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Sjogren syndrome

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https://www.readbyqxmd.com/read/29667944/proximal-muscle-weakness-with-overlying-hypokalemic-periodic-paralysis-in-sj%C3%A3-gren-syndrome-report-of-6-cases
#1
Vimal K Paliwal, Anmol Singh Rai, Surendra Kumar, Ritu Verma, Vikas Agarwal
No abstract text is available yet for this article.
April 5, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29666604/the-association-of-sj%C3%A3-gren-syndrome-and-autoimmune-thyroid-disorders
#2
REVIEW
Chiara Baldini, Francesco Ferro, Marta Mosca, Poupak Fallahi, Alessandro Antonelli
Sjögren's syndrome (SS) and autoimmune thyroid diseases (AITD) may frequently coexist in clinical practice, resulting in a complex overlapping disorder that represents a particular example of the expression of heterogeneity in patients with autoimmune disorders. Objective of this review was to describe the prevalence of the SS-AITD association in the most recent literature, exploring in particular to what extent the presence of AITD might influence the clinical expression of SS and vice versa . Moreover, we summarized some of the proposed genetic, biologic, and molecular mechanisms implied in the pathogenesis of AITD-SS association...
2018: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/29666155/sil1-the-er-hsp70-co-chaperone-plays-a-critical-role-in-maintaining-skeletal-muscle-proteostasis-and-physiology
#3
Viraj P Ichhaporia, Jieun Kim, Kanisha Kavdia, Peter Vogel, Linda Horner, Sharon Frase, Linda M Hendershot
Mutations in SIL1 , a co-factor for the endoplasmic reticular (ER) chaperone BiP, cause Marinesco-Sjögren syndrome (MSS), an autosomal recessive disorder. Using a mouse model, we characterized molecular aspects of the progressive myopathy associated with MSS. Proteomic-profiling of quadriceps at the onset of myopathy revealed that SIL1 deficiency affected multiple pathways critical to muscle physiology. We observed an increase in ER chaperones prior to the onset of muscle weakness, which was complemented by up-regulation of multiple components of cellular protein degradation pathways...
April 9, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29665238/cp-25-alleviates-experimental-sj%C3%A3-gren-s-syndrome-features-in-nod-ltj-mice-and-modulates-t-lymphocyte-subsets
#4
Fang Gu, Shixia Xu, Pengying Zhang, Xiaoyun Chen, Yujing Wu, Chun Wang, Mei Gao, Min Si, Xinming Wang, Korner Heinrich, Huaxun Wu, Wei Wei
Primary Sjögren's syndrome (pSS) is a chronic inflammatory autoimmune illness of the moisture-producing glands such as salivary glands that is characterized by various immune abnormalities. The aetiology of pSS remains unclear and there is no curative agent. In this study, we investigated the putative therapeutic effects on a NOD/Ltj mouse model of Sjögren's syndrome-like disorders of an ester derivative of paeoniflorin, Paeoniflorin-6'O-benzene (termed CP-25). Our study showed that CP-25 alleviated effectively clinical manifestations in NOD/Ltj mice resulting for example in increased salivary flow and reduced histopathological scores...
April 17, 2018: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29663717/salivary-gland-stem-cells-a-review-of-development-regeneration-and-cancer
#5
REVIEW
Elaine Emmerson, Sarah M Knox
Salivary glands are responsible for maintaining the health of the oral cavity and are routinely damaged by therapeutic radiation for head and neck cancer as well as by autoimmune diseases such as Sjögren's syndrome. Regenerative approaches based on the reactivation of endogenous stem cells or the transplant of exogenous stem cells hold substantial promise in restoring the structure and function of these organs to improve patient quality of life. However, these approaches have been hampered by a lack of knowledge on the identity of salivary stem cell populations and their regulators...
April 16, 2018: Genesis: the Journal of Genetics and Development
https://www.readbyqxmd.com/read/29663342/associations-of-chronic-urticaria-with-atopic-and-autoimmune-comorbidities-a-nationwide-population-based-study
#6
Hsien-Yi Chiu, Chih-Hsin Muo, Fung-Chang Sung
BACKGROUND: Most cases of chronic urticaria (CU) are idiopathic. Circumstantial evidence suggests that some CU cases have an autoimmune pathogenesis. Previous research indicates that a substantial percentage of patients with CU have an atopic background. OBJECTIVES: This study aims to examine the association between CU, and atopic and autoimmune diseases. METHODS: This population-based retrospective cohort study identified 9,332 patients with CU and 37,328 controls matched for age, sex, and number of dermatological clinic visits from the Taiwan National Health Insurance Research Database for 2004-2009...
April 16, 2018: International Journal of Dermatology
https://www.readbyqxmd.com/read/29663334/the-rheumatic-disease-associated-fam167a-blk-locus-encodes-diora-1-a-novel-disordered-protein-highly-expressed-in-bronchial-epithelium-and-alveolar-macrophages
#7
Lara Mentlein, Gudny Ella Thorlacius, Lauro Meneghel, Lara A Aqrawi, Jorge I Ramírez Sepúlveda, Johan Grunewald, Alexander Espinosa, Marie Wahren-Herlenius
OBJECTIVES: Triggering of autoimmunity that leads to rheumatic disease has been suggested to depend on gene-environment interactions occurring in epithelial barriers and associated immune cells. Genetic studies have identified associations of the FAM167A-BLK locus with rheumatoid arthritis, SLE, and Sjögren's syndrome. While BLK (B lymphocyte kinase) has a well-established role in B cells, family with sequence similarity 167 member A (FAM167A) and its gene family remain uncharacterized...
April 17, 2018: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/29662487/acquisition-of-n-glycosylation-sites-in-immunoglobulin-heavy-chain-genes-during-local-expansion-in-parotid-salivary-glands-of-primary-sj%C3%A3-gren-patients
#8
Annie Visser, Marieke E Doorenspleet, Niek de Vries, Fred K L Spijkervet, Arjan Vissink, Richard J Bende, Hendrika Bootsma, Frans G M Kroese, Nicolaas A Bos
Previous studies revealed high incidence of acquired N-glycosylation sites acquired N-glycosylation sites in RNA transcripts encoding immunoglobulin heavy variable region (IGHV) 3 genes from parotid glands of primary Sjögren's syndrome (pSS) patients. In this study, next generation sequencing was used to study the extent of ac-Nglycs among clonally expanded cells from all IGVH families in the salivary glands of pSS patients. RNA was isolated from parotid gland biopsies of five pSS patients and five non-pSS sicca controls...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29657585/deterioration-in-saliva-quality-in-patients-with-sj%C3%A3-gren-s-syndrome-impact-of-decrease-in-salivary-epidermal-growth-factor-on-the-severity-of-intraoral-manifestations
#9
REVIEW
Naoto Azuma, Yoshinori Katada, Hajime Sano
Background: Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration of the exocrine glands, especially the salivary and lacrimal glands. As a result of salivary gland dysfunction, most patients with SS have xerostomia related to a reduced salivary flow rate. In addition to the discomfort due to xerostomia, dry mouth can cause various intraoral manifestations such as refractory stomatitis, ulcer, and atrophic changes in the oral mucosa and tongue, and the patient's quality of life (QoL) is severely impaired...
2018: Inflammation and Regeneration
https://www.readbyqxmd.com/read/29657352/experience-of-sleep-disruption-in-primary-sj%C3%A3-gren-s-syndrome-a-focus-group-study
#10
Katie L Hackett, Vincent Deary, Katherine Ho Deane, Julia L Newton, Wan-Fai Ng, Tim Rapley
Introduction: Primary Sjögren's syndrome is the third most common systemic autoimmune rheumatic disease, following rheumatoid arthritis and systemic lupus erythematosus, and results in dryness, fatigue, discomfort and sleep disturbances. Sleep is relatively unexplored in primary Sjögren's syndrome. We investigated the experiences of sleep disturbances from the viewpoint of primary Sjögren's syndrome patients and their partners and explored the acceptability of cognitive behavioural therapy for insomnia...
April 2018: British Journal of Occupational Therapy
https://www.readbyqxmd.com/read/29657223/distal-renal-tubular-acidosis-in-sj%C3%A3-gren-s-syndrome
#11
Ram Narayan, Mansoor C Abdulla, Jemshad Alungal, N C Krishnadas
Interstitial nephritis and immune complex-mediated glomerulonephritis are the two common renal manifestations of primary Sjögren's syndrome (SS). Here, we discuss three cases of primary SS where presenting manifestation was distal renal tubular acidosis. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with distal tubular acidosis or recurrent hypokalemic periodic paralysis as treatment of primary disease improves the outcome of illness.
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29657217/systemic-lupus-erythematosus-with-sj%C3%A3-gren-s-syndrome-and-renal-tubular-acidosis-presenting-as-nephrogenic-diabetes-insipidus
#12
Ashaq Hussain Parrey, Fayaz Ahmad, Mushtaq Ahmad, Adil Basher
Systemic lupus erythematosus (SLE) presenting as diabetes insipidus (DI) is a rare association; there is a case report of neurogenic DI in patients of SLE; however, SLE and nephrogenic DI has not been reported in literature. We present a case of SLE presenting as nephrogenic DI. We report a case who presented with DI (nephrogenic) and fulfilled criteria for SLE and Sjögren's syndrome with renal tubular acidosis.
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29656524/the-prevalence-of-meibomian-gland-dysfunction-tear-film-and-ocular-surface-parameters-in-an-austrian-dry-eye-clinic-population
#13
Dieter Franz Rabensteiner, Haleh Aminfar, Ingrid Boldin, Gerold Schwantzer, Jutta Horwath-Winter
PURPOSE: The purpose of this study was to assess the prevalence of meibomian gland dysfunction (MGD) and its association with tear film and ocular surface parameters in an Austrian clinical population of dry eye patients. METHODS: The records of 1372 consecutive patients from a dry eye unit were analysed retrospectively. Symptoms and objective tear film and ocular surface parameters were evaluated. Patients were classified into pure MGD, pure aqueous tear deficiency (ATD), MGD combined with ATD, pure anterior blepharitis (AB), Sjogren's syndrome (SS) without MGD and SS together with MGD...
April 15, 2018: Acta Ophthalmologica
https://www.readbyqxmd.com/read/29655640/how-are-ocular-signs-and-symptoms-of-dry-eye-associated-with-depression-in-women-with-and-without-sj%C3%A3-gren-s-syndrome
#14
John A Gonzales, Annie Chou, Jennifer R Rose-Nussbaumer, Vatinee Y Bunya, Lindsey A Criswell, Caroline H Shiboski, Thomas M Lietman
PURPOSE: To determine whether ocular phenotypic features of keratoconjunctivitis sicca (KCS) and/or participant-reported symptoms of dry eye disease are associated with depression in women participants enrolled in the Sjögren's International Collaborative Clinical Alliance (SICCA). DESIGN: Cross-sectional study. METHODS: Women enrolled in the SICCA registry from 9 international research sites. Participants met at least one of five inclusion criteria for registry enrollment (including complaints of dry eyes or dry mouth, a previous diagnosis of Sjögren's syndrome (SS), abnormal serology (positive anti-Sjögren's syndrome-related antigen A and/or B (anti-SSA and/or anti-SSB), or elevated anti-nuclear antibody and rheumatoid factor), bilateral parotid gland enlargement, or multiple dental caries)...
April 12, 2018: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/29652832/sympathetic-nerve-hyperactivity-in-the-spleen-causal-for-nonpathogenic-driven-chronic-immune-mediated-inflammatory-diseases-imids
#15
REVIEW
Denise L Bellinger, Dianne Lorton
Immune-Mediated Inflammatory Diseases (IMIDs) is a descriptive term coined for an eclectic group of diseases or conditions that share common inflammatory pathways, and for which there is no definitive etiology. IMIDs affect the elderly most severely, with many older individuals having two or more IMIDs. These diseases include, but are not limited to, type-1 diabetes, obesity, hypertension, chronic pulmonary disease, coronary heart disease, inflammatory bowel disease, and autoimmunity, such as rheumatoid arthritis (RA), Sjőgren's syndrome, systemic lupus erythematosus, psoriasis, psoriatic arthritis, and multiple sclerosis...
April 13, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29649576/epigenetic-alterations-in-primary-sj%C3%A3-gren-s-syndrome-an-overview
#16
Juliana Imgenberg-Kreuz, Johanna K Sandling, Gunnel Nordmark
Primary Sjögren's syndrome (pSS) is a chronic autoimmune rheumatic disease characterized by inflammation of exocrine glands, mainly salivary and lacrimal glands. In addition, pSS may affect multiple other organs resulting in systemic manifestations. Although the precise etiology of pSS remains elusive, pSS is considered to be a multi-factorial disease, where underlying genetic predisposition, environmental factors and epigenetic mechanisms contribute to disease development. Epigenetic mechanisms, such as DNA methylation, histone modifications and non-coding RNAs, may constitute a dynamic link between genome, environment and phenotypic manifestation by their modulating effects on gene expression...
April 9, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29648677/excess-productivity-costs-of-systemic-lupus-erythematosus-systemic-sclerosis-and-sjogren-s-syndrome-a-general-population-based-study
#17
Natalie McCormick, Carlo A Marra, Mohsen Sadatsafavi, Jacek A Kopec, J Antonio Aviña-Zubieta
OBJECTIVE: Determine excess productivity losses and costs of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjogren's syndrome (SjS) at the population level. METHODS: Administrative databases from the province of British Columbia, Canada, were used to establish population-based cohorts of SLE, SSc, and SjS, and matched comparison cohorts selected from the general population. Random samples from these cohorts were surveyed about time absent from paid and unpaid work, and working at reduced levels/efficiency (presenteeism), using validated labour questionnaires...
April 12, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29644928/salivary-gland-ultrasound-is-linked-to-the-autoimmunity-profile-in-patients-with-primary-sj%C3%A3-gren-s-syndrome
#18
Juan Carlos Nieto-González, Juan G Ovalles-Bonilla, Eduardo Estrada, Belén Serrano-Benavente, Julia Martínez-Barrio, Carlos M González-Fernández, Roberto González-Benítez, Cristina Vergara Dangond, Indalecio Monteagudo, Luis Collado Yurrita, Francisco Javier López-Longo
Objective Salivary gland ultrasound (SGU) is a reliable technique for assessing the salivary glands in patients with primary Sjögren's syndrome (pSS). The aim of this study was to elucidate the relationship between SGU findings and autoimmunity in patients with pSS. Methods Patients with pSS underwent an SGU assessment. The patients were classified into three groups according to their autoimmunity profile: the complete positive group (positive rheumatoid factor, antinuclear antibodies, and anti-Ro/anti-La antibodies), the partial seropositive group (positivity of at least one autoantibody but not all), and the seronegative group...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29627826/meibomian-gland-dysfunction-in-primary-and-secondary-sj%C3%A3-gren-syndrome
#19
David A Sullivan, Reza Dana, Rose M Sullivan, Kathleen L Krenzer, Afsun Sahin, Beril Arica, Yang Liu, Wendy R Kam, Athena S Papas, Jennifer M Cermak
PURPOSE: We hypothesized that women with primary (pSS) and secondary Sjögren syndrome (sSS; with systemic lupus erythematosus [SLE] or rheumatoid arthritis [RA]) have meibomian gland dysfunction (MGD). We sought to test our hypothesis. METHODS: Subjects with pSS, sSS + SLE, sSS + RA, and non-SS-related MGD were recruited from the Sjögren's Syndrome Foundation or outpatient clinics at Tufts University School of Dental Medicine or Brigham and Women's Hospital. The control population was recruited from the Greater Boston area...
April 6, 2018: Ophthalmic Research
https://www.readbyqxmd.com/read/29624878/elastographic-ultrasound-an-additional-image-tool-in-sj%C3%A3-gren-s-syndrome
#20
Eric Kimura-Hayama, Sergio Criales-Vera, Luis Azpeitia-Espinosa, Carlos Pacheco-Molina, Edgardo Reyes, Guadalupe Lima, Diego Hernandez-Ramirez, Luis Llorente, Gabriela Hernandez-Molina
AIM: To evaluate the stiffness of parotid and submandibular glands using elastography ultrasound and to correlate it with B-mode ultrasonographical, clinical and serological features, salivary profibrotic and inflammatory chemokines, and salivary gland fibrosis. METHODS: We performed B-mode and elastography ultrasound of major salivary glands of 26 patients with primary Sjögren's syndrome. We registered the shear wave velocity (SWV) and correlated it with the morphologic ultrasonographic changes assessed by the Hocevar scale...
April 6, 2018: International Journal of Rheumatic Diseases
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