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Sjogren's Syndrome

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https://www.readbyqxmd.com/read/29141312/-clinical-manifestation-and-gene-analyses-of-15-patients-with-intellectual-disability-or-developmental-delay-complicated-with-congenital-nystagmus
#1
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/29138979/autoantibodies-binding-to-stathmin-4-new-marker-for-polyneuropathy-in-primary-sj%C3%A3-gren-s-syndrome
#2
Sabrina Duda, Torsten Witte, Martin Stangel, Jan Adams, Reinhold E Schmidt, Niklas T Baerlecken
No abstract text is available yet for this article.
November 14, 2017: Immunologic Research
https://www.readbyqxmd.com/read/29138598/familial-aggregation-of-myasthenia-gravis-in-affected-families-a-population-based-study
#3
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
#4
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
#5
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
#6
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/29123309/oral-dryness-and-sj%C3%A3-gren-s-an-update
#7
P J Shirlaw, A Khan
Oral dryness is a very common condition presenting to a general dental practitioner or hospital specialist. The most common cause of oral dryness is drug related, however, patients with Sjögren's syndrome, a multisystem autoimmune condition, may present to their dentist rather than their GP complaining of dry mouth and dry eyes. This update article explores the causes of oral dryness and how to manage it. The update on Sjögren's syndrome explains the latest relevant diagnostic criteria, presenting signs, symptoms, investigations and management principles...
November 10, 2017: British Dental Journal
https://www.readbyqxmd.com/read/29122296/a-case-of-neuromyelitis-optica-spectrum-disorder-presenting-with-undiagnosed-sjogren-s-syndrome-and-a-single-atypical-tumefactive-lesion-a-diagnostic-challenge
#8
EDITORIAL
Jeanie C McGee, Alireza Minagar
No abstract text is available yet for this article.
November 1, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29119483/low-level-laser-therapy-for-xerostomia-in-primary-sj%C3%A3-gren-s-syndrome-a-randomized-trial
#9
Tania Fidelix, Adriano Czapkowski, Sergio Azjen, Adagmar Andriolo, Pedro Horvath Neto, Virgínia Trevisani
To evaluate the effectiveness of low-level laser therapy (LLLT) in the treatment of xerostomia in primary Sjögren's syndrome (SS), a randomized clinical trial of patients with dry mouth symptoms associated with primary SS receiving care at a university hospital was conducted. Sixty-six patients were randomly assigned with a 1:1 allocation ratio to receive LLLT (laser group, n = 33) or placebo treatment (placebo group, n = 33). Patients in the laser group received LLLT twice a week for 6 weeks, for a total of 12 treatment sessions...
November 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29118759/b-cells-are-indispensable-for-a-novel-mouse-model-of-primary-sj%C3%A3-gren-s-syndrome
#10
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/29117464/salivary-scintigraphy-for-sj%C3%A3-gren-s-syndrome-in-patients-with-xerostomia-a-retrospective-study
#11
Marion Nadal, Maeva Levy, Aquib Bakhsh, Aline Joly, Annabel Maruani, Loïc Vaillant, Benoît Erra, Mahtab Samimi
OBJECTIVES: The value of salivary gland scintigraphy in the diagnosis of Sjögren's syndrome remains controversial. The primary aim of this study was to estimate the diagnostic accuracy of salivary gland scintigraphy in the diagnosis of Sjögren's syndrome among 237 patients with xerostomia. METHODS: We retrospectively compared eight scintigraphy parameters between 106 Sjögren patients and 131 non-Sjögren patients. RESULTS: Seven of the eight parameters were significantly decreased in Sjögren patients; however their diagnostic accuracy was low, with AUCs ranging from 0...
November 8, 2017: Oral Diseases
https://www.readbyqxmd.com/read/29116459/neonatal-systemic-lupus-erythematosus-syndrome-a-comprehensive-review
#12
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
#13
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/29113317/hydroxychloroquine-and-risk-of-cancer-in-patients-with-primary-sj%C3%A3-gren-syndrome-propensity-score-matched-landmark-analysis
#14
Yao-Fan Fang, Yen-Fu Chen, Ting-Ting Chung, Lai-Chu See, Kuang-Hui Yu, Shue-Fen Luo, Chang-Fu Kuo, Jenn-Haung Lai
Hydroxychloroquine inhibits systemic inflammation and autophagy and may thus have antineoplastic effects [1]. We investigated the effect of hydroxychloroquine on cancer risk in patients with primary Sjögren syndrome(pSS). We used the Taiwan National Health Insurance Database to compare cancer incidence between incident pSS patients with or without at least 6-month hydroxychloroquine use within a 1- or 3-year period. Propensity score matched landmark analysis was used. We included 4194 alive patients without cancer 1 year after pSS diagnosis from 2000 through 2005...
October 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/29109527/the-rna-binding-protein-la-ss-b-promotes-rig-i-mediated-type-i-and-type-iii-ifn-responses-following-sendai-viral-infection
#15
Rebecca Mahony, Lindsay Broadbent, Jacen S Maier-Moore, Ultan F Power, Caroline A Jefferies
La/SS-B (or La) is a 48 kDa RNA-binding protein and an autoantigen in autoimmune disorders such as systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). La involvement in regulating the type I interferon (IFN) response is controversial - acting through both positive and negative regulatory mechanisms; inhibiting the IFN response and enhancing viral growth, or directly inhibiting viral replication. We therefore sought to clarify how La regulates IFN production in response to viral infection. ShRNA knockdown of La in HEK 293 T cells increased Sendai virus infection efficiency, decreased IFN-β, IFN-λ1, and interferon-stimulated chemokine gene expression...
November 6, 2017: Scientific Reports
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
#16
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
#17
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
#18
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
#19
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/29093421/hypertrophic-pachymeningitis-in-sj%C3%A3-gren-s-syndrome
#20
Yumiko Nakano, Masayoshi Yamamoto, Kenichi Komatsu, Masato Yagita, Masaaki Fujita
Although central nervous system manifestations seem common in primary Sjögren's syndrome, hypertrophic pachymeningitis is rare. We herein describe a case of Sjögren's syndrome that was associated with hypertrophic pachymeningitis. Sjögren's syndrome should be considered as a cause of hypertrophic pachymeningitis.
November 1, 2017: Internal Medicine
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