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https://www.readbyqxmd.com/read/28730224/histopathological-and-immunohistochemical-profile-in-primary-sj%C3%A3-gren-s-syndrome
#1
Ştefan Cristian Dinescu, Mircea Cătălin ForŢofoiu, Ana Maria Bumbea, Paulina Lucia Ciurea, Cristina Jana Busuioc, Anca Emanuela Muşetescu
Sjögren's syndrome (SS) is an autoimmune disease characterized by hypofunction of the salivary and lachrymal glands. Main clinical features of SS are sicca symptoms, due to the altered glandular function. Also, in advanced stages, bilateral swelling of the parotid glands can be noted, indicative of severe glandular involvement. Phenotypic expression of various mononuclear cells present in the affected tissue offers additional insight into cellular proliferation, survival, migration, antibody secretion and also the potential of forming tertiary lymphoid tissue, i...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28729155/incidence-mortality-and-causes-of-death-in-physician-diagnosed-primary-sj%C3%A3-gren-s-syndrome-in-korea-a-nationwide-population-based-study
#2
Hyun Jung Kim, Kyoung Hoon Kim, Hoo Jae Hann, Seungjin Han, Yuri Kim, Sang Hyuk Lee, Dong Sook Kim, Hyeong Sik Ahn
OBJECTIVES: The objective of this study was to investigate the epidemiological features of primary Sjögren's syndrome (pSS) in Korea at a national level, including the incidence, mortality, and causes of death. METHODS: We used a national, population-based registry database called the Rare Intractable Disease Registration Program from the Health Insurance Review and Assessment Service to obtain pSS patient data for the period between 2010 and 2014. pSS was diagnosed by a physician based on uniform criteria...
March 8, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28728856/achaete-scute-complex-homologue-2-accelerates-the-development-of-sj%C3%A3-gren-s-syndrome-like-disease-in-the-nod-shiltj-mouse
#3
Sung-Min Kim, Jeong-Eun Kwon, Jin-Sil Park, Hyeon-Beom Seo, Kyung-Ah Jung, Young-Mee Moon, Jennifer Lee, Seung-Ki Kwok, Mi-La Cho, Sung-Hwan Park
Achaete-scute complex homologue 2 (Ascl2) has been reported to induce the differentiation and activation of follicular helper T (TFH) cells, which are essential for development of Sjögren's syndrome (SS). This study examined whether Ascl2 plays a role in the development of SS. NOD/ShiLtJ mice were injected with an Ascl2-overexpression vector, and the infiltration of lymphocytes into salivary and lacrimal glands was assessed. The expression of inflammatory cytokines and chemoattractants for T or B cells was measured...
July 17, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28727987/associations-of-autoimmunity-immunodeficiency-lymphomagenesis-and-gut-microbiota-in-mice-with-knockins-for-a-pathogenic-autoantibody
#4
Shweta Jain, Jerrold M Ward, Dong-Mi Shin, Hongsheng Wang, Zohreh Naghashfar, Alexander L Kovalchuk, Herbert C Morse
A number of mouse strains transgenic for B-cell receptors specific for nucleic acids or other autoantigens have been generated to understand how autoreactive B cells are regulated in normal and autoimmune mice. Previous studies of nonautoimmune C57BL/6 mice heterozygous for both the IgH and IgL knockins of the polyreactive autoantibody, 564, produced high levels of autoantibodies in a largely Toll-like receptor 7-dependent manner. Herein, we describe studies of mice homozygous for the knockins that also expressed high levels of autoantibodies but, unlike the heterozygotes, exhibited a high incidence of mature B-cell lymphomas and enhanced susceptibility to bacterial infections...
July 17, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28725949/tubulointerstitial-nephritis-induced-hypophosphatemic-osteomalacia-in-sj%C3%A3-gren-s-syndrome-a-case-report-and-review-of-the-literature
#5
REVIEW
Yan Geng, Youlu Zhao, Zhuoli Zhang
Sjögren's syndrome (SS) is a chronic autoimmune inflammatory disease that typically affects the salivary and lacrimal glands. Renal involvement is relatively uncommon and may precede other complaints. Tubulointestitial nephritis (TIN) is the most common renal involvement in SS. Osteomalacia occurring as the first manifestation of renal tubular disorder due to SS is very rare. We report a 39-year-old male who presented with polydipsia, polyuria, and multiple bone pain. Bone density test showed severe osteoporosis, and laboratory findings suggested hypokalemia, hypophosphatemia, and vitamin D deficiency, which supported the diagnosis of hypophosphatemic osteomalacia...
July 20, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28711146/drug-repositioning-strategies-for-the-identification-of-novel-therapies-for-rheumatic-autoimmune-inflammatory-diseases
#6
REVIEW
Amrie C Grammer, Peter E Lipsky
Rheumatic Autoimmune Inflammatory Diseases such as Sjögren's and lupus lack modern treatments. Less than 5% of drugs approved by the FDA from 2014 to mid-2016 had a RAID indication. Many RAID standard-of-care drugs were repurposed based on serendipitous observations, similarity-of-disease categorization, and/or off-target effects. Recently, drug repurposing has become more intentional, relying on an evolving awareness of molecular underpinnings, as well as a better understanding of drug-target interactions by computational modeling...
August 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/28710538/-manifestations-of-autoimmune-disorders-in-otorhinolaryngology-classical-symptoms-and-diagnostic-approach
#7
B Hofauer, A Chaker, K Thürmel, A Knopf
Manifestations of autoimmune disorders represent a diagnostic challenge. Many autoimmune disorders show typical manifestations in the ear, nose and throat and should be taken into account in the differential diagnosis. Sjogren's syndrome, various vasculitides and sarcoidosis represent the most common autoimmune disorders in otorhinolaryngology, but IgG4-related diseases, relapsing polychondritis and autoimmune disorders of the mucosa are also observed. Autoimmune diseases can lead to sensorineural deficits...
July 14, 2017: HNO
https://www.readbyqxmd.com/read/28705780/international-consensus-what-else-can-we-do-to-improve-diagnosis-and-therapeutic-strategies-in-patients-affected-by-autoimmune-rheumatic-diseases-rheumatoid-arthritis-spondyloarthritides-systemic-sclerosis-systemic-lupus-erythematosus-antiphospholipid-syndrome
#8
REVIEW
Roberto Giacomelli, Antonella Afeltra, Alessia Alunno, Chiara Baldini, Elena Bartoloni-Bocci, Onorina Berardicurti, Francesco Carubbi, Alberto Cauli, Ricard Cervera, Francesco Ciccia, Paola Cipriani, Fabrizio Conti, Salvatore De Vita, Paola Di Benedetto, Andrea Doria, Alexandros A Drosos, Ennio Giulio Favalli, Saviana Gandolfo, Mariele Gatto, Rosa Daniela Grembiale, Vasiliki Liakouli, Rik Lories, Ennio Lubrano, Claudio Lunardi, Domenico Paolo Emanuele Margiotta, Laura Massaro, Pierluigi Meroni, Antonia Minniti, Luca Navarini, Monica Pendolino, Federico Perosa, Jacques-Olivier Pers, Marcella Prete, Roberta Priori, Francesco Puppo, Luca Quartuccio, Amelia Ruffatti, Piero Ruscitti, Barbara Russo, Piercarlo Sarzi-Puttini, Yehuda Shoenfeld, George A Somarakis, Francesca Romana Spinelli, Elisa Tinazzi, Giovanni Triolo, Francesco Ursini, Gabriele Valentini, Guido Valesini, Serena Vettori, Claudio Vitali, Athanasios G Tzioufas
Autoimmune diseases are a complex set of diseases characterized by immune system activation and, although many progresses have been done in the last 15 years, several unmet needs in the management of these patients may be still identified. Recently, a panel of international Experts, divided in different working groups according to their clinical and scientific expertise, were asked to identify, debate and formulate a list of key unmet needs within the field of rheumatology, serving as a roadmap for research as well as support for clinicians...
July 10, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28704602/defective-early-b-cell-tolerance-checkpoints-in-sj%C3%A3-gren-s-syndrome-patients
#9
Salome Glauzy, Joel Sng, Jason Bannock, Jacques-Eric Gottenberg, Anne-Sophie Korganow, Patrice Cacoub, David Saadoun, Eric Meffre
OBJECTIVE: Central and peripheral B cell tolerance checkpoints are defective in many patients with autoimmune diseases, but the functionality of each discrete checkpoint has not been assessed in SS patients. METHODS: Using a PCR-based approach that allows us to clone and express, in vitro, recombinant antibodies produced by single B cells, we tested the reactivity of recombinant antibodies cloned from single CD19(+) CD21(low) CD10(+) IgM(hi) CD27(-) new emigrant/transitional and CD19(+) CD21(+) CD10(-) IgM(+) CD27(-) mature naïve B cells from five SS patients...
July 13, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28704397/impact-of-sjogren-s-syndrome-on-parkinson-s-disease-a-nationwide-case-control-study
#10
Ming-Chi Wu, Xun Xu, Shan-Ming Chen, Yeu-Sheng Tyan, Jeng-Yuan Chiou, Yu-Hsun Wang, Li-Chi Lin, Chyong-Mei Chen, James Cheng-Chung Wei
OBJECTIVE: To investigate whether Sjogren's syndrome would have an influence on the development of Parkinson's disease. METHODS: A population-based case-control study was conducted. Participants consisted of 7716 subjects with newly diagnosed Parkinson's disease and a population of 75129 matched control subjects between 2000 and 2010. We measured the risk of Parkinson's disease in association with Sjogren's syndrome by using adjusted odds ratios. RESULTS: A total of 143 Parkinson's disease subjects (1...
2017: PloS One
https://www.readbyqxmd.com/read/28702966/possible-role-of-helicobacter-pylori-in-diseases-of-dermatological-interest
#11
C Guarneri, J Lotti, M Fioranelli, M G Roccia, T Lotti, F Guarneri
Helicobacter pylori is a gram-negative, flagellate, microaerophilic bacterium identified for the first time about 30 years ago, as a pathogenic factor of gastritis and peptic ulcer. Soon after, it was linked to several gastrointestinal and extra-gastrointestinal diseases (hematological, cardiovascular, neurological, pulmonary and ocular diseases, obesity, diabetes mellitus, growth retardation and extragastric MALT lymphoma). Association and possible cause-effect correlation with H. pylori infection were suggested in diseases of dermatological interest such as chronic urticaria, rosacea, Henoch-Schoenleins purpura, idiopathic thrombocytopenic purpura, cutaneous and oral lichen planus, atopic dermatitis, recurrent aphthous stomatitis, systemic sclerosis, psoriasis, Sjögrens syndrome, Behçet's disease, pruritus, alopecia areata, primary cutaneous marginal zone B-cell lymphomas, vitiligo, chronic prurigo, multiformis, prurigo nodularis, leukocytoclastic vasculitis, prurigo pigmentosa, eczema nummulare, primary cutaneous MALT-type lymphoma, sublamina densa-type linear IgA bullous dermatosis, Sweet's syndrome, cutaneous T-cell pseudolymphoma and pemphigus vulgaris...
July 13, 2017: Journal of Biological Regulators and Homeostatic Agents
https://www.readbyqxmd.com/read/28692793/rare-x-chromosome-abnormalities-in-systemic-lupus-erythematosus-and-sj%C3%A3-gren-s-syndrome
#12
Rohan Sharma, Valerie M Harris, Joshua Cavett, Biji T Kurien, Ke Liu, Kristi A Koelsch, Anum Fayaaz, Kaustubh S Chaudhari, Lida Radfar, David Lewis, Donald U Stone, C Erick Kaufman, Shibo Li, Barbara Segal, Daniel J Wallace, Michael H Weisman, Swamy Venuturupalli, Jennifer A Kelly, Bernardo Pons-Estel, Roland Jonsson, Xianglan Lu, Jacques-Eric Gottenberg, Juan-Manuel Anaya, Deborah S Cunninghame-Graham, Andrew J W Huang, Michael T Brennan, Pamela Hughes, Ilias Alevizos, Corinne Miceli-Richard, Edward C Keystone, Vivian P Bykerk, Gideon Hirschfield, Gang Xie, Gunnel Nordmark, Sara Magnusson Bucher, Per Eriksson, Roald Omdal, Nelson L Rhodus, Maureen Rischmueller, Michael Rohrer, Marie Wahren-Herlenius, Torsten Witte, Marta Alarcon-Riquelme, Xavier Mariette, Christopher J Lessard, John B Harley, Wan-Fai Ng, Astrid Rasmussen, Kathy L Sivils, R Hal Scofield
BACKGROUND: Sjögren's syndrome and systemic lupus erythematosus (SLE) are related by clinical and serological manifestations as well as genetic risks. Both diseases are more commonly found in women compared to men at a ratio of about 10 to 1. Common X chromosome aneuploidies, 47,XXY and 47,XXX, are enriched among men and women, respectively, in either disease suggesting a dose effect on the X chromosome. METHODS: We examined cohorts of Sjögren's syndrome or SLE patients with intensity plots of X chromosome single nucleotide polymorphism (SNP) alleles along with karyotype of selected subjects...
July 10, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28692451/the-value-of-musculoskeletal-ultrasound-in-geriatric-care-and-rehabilitation
#13
Büşra Can, Murat Kara, Özgür Kara, Zekeriya Ülger, Walter Frontera, Levent Özçakar
The WHO reports that one of the major chronic conditions affecting the elderly worldwide is musculoskeletal disorders that are associated with long-term pain and disability. Considering the healthcare needs of the elderly (i.e. comprehensive, accessible, efficient) and the advantages of ultrasound (US) use (patient-friendly, convenient, cost-effective, and does not require exposure to radiation or magnetic fields), there seems to be a 'gap' in the actual clinical practice. In this paper, we aimed to highlight the potential value of US imaging in the management of the elderly with a wide spectrum of musculoskeletal conditions (degenerative/rheumatic joint diseases, falls/trauma, nursing care, peripheral nerve problems, sarcopenia, and interventions)...
July 7, 2017: International Journal of Rehabilitation Research. Revue Internationale de Recherches de Réadaptation
https://www.readbyqxmd.com/read/28690324/proteasome-inhibition-suppresses-th17-cell-generation-and-ameliorates-autoimmune-development-in-experimental-sj%C3%A3-gren-s-syndrome
#14
Fan Xiao, Xiang Lin, Jie Tian, Xiaohui Wang, Qian Chen, Ke Rui, Jie Ma, Shengjun Wang, Qingwen Wang, Xiaoqi Wang, Dongzhou Liu, Lingyun Sun, Liwei Lu
Immunoproteasome activation in immune cells is involved in the modulation of immune responses. Increasing evidence indicates that proteasome inhibitors show beneficial effects in treating autoimmune diseases, but it remains unclear whether proteasome inhibition is an effective approach for suppressing autoimmune development in Sjögren's syndrome (SS). Our previous work has demonstrated a critical role for Th17 cells in the development of experimental SS (ESS) in mice. In this study, we detected high levels of low-molecular-weight protein 7 (LMP7), a key subunit of the immunoproteasome, in Th17 cells from ESS mice...
July 10, 2017: Cellular & Molecular Immunology
https://www.readbyqxmd.com/read/28690029/high-levels-of-soluble-gpr56-adgrg1-are-associated-with-positive-rheumatoid-factor-and-elevated-tumor-necrosis-factor-in-patients-with-rheumatoid-arthritis
#15
Wen-Yi Tseng, Yeong-Jian Jan Wu, Tai-Yun Yang, Nien-Yi Chiang, Wen-Pin Tsai, Siamon Gordon, Gin-Wen Chang, Chang-Fu Kuo, Shue-Fen Luo, Hsi-Hsien Lin
BACKGROUND: GPR56/ADGRG1 is a member of the adhesion-class G protein-coupled receptor (aGPCR) family important in brain development, oncogenesis and tumor metastasis. Like other aGPCRs, GPR56 is cleaved at the GPCR proteolysis site (GPS) motif into an N-terminal fragment (NTF) and a C-terminal fragment (CTF). Existence of soluble GPR56 (sGPR56) has been shown in vitro, however the underlying mechanism and its pathophysiologic role remains undetermined. OBJECTIVE: To assess the presence of sGPR56 in human serum using ELISA assay and compare the serum sGPR56 levels among patients of various chronic inflammatory diseases and healthy subjects...
June 28, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/28687351/prevalence-of-anti-nt5c1a-antibodies-in-japanese-patients-with-autoimmune-rheumatic-diseases-in-comparison-with-other-patient-cohorts
#16
Yoshinao Muro, Hirotaka Nakanishi, Masahisa Katsuno, Michihiro Kono, Masashi Akiyama
BACKGROUND: Sporadic inclusion body myositis (sIBM) is usually classified as an idiopathic inflammatory myopathies. Although the diagnosis of sIBM is sometimes challenging, recent studies have shown that the autoantibodies against cytosolic 5'-nucleotidase 1A (NT5C1A) are the possible diagnostic biomarker for sIBM. Few reports have shown the frequencies of anti-NT5C1A antibodies in systemic autoimmune rheumatic diseases (SARDs) using large cohorts of SARDs. METHODS: Serum samples obtained from 314 patients including dermatomyositis (DM) (n=144), systemic lupus erythematosus (SLE) (n=50), systemic sclerosis (SSc) (n=50), Sjögren's syndrome (SS) (n=50), polymyositis (PM) (n=10) and mixed connective tissue disease (n=10), and healthy controls (n=42) in addition to 10 patients with typical sIBM were analysed for the presence of autoantibodies using full-length recombinant NT5C1A ELISA...
July 4, 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28686532/management-of-hydrocephalus-associated-with-autoimmune-diseases-a-series-of-19-cases
#17
Baitao Ma, Hao Wu, Hexiang Yin, Jianbo Chang, Li Wang, Renzhi Wang, Wenbin Ma, Yongning Li, Jian Guan, Jinjing Liu, Junji Wei
OBJECTIVES: To analyze the diagnosis and treatment of hydrocephalus associated with autoimmune diseases and to explore the possible mechanism of hydrocephalus in these patients. METHODS: A retrospective case series study was conducted at Peking Union Medical College Hospital, Beijing, China. Files were retrieved from the hospital archives by screening records from Jan 1990 to Jan 2016. Medical records were screened for data regarding (1) the number of patients diagnosed with hydrocephalus associated with autoimmune diseases, (2) the clinical manifestation of hydrocephalus associated with autoimmune disease, and (3) the outcomes of these patients treated with medication or ventriculoperitoneal shunt (VPS)...
July 7, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28686485/impaired-salivary-gland-activity-in-patients-with-autoimmune-polyendocrine-syndrome-type-i
#18
Bergithe E Oftedal, Mihaela Cuida Marthinussen, Martina M Erichsen, Maria K Tveitarås, Anja Kjellesvik-Kristiansen, Daniel Hammenfors, Malin V Jonsson, Kai Kisand, Roland Jonsson, Anette S B Wolff
Autoimmune polyendocrine syndrome type I (APS-I) is a severe disease caused by mutations in the autoimmune regulator (AIRE) gene. We hypothesized that salivary gland dysfunction could be a possible unexplored component of these patients and here aimed to investigate salivary and lachrymal symptoms in the Norwegian cohort of APS-I patients (N = 41) and the aetiology behind it. Sicca symptoms and possible corresponding underlying factors were assessed by subjective reports combined with objective measures of saliva and tear flow, serological testing, immune fluorescence microscopy, ultrasonography and searching for putative autoantibodies in the salivary glands...
July 7, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28684154/coexistence-of-multiple-sclerosis-and-ankylosing-spondylitis-report-of-four-cases-from-russia-and-review-of-the-literature
#19
Vera Fominykh, Tatyana Shevtsova, Narine Arzumanian, Lev Brylev
Multiple sclerosis is a chronic demyelinating disorder of the central nervous system. There are many cases of multiple sclerosis - like syndrome and demyelinating disorders in systemic lupus erythematosus, Sjogren disease, Behcet disease and other autoimmune conditions. Coexistence of ankylosing spondylitis and multiple sclerosis usually is rare but in this article we report 4 Russian patients with concomitant multiple sclerosis and ankylosing spondylitis diseases. None of these patients received anti-tumor necrosis factor alpha therapy prior to diagnosis of multiple sclerosis...
July 3, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28678161/t-helper-17-cells-in-primary-sj%C3%A3-gren-s-syndrome
#20
REVIEW
Kiyoshi Matsui, Hajime Sano
Primary Sjögren's syndrome is an autoimmune disease characterized by diffuse infiltration of lymphocytes into exocrine glands and other tissues. The infiltrating lymphocytes have been identified as subsets of B cells and T cells, including T helper 17 cells, T regulatory cells and follicular helper T cells. The role of these cells in the development of the syndrome is now known, as is their impact on the production of proinflammatory cytokines such as IL-6, IL-17, IL-22 and IL-23. In particular, experimental animal models and patients suggest that a shift in Th17/Treg balance toward the proinflammatory Th17 axis exacerbates primary Sjögren's syndrome and other autoimmune disorders...
July 5, 2017: Journal of Clinical Medicine
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