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Sjogrens Syndrome

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https://www.readbyqxmd.com/read/29166262/antiphosphatidylserine-prothrombin-antibodies-aps-pt-as-potential-diagnostic-markers-and-risk-predictors-of-venous-thrombosis-and-obstetric-complications-in-antiphospholipid-syndrome
#1
Hui Shi, Hui Zheng, Yu-Feng Yin, Qiong-Yi Hu, Jia-Lin Teng, Yue Sun, Hong-Lei Liu, Xiao-Bing Cheng, Jun-Na Ye, Yu-Tong Su, Xin-Yao Wu, Jin-Feng Zhou, Gary L Norman, Hui-Yun Gong, Xin-Ming Shi, Yi-Bing Peng, Xue-Feng Wang, Cheng-De Yang
BACKGROUND: The aim of the study was to determine the prevalence and clinical associations of antiphosphatidylserine/prothrombin antibodies (aPS/PT) with thrombosis and pregnancy loss in Chinese patients with antiphospholipid syndrome (APS) and seronegative APS (SNAPS). METHODS: One hundred and eighty six Chinese patients with APS (67 primary, 119 secondary), 48 with SNAPS, 176 disease controls (79 systemic lupus erythematosus [SLE], 29 Sjogren's syndrome [SS], 30 ankylosing spondylitis [AS], 38 rheumatoid arthritis [RA]) and 90 healthy donors were examined...
November 22, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/29160038/endoplasmic-reticulum-in-health-and-disease-the-12th-international-calreticulin-workshop-delphi-greece
#2
REVIEW
Aristidis S Charonis, Marek Michalak, Jody Groenendyk, Luis B Agellon
Starting from 1994, every 2 years, an international workshop is organized focused on calreticulin and other endoplasmic reticulum chaperones. In 2017, the workshop took place at Delphi Greece. Participants from North and South America, Europe, Asia and Australia presented their recent data and discussed them extensively with their colleagues. Presentations dealt with structural aspects of calreticulin and calnexin, the role of Ca(2+) in cellular signalling and in autophagy, the endoplasmic reticulum stress and the unfolded protein response, the role of calreticulin in immune responses...
November 21, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29158045/-primary-sj%C3%A3-gren-syndrome-in-a-child
#3
I Majdoub, S Kallel, M Hsairi, M Snoussi, M Charfi, A Ben Halima, A Frikha, M Weli, F Safi, L Gargouri, N Ben Halima, A Mahfoudh, A Ghorbel, Z Bahloul
Sjögren syndrome is uncommon in children and occurs most often in association with autoimmune diseases (secondary Sjögren syndrome). We describe the clinical and biological features of a 7-year-old girl with primary Sjögren syndrome revealed by recurrent parotiditis. CASE REPORT: A 7-year-old girl was referred for investigation of multiple episodes of parotid swelling since age 4 years, without systemic symptoms. The examination was unremarkable except for enlarged and painless parotid glands...
November 17, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29154979/small-fiber-neuropathy-diagnosis-causes-and-treatment
#4
Damien Sène
Small fiber neuropathy, which affects the sensory A≏ and C fibers, is now a major diagnostic and therapeutic challenge. Nearly 7% of the general population have chronic neuropathic pain responsible for severe quality-of-life impairments. Awareness must therefore be raised among clinicians of the somatosensory and autonomic symptoms that can reveal small fiber neuropathy, appropriate diagnostic investigations, most common causes, and best treatment options for each patient profile. To help achieve this goal, the present review article discusses the clinical presentation of neuropathic pain and paresthesia and/or autonomic dysfunction due to involvement of nerves supplying exocrine glands and smooth muscle; normal findings from standard electrophysiological investigations; most informative diagnostic tests (epidermal nerve fiber density in a skin biopsy, laser-evoked potentials, heat- and cold-detection thresholds, electrochemical skin conductance); main causes, which consist chiefly of metabolic diseases (diabetes mellitus, glucose intolerance), dysimmunity syndromes (Sjögren's syndrome, sarcoidosis, monoclonal gammopathy), and genetic abnormalities (familial amyloidosis due to a transthyretin mutation, Fabry disease, sodium channel diseases); and the available symptomatic and etiological treatments...
November 15, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29152879/y-rna-derived-small-rnas-in-sj%C3%A3-gren-s-syndrome-candidate-biomarkers
#5
Jayakanthan Kabeerdoss, Pulukool Sandhya, Debashish Danda
Anti-Ro and anti-La antibodies are important in pathogenesis and diagnosis of Sjögren's syndrome (SS). Ro60, Ro52 and La are RNA binding proteins of Y RNA, which were discovered more than three decades ago. Significance of Y RNA is not appreciated as much as Ro and La in SS. It can be hypothesised that 5'-YsRNA, short fragment derived from Y RNA may be recognized by TLR7 in pDC, which induces type I interferon signature in SS. New genomics tools, namely RNA seq, enables assay of 5'-YsRNA in blood. 5'-YsRNA has the potential to be a novel biomarker of SS...
November 19, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29151514/a-case-of-dermatomyositis-complicated-by-digital-ischemia-and-lung-adenocarcinoma-in-a-patient-with-positive-anti-signal-recognition-particle-antibodies
#6
Takashi Nawata, Makoto Kubo, Hitomi Mitsui, Keiji Oishi, Masatoshi Omoto, Takashi Kanda, Masafumi Yano
A 58-year-old Japanese woman was diagnosed with anti-signal recognition particle (SRP)-positive dermatomyositis associated with Sjögren's syndrome, rheumatoid arthritis and lung adenocarcinoma. She presented with cutaneous lesions, including ulceration of her right middle finger. Tissue specimens obtained from her right deltoid muscle were positive for CD4(+) T-cell infiltration and the sarcolemma showed the upregulation of major histocompatibility complex (MHC) class I antigens. The present case suggests that overlapping autoimmune diseases or complications of malignancy may result in an atypical clinical presentations and histological findings in patients with anti-SRP antibody-positive dermatomyositis...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29151171/clinical-and-serological-associations-of-anti-ribosomal-p0-protein-antibodies-in-systemic-lupus-erythematosus
#7
Yong-Jun Mei, Peng Wang, Chao Jiang, Tao Wang, Lin-Jie Chen, Zhi-Jun Li, Hai-Feng Pan
The purpose of this study is to investigate the clinical and serological associations of anti-ribosomal P0 protein antibodies (anti-Rib-P0) in patients with systemic lupus erythematosus (SLE). The sera of 470 patients with SLE and 124 patients with primary Sjogren's Syndrome (pSS) were collected. Line immunoassay (LIA) was used to detect anti-Rib-P0 and other related antibodies. A complete laboratory evaluation and clinical examination were also performed in each SLE patient. The prevalence of anti-Rib-P0 in SLE patients was significantly higher than that in pSS patients (35...
November 18, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29149928/rheumatic-manifestations-in-autoimmune-liver-disease
#8
REVIEW
Carlo Selmi, Elena Generali, Merrill Eric Gershwin
Autoimmune liver diseases coexist with rheumatic disorders in approximately 30% of cases and may also share pathogenic mechanisms. Autoimmune liver diseases result from an immune-mediated injury of different tissues, with autoimmune hepatitis (AIH) targeting hepatocytes, and primary biliary cholangitis (PBC) and primary sclerosing cholangitis targeting cholangiocytes. Sjogren syndrome is diagnosed in 7% of AIH cases and serologic autoimmunity profiles are a common laboratory abnormality, particularly in the case of serum antimitochondrial (PBC) or anti-liver kidney microsomal antibodies (AIH)...
February 2018: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29149922/gastrointestinal-and-hepatic-disease-in-sjogren-syndrome
#9
REVIEW
Yevgeniy Popov, Karen Salomon-Escoto
Sjogren syndrome (SS) is a lymphocyte-mediated, infiltrative autoimmune disorder characterized by destruction of exocrine glands leading to secretory dysfunction. The typical manifestations include xerostomia and xerophthalmia; however, extensive gastrointestinal involvement is increasingly being recognized, emphasizing the variable and systemic nature of SS.
February 2018: Rheumatic Diseases Clinics of North America
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
#10
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
#11
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/29147924/monoclonal-gammopathy-of-renal-significance-presenting-as-monotypic-plasma-cell-interstitial-nephritis-in-two-patients-with-sj%C3%A3-gren-s-syndrome
#12
Arzu Saglam, Seda Balaban, Tolga Yıldırım, Yunus Erdem, Aysegul Uner, Yahya Büyükaşık
No abstract text is available yet for this article.
November 16, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29141312/-clinical-manifestation-and-gene-analyses-of-15-patients-with-intellectual-disability-or-developmental-delay-complicated-with-congenital-nystagmus
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
EDITORIAL
Jeanie C McGee, Alireza Minagar
No abstract text is available yet for this article.
November 1, 2017: Journal of the Neurological Sciences
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