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https://www.readbyqxmd.com/read/29148412/psychological-comorbidities-associated-with-subclinical-atherosclerosis-in-greek-patients-with-primary-sj%C3%A3-gren-s-syndrome-a-potential-contribution-of-sleep-impairment
#1
Theofanis Karageorgas, Dimitrios Ioakeimidis, Clio P Mavragani
OBJECTIVES: Impaired sleep and psychological disorders are increasingly recognised as prevalent comorbidities in patients with primary Sjögren's syndrome (pSS), as well as important contributors of atherosclerosis in the general population. In the current study we sought to explore a potential role of psychological comorbidities in the pronounced atherosclerotic risk of pSS patients. METHODS: Fifty-nine pSS patients fulfilling the ACR/EULAR criteria completed specific validated questionnaires assessing fatigue, depression, anxiety and sleep disturbances...
November 14, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29148407/minor-salivary-gland-fibrosis-in-sj%C3%A3-gren-s-syndrome-is-elevated-associated-with-focus-score-and-not-solely-a-consequence-of-aging
#2
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, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVES: Evaluate the presence of minor salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS) as a function of disease pathology or a consequence of ageing. METHODS: Subjects with sicca symptoms attending a Sjögren's research clinic were classified by American European Consensus Group (AECG) criteria as either pSS or non-SS (nSS). Discovery (n=34 pSS, n=28 nSS) and replication (n=35 pSS, n=31 nSS) datasets were evaluated. Minor SG cross-sections from haematoxylin and eosin stained slides were imaged, digitally reconstructed and analysed for percent area fibrosis...
October 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29141312/-clinical-manifestation-and-gene-analyses-of-15-patients-with-intellectual-disability-or-developmental-delay-complicated-with-congenital-nystagmus
#3
Z J Gao, Q Jiang, Q Chen, K M Xu, Z Q Liu, X B Chen, X L Chen
Objective: To analyze the clinical and genetic features of 15 cases with intellectual disability or developmental delay (ID/DD) complicated with congenital nystagmus. Method: The clinical characteristics and the results of laboratory tests, images and genetics of 15 patients with ID/DD complicated with congenital nystagmus, confirmed by gene diagnosis in the Department of Neurology, Children's Hospital Affiliated to Capital Institute of Pediatrics from March 2015 to October 2016, were retrospectively analyzed...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29138598/familial-aggregation-of-myasthenia-gravis-in-affected-families-a-population-based-study
#4
Fu-Chao Liu, Chang-Fu Kuo, Lai-Chu See, Hsin-I Tsai, Huang-Ping Yu
Introduction: Myasthenia gravis (MG) is clinically heterogeneous and can be life-threatening if bulbar or respiratory muscles are involved. However, relative contributions of genetic, shared, and nonshared environmental factors to MG susceptibility remain unclear. The aim of this study was to examine the familial aggregation and heritability of MG and the relative risks (RRs) of other autoimmune diseases in the relatives of patients with MG. Methods: A population-based family study using the Taiwan National Health Insurance (NHI) Database was conducted...
2017: Clinical Epidemiology
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#5
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29127143/satb1-conditional-knockout-results-in-sj%C3%A3-gren-s-syndrome-in-mice
#6
Yuriko Tanaka, Takehiko Sotome, Akiko Inoue, Takanori Mukozu, Taku Kuwabara, Tetuo Mikami, Terumi Kowhi-Shigematsu, Motonari Kondo
Sjögren's syndrome (SS) is an autoimmune disease in which exocrine tissues are affected by cellular and humoral immunity. As a result, the salivary and lacrimal glands of patients with SS are damaged, leading to xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). Because experimental approaches to investigate SS pathogenesis in human patients are limited, development of a mouse model is indispensable for understanding the disease. In this study, we show that special AT-rich sequence binding protein-1 conditional knockout (SATB1cKO) mice, in which the SATB1 gene is specifically deleted from hematopoietic cells, develop SS by 4 wk of age, soon after weaning...
November 10, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29125145/the-2017-doyne-lecture-the-orbit-as-a-window-to-systemic-disease
#7
REVIEW
A A McNab
A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Thrombosis of orbital veins and ischaemic necrosis of orbital and ocular adnexal tissues occur with thrombophilic disorders, vasculitis, and certain bacterial and fungal infections...
November 10, 2017: Eye
https://www.readbyqxmd.com/read/29118759/b-cells-are-indispensable-for-a-novel-mouse-model-of-primary-sj%C3%A3-gren-s-syndrome
#8
Junfeng Zheng, Qiaoniang Huang, Renliang Huang, Fengyuan Deng, Xiaoyang Yue, Junping Yin, Wenjie Zhao, Yan Chen, Lifang Wen, Jun Zhou, Renda Huang, Gabriela Riemekasten, Zuguo Liu, Frank Petersen, Xinhua Yu
Primary Sjögren's syndrome (pSS) is characterized by a panel of autoantibodies, while it is not clear whether B cells and autoantibodies play an essential role in pathogenesis of the disease. Here, we report a novel mouse model for pSS which is induced by immunization with the Ro60_316-335 peptide containing a predominant T cell epitope. After immunization, mice developed several symptoms mimicking pSS, including a decreased secretion of tears, lymphocytic infiltration into the lacrimal glands, autoantibodies, and increased levels of inflammatory cytokines...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29116459/neonatal-systemic-lupus-erythematosus-syndrome-a-comprehensive-review
#9
REVIEW
Federica Vanoni, Sebastiano A G Lava, Emilio F Fossali, Riccardo Cavalli, Giacomo D Simonetti, Mario G Bianchetti, Marie-Ange Bozzini, Carlo Agostoni, Gregorio P Milani
Neonatal lupus erythematosus is an uncommon syndrome, which is caused by transplacental passage of maternal autoantibodies to Sjögren's syndrome A or B autoantigens. The clinical presentation includes distinctive cutaneous lesions resembling those seen in systemic lupus erythematosus, hepatobiliary disease, and cytopenias, which disappear with the clearance of maternal autoantibodies. The most severe presentation is a total atrioventricular heart block, which begins during the second trimester of gestation and is irreversible...
November 8, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29115899/therapeutic-inhibitors-for-the-treatment-of-dry-eye-syndrome
#10
Candela Rodríguez-Pomar, Jesus Pintor, Basilio Colligris, Gonzalo Carracedo
Dry eye disease (DED), defined as a multifactorial disease of tears and ocular surface, results in symptoms of discomfort, ocular irritation, visual disturbance and tear film instability. This syndrome is accompanied of ocular surface inflammation and it is produced by a deficient activity of the lacrimal functional unit. In addition, it is associated with systemic autoimmune diseases such as Sjögren´s Syndrome, rheumatoid arthritis, systemic lupus erythematosus and some drug administration. The treatment of dry eye disease is based on the typical signs and symptoms of dry eye, which are associated with hyperosmolarity, ocular surface inflammation, discomfort, visual disturbance, and tear film instability...
November 14, 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29105068/activation-of-plasmacytoid-dendritic-cells-by-apoptotic-particles-mechanism-for-the-loss-of-immunologic-tolerance-in-sj%C3%A3-gren-s-syndrome
#11
Mari Ainola, Pauliina Porola, Yuya Takakubo, Beata Przybyla, Vesa-Petteri Kouri, Tuomas Aleksi Tolvanen, Arno Hänninen, Dan Christian Nordström
Sjögren's syndrome (SS) is a common autoimmune disease targeting salivary and lacrimal glands. It is strongly female-dominant characterized by low estrogen levels combined with a local intracrine dihydrotestosterone defect. We hypothesized that these hormonal deficits lead to increased apoptosis of the epithelial cells and plasmacytoid dendritic cell (pDC) mediated pro-inflammatory host responses. Expression of Toll-like receptors (TLRs) 7 and 9 and cytokine profile was studied in pDCs treated with apoptotic particles collected in consecutive centrifugation steps of media from apoptotic cells...
November 4, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29102045/-myelitis-as-a-differential-diagnosis-of-spinal-cord-tumors
#12
P Vermersch, O Outteryck, D Ferriby, H Zéphir
BACKGROUND AND PURPOSE: Myelitis is common, related to multiple aetiologies and constitute in some cases a differential diagnosis for spinal cord tumors. Our objective was to review the clinical and paraclinical aspects of the main aetiologies of myelitis. METHODS: These aetiologies will be reviewed based on data not only from the scientific literature but also from our personal experience reported in different cohorts of patients. RESULTS: Multiple sclerosis is the main cause of partial myelitis in young adults...
November 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29098296/manipulation-of-panx1-activity-increases-the-engraftment-of-transplanted-lacrimal-gland-epithelial-progenitor-cells
#13
Liana V Basova, Xin Tang, Takeshi Umasume, Anastasia Gromova, Tatiana Zyrianova, Taisia Shmushkovich, Alexey Wolfson, Dillon Hawley, Driss Zoukhri, Valery I Shestopalov, Helen P Makarenkova
Purpose: Sjögren's syndrome is a systemic chronic autoimmune inflammatory disease that primarily targets the salivary and lacrimal glands (LGs). Currently there is no cure; therefore, cell-based regenerative therapy may be a viable option. LG inflammation is facilitated by extracellular ATP and mediated by the Pannexin-1 (Panx1) membrane channel glycoprotein. We propose that suppression of inflammation through manipulation of Panx1 activity can stimulate epithelial cell progenitor (EPCP) engraftment...
November 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29098081/reduced-expression-of-vamp8-in-lacrimal-gland-affected-by-chronic-graft-versus-host-disease
#14
Masaki Fukui, Yoko Ogawa, Shin Mukai, Mizuka Kamoi, Teru Asato, Yutaka Kawakami, Kazuo Tsubota
Purpose: To investigate whether the SNARE protein vesicle-associated membrane protein 8 (VAMP8) was implicated in the development of chronic ocular graft-versus-host disease (GVHD). Methods: Firstly, the chronic GVHD (cGVHD) and Sjögren's syndrome (SS)-impaired lacrimal gland (LG) tissue sections from humans for diagnostic purpose were evaluated for VAMP8 expression by histopathology and immunohistochemistry. Next, serial changes of tear secretion and VAMP8 expression at both protein and mRNA level of LG in an animal cGVHD model compared with the syngeneic control...
2017: Journal of Ophthalmology
https://www.readbyqxmd.com/read/29092775/cytokines-as-therapeutic-targets-in-primary-sj%C3%A3-gren-syndrome
#15
REVIEW
Soledad Retamozo, Alejandra Flores-Chavez, Marta Consuegra-Fernández, Francisco Lozano, Manuel Ramos-Casals, Pilar Brito-Zerón
Primary Sjögren syndrome (SjS) is a systemic autoimmune disease that may affect 1 in 1000 people (overwhelmingly women) and that can be a serious disease with excess mortality due to severe organ-specific involvements and the development of B cell lymphoma; systemic involvement clearly marks the disease prognosis, and strongly suggests the need for closer follow-up and more robust therapeutic management. Therapy is established according to the organ involved and severity. As a rule, the management of systemic SjS should be organ-specific, with glucocorticoids and immunosuppressive agents limited to potentially-severe involvements; unfortunately, the limited evidence available for these drugs, together with the potential development of serious adverse events, makes solid therapeutic recommendations difficult...
October 29, 2017: Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29078844/risk-of-ischemic-heart-disease-in-patients-with-sj%C3%A3-gren-s-syndrome
#16
Faraz Khan Luni, Sonia Ali Malik, Abdur Rahman Khan, Haris Riaz, Hemindermeet Singh, Douglas Federman, Yusuf Kanjwal, Osama Dasa, Sadik Khuder, Ameer Kabour
BACKGROUND: Ischemic heart disease (IHD) has emerged as a major cause of morbidity and mortality in patients with autoimmune conditions such as systemic lupus erythematosus and rheumatoid arthritis, but the risk of IHD in Sjögren's syndrome (SjS) is unknown. To fill this knowledge gap, we estimated the prevalence and risk of IHD with SjS compared to controls from the general population using the Healthcare Cost and Utilization Project National Inpatient Sample 2011 database. MATERIALS AND METHODS: The Healthcare Cost and Utilization Project administrative longitudinal database contains encounter-level information on inpatient stays, emergency department visits and ambulatory surgery in all U...
October 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29074982/neurofilament-light-protein-in-csf-and-blood-is-associated-with-neurodegeneration-and-disease-severity-in-huntington-s-disease-r6-2-mice
#17
Rana Soylu-Kucharz, Åsa Sandelius, Marie Sjögren, Kaj Blennow, Edward J Wild, Henrik Zetterberg, Maria Björkqvist
There is an unmet need to reliably and non-invasively monitor disease progression in preclinical Huntington's disease (HD) models. As a marker of axonal damage, neurofilament light chain (NfL) has been suggested a marker for neurodegeneration. NfL concentrations in blood and CSF were recently shown to have prognostic value for clinical HD progression and brain atrophy. We therefore hypothesized that CSF and blood NfL concentrations could be useful preclinical HD markers, reflecting underlying pathology. To test our hypothesis we utilized the R6/2 mouse model of HD and measured NfL concentrations in CSF and serum using the ultrasensitive Single molecule array (Simoa) platform...
October 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29072325/patients-with-primary-sj%C3%A3-gren-s-syndrome-have-alterations-in-absolute-quantities-of-specific-peripheral-leukocyte-populations
#18
Richard Davies, Daniel Hammenfors, Brith Bergum, Kjerstin Jakobsen, Magne Solheim, Petra Vogelsang, Johan G Brun, Yenan Bryceson, Roland Jonsson, Silke Appel
An accurate dissection of peripheral blood enumeration is lacking in primary Sjögren's syndrome. The purpose of this study was to quantify different leukocyte populations in peripheral blood of patients with pSS. Numbers of specific leukocyte subsets were determined in 86 pSS patients and 74 healthy donors quantifying 21 distinct subtypes by flow cytometry. Subgroups of pSS patients were stratified based on presence of extraglandular manifestations (EGM) and SSA/SSB autoantibodies. Overall, pSS patients manifested decreased lymphocyte subpopulations compared to healthy donors...
October 26, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29070082/sex-influences-eqtl-effects-of-sle-and-sj%C3%A3-gren-s-syndrome-associated-genetic-polymorphisms
#19
Magdalena Lindén, Jorge I Ramírez Sepúlveda, Tojo James, Gudny Ella Thorlacius, Susanna Brauner, David Gómez-Cabrero, Tomas Olsson, Ingrid Kockum, Marie Wahren-Herlenius
BACKGROUND: Systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) are autoimmune disorders characterized by autoantibodies, dysregulated B cells, and notably high female-to-male incidence ratios. Genome-wide association studies have identified several susceptibility SNPs for both diseases. Many SNPs in the genome are expression quantitative trait loci (eQTLs), with context-dependent effects. Assuming that sex is a biological context, we investigated whether SLE/pSS SNPs act as eQTLs in B cells and used a disease-targeted approach to understand if they display sex-specific effects...
October 25, 2017: Biology of Sex Differences
https://www.readbyqxmd.com/read/29068606/salivary-lipids-a-review
#20
REVIEW
Jan Matczuk, Małgorzata Żendzian-Piotrowska, Mateusz Maciejczyk, Krzysztof Kurek
Saliva is produced by both large and small salivary glands and may be considered one of the most important factors influencing the behavior of oral cavity homeostasis. Secretion of saliva plays an important role in numerous significant biological processes. Saliva facilitates chewing and bolus formation as well as performs protective functions and determines the buffering and antibacterial prosperities of the oral environment. Salivary lipids appear to be a very important component of saliva, as their qualitative and quantitative composition can be changed in various pathological states and human diseases...
September 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
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