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Sjogren's disease

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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
#1
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
#2
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
#3
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/29019884/utility-of-osteosclerotic-lesion-biopsy-in-diagnosis-of-poems-syndrome-a-case-report
#4
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
#5
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
#6
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
#7
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/28988418/clinical-utility-of-18-f-fluorodeoxyglucose-positron-emission-tomography-in-diagnosis-of-immunoglobulin-g4-related-sclerosing-sialadenitis
#8
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
#9
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/28982917/gastric-spindle-cell-neuroendocrine-tumor-mimicking-gastrointestinal-stromal-tumor-unique-morphology-and-diagnostic-pitfall
#10
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/28978984/genetic-risk-factors-influence-nighttime-blood-pressure-and-related-cardiovascular-complications-in-patients-with-coronary-heart-disease
#11
Marcin Wirtwein, Olle Melander, Marketa Sjőgren, Michal Hoffmann, Krzysztof Narkiewicz, Marcin Gruchala, Wojciech Sobiczewski
Genetic predisposition of elevated nighttime blood pressure (BP) in patients with coronary heart disease is unknown. We evaluated genetic predisposition and the relationship between elevated nighttime BP and cardiovascular complications over a median of 8.6 years of observation of hypertensive subjects with coronary atherosclerosis confirmed by coronary angiography. Genetic Risk Score (GRS19) was constructed to evaluate the additive effect of single-nucleotide polymorphisms for daytime and nighttime BP. The Receiver Operating Characteristic was used for determination of cutoff points for daytime BP (systolic BP (SBP) 133 mm Hg and diastolic BP (DBP) 77 mm Hg) and nighttime BP (SBP 122 mm Hg and DBP 73 mm Hg)...
October 5, 2017: Hypertension Research: Official Journal of the Japanese Society of Hypertension
https://www.readbyqxmd.com/read/28971643/novel-therapeutic-strategies-in-primary-sj%C3%A3-gren-s-syndrome
#12
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
#13
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/28968830/antibodies-to-aquaporins-are-frequent-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#14
John S Tzartos, Christos Stergiou, Dimitrios Daoussis, Paraskevi Zisimopoulou, Andrew P Andonopoulos, Vassiliki Zolota, Socrates J Tzartos
Objectives: Several aquaporins (AQPs) are present in the salivary glands, likely contributing to their secretions. AQP dysfunction may contribute to the salivary gland dysfunction in SS. Antibodies to AQP4 and AQP1 are detected in neuromyelitis optica and are believed to play a pathogenic role. We aimed to search for antibodies to several AQPs in the sera from SS patients in an effort to shed light on the pathogenic mechanisms of SS. Methods: We searched for antibodies to six AQPs in the sera of 34 SS patients without neurological findings using ELISAs with synthetic peptides corresponding to the three extracellular domains of each AQP, radioimmunoassays with AQPs, Western blots and competition experiments with cell-embedded AQPs...
August 31, 2017: Rheumatology
https://www.readbyqxmd.com/read/28965771/enhanced-expression-of-nlrp3-inflammasome-related-inflammation-in-peripheral-blood-mononuclear-cells-in-sj%C3%A3-gren-s-syndrome
#15
Seong-Kyu Kim, Jung-Yoon Choe, Geon Ho Lee
OBJECTIVE: The aim of this study was to identify the association of NLRP3 inflammasome-induced inflammation with disease activity and damage in Sjögren's syndrome. METHODS: A total of 33 female patients with Sjögren's syndrome and 34 sex- and age-matched, healthy controls were consecutively enrolled. The mRNA expression levels of NLRP3, ASC, caspase-1, interleukin-1β (IL-1β), and IL-18 in peripheral blood mononuclear cells (PBMCs) were measured, as well as serum IL-1β and IL-18 protein expression levels...
September 29, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28964279/pediatric-primary-sj%C3%A3-gren-syndrome-presenting-with-bilateral-ranulas-a-case-report-and-systematic-review-of-the-literature
#16
REVIEW
Casey Means, Mark A Aldape, Ericka King
CONTEXT: Primary Sjögren syndrome is uncommon in children, and the standard clinical criteria used in diagnosis of adult Sjögren syndrome will miss many children with the disease. Floor of mouth ranulas have not been described in Sjögren syndrome. OBJECTIVE: This study aims to describe a novel presentation of juvenile primary Sjögren syndrome, and to present a comprehensive systematic review of the literature regarding the presentation and diagnosis of Sjögren syndrome in children...
October 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28955493/patient-burden-of-sj%C3%A3-gren-s-a-comprehensive-literature-review-revealing-the-range-and-heterogeneity-of-measures-used-in-assessments-of-severity
#17
Katherine M Hammitt, April N Naegeli, Remon W M van den Broek, Julie A Birt
CONTEXT: The severity of Sjögren's syndrome has been evaluated using a wide variety of clinical measures and patient-reported outcomes (PROs). This may contribute to the lack of clarity concerning the burden of Sjögren's from the patient perspective. OBJECTIVE: To perform a comprehensive peer-reviewed literature analysis of the patient aspects of Sjögren's, focusing on PROs, to investigate the complexity underlying the evaluation of the syndrome and to elucidate the discordance between the different measures...
2017: RMD Open
https://www.readbyqxmd.com/read/28951424/myd88-is-required-for-disease-development-in-a-primary-sj%C3%A3-gren-s-syndrome-mouse-model
#18
Jeremy Kiripolsky, Liam G McCabe, Daniel P Gaile, Jill M Kramer
Sjögren's syndrome (SS) is an autoimmune disease that often results in diminished exocrine gland function. SS patients also experience systemic disease manifestations, including hypergammaglobulinemia and pulmonary and renal pathoses. MyD88 is a ubiquitously expressed adaptor molecule used by all immune cells that is required for IL-1 receptor (IL-1R), IL-18R, and most TLR signaling. The precise role of MyD88 in SS has not been evaluated, although this adaptor is critical for development of lupus, a related autoimmune disease...
September 26, 2017: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/28947947/discovery-of-cdz173-leniolisib-representing-a-structurally-novel-class-of-pi3k-delta-selective-inhibitors
#19
Klemens Hoegenauer, Nicolas Soldermann, Frédéric Zécri, Ross S Strang, Nadege Graveleau, Romain M Wolf, Nigel G Cooke, Alexander B Smith, Gregory J Hollingworth, Joachim Blanz, Sascha Gutmann, Gabriele Rummel, Amanda Littlewood-Evans, Christoph Burkhart
The predominant expression of phosphoinositide 3-kinase δ (PI3Kδ) in leukocytes and its critical role in B and T cell functions led to the hypothesis that selective inhibitors of this isoform would have potential as therapeutics for the treatment of allergic and inflammatory disease. Targeting specifically PI3Kδ should avoid potential side effects associated with the ubiquitously expressed PI3Kα and β isoforms. We disclose how morphing the heterocyclic core of previously discovered 4,6-diaryl quinazolines to a significantly less lipophilic 5,6,7,8-tetrahydropyrido[4,3-d]pyrimidine, followed by replacement of one of the phenyl groups with a pyrrolidine-3-amine, led to a compound series with an optimal on-target profile and good ADME properties...
September 14, 2017: ACS Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/28947686/-oral-immune-system-and-oral-immune-disorder
#20
Naozumi Ishimaru
Oral immune system is maintained by various lymphoid tissues in head and neck region and efficient function of oral mucosa and saliva. A variety of oral immune disorders such as infection, allergic disease and autoimmune disease are induced by dysfunction of oral immune system. Sjogren's syndrome is an autoimmune disease that affects exocrine glands, including salivary glands, causing dry mouth symptoms. Because the pathogenesis of Sjogren's syndrome is complex, the study with multiple approaches using many models has been developed to understand the precise mechanism of the disease onset...
2017: Clinical Calcium
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