
Alessia Alunno, Francesco Carubbi, Onelia Bistoni, Elena Bartoloni, Valentina Valentini, Roberto Gerli
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease mainly affecting exocrine glands. However, a subgroup of patients experiences extraglandular manifestations which worsens disease prognosis. To date evidence based guidelines for the management of pSS are lacking, hence the therapeutic approach is mainly based on expert opinion and data from other connective tissue diseases. In recent years, several studies have explored the efficacy and safety of biologic agents in pSS and after the failure of tumor necrosis factor inhibitors, the attention has been focused on compounds directly targeting B or T lymphocytes...
September 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28970488/single-cell-analysis-reveals-sexually-dimorphic-repertoires-of-interferon-%C3%AE-and-il-17a-producing-t-cells-in-salivary-glands-of-sj%C3%A3-gren-s-syndrome-mice
#19
Arun Wanchoo, Alexandria Voigt, Sukesh Sukumaran, Carol M Stewart, Indraneel Bhattacharya, Cuong Q Nguyen
The development of Sjögren's syndrome (SjS) is a dynamic and temporal process with a female predilection. Following the initial influx of immune cells, T cell clusters develop, accelerating the pathology in the salivary glands. Proinflammatory cytokines, IFN-γ and IL-17A, produced by T cells contribute synergistically to the disease. In this study, we examined the sexual dimorphism in cellular infiltrates of the salivary glands by using functional single-cell microengraving analysis. Using high-throughput sequencing, we investigated the clonal diversity of the T cell receptors (TCRs) of infiltrating IFN-γ and IL-17A-producing T cells in male and female SjS-susceptible (SjS(s)) C57BL/6...
October 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28970210/the-parotid-gland-connection-ultrasound-and-biopsies-in-primary-sj%C3%A3-gren-s-syndrome
#20
Esther Mossel, Konstantina Delli, Jolien F van Nimwegen, Alja J Stel, Erlin A Haacke, Frans G M Kroese, Fred K L Spijkervet, Arjan Vissink, Suzanne Arends, Hendrika Bootsma
No abstract text is available yet for this article.
September 29, 2017: Annals of the Rheumatic Diseases
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