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Systemic sclerosis

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https://www.readbyqxmd.com/read/29444467/olfactory-function-in-systemic-lupus-erythematosus-and-systemic-sclerosis-a-longitudinal-study-and-review-of-the-literature
#1
REVIEW
Mariana Freschi Bombini, Fernando Augusto Peres, Aline Tamires Lapa, Nailú Angélica Sinicato, Beatriz Ricato Quental, Ágatha de Souza Melo Pincelli, Tiago Nardi Amaral, Caroline Cristina Gomes, Ana Paula Del Rio, João Francisco Marques-Neto, Lilian T L Costallat, Paula Teixeira Fernandes, Fernando Cendes, Leticia Rittner, Simone Appenzeller
BACKGROUND/PURPOSE: To evaluate olfactory function in systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and healthy controls over a 2-year period, and to determine the association of olfactory dysfunction with age, disease activity, disease damage, treatment, anxiety and depression symptoms and limbic structures volumes. METHODS: Consecutive SLE and SSc patients were enrolled in this study. Clinical, laboratory disease activity and damage were assessed according to diseases specific guidelines...
February 11, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29444362/the-use-of-jak-inhibitors-in-the-treatment-of-progressive-systemic-sclerosis
#2
Sandhya Chowdary Deverapalli, David Rosmarin
Systemic sclerosis is a connective tissue disorder characterized by fibrosis. Patients may have restricted movement of the joints, Raynaud's phenomenon, self-amputation of the digits, dyspnea, and upper gastrointestinal symptoms. Excessive production of extracellular matrix (ECM) proteins such as type I collagen via transforming growth factor β (TGF β) are thought to be responsible for the fibrosis. 1 This article is protected by copyright. All rights reserved.
February 14, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29442534/efficacy-and-safety-of-combination-therapy-with-prednisolone-and-oral-tacrolimus-for-progressive-interstitial-pneumonia-with-systemic-sclerosis-a-retrospective-study
#3
Junichi Konma, Takuya Kotani, Takeshi Shoda, Takayasu Suzuka, Youhei Fujiki, Koji Nagai, Kenichiro Hata, Shuzo Yoshida, Tohru Takeuchi, Shigeki Makino, Shigeki Arawaka
OBJECTIVES: We retrospectively investigated efficacy and safety of combination therapy with prednisolone (PSL) and tacrolimus (TAC) for progressive interstitial pneumonitis with systemic sclerosis (SSc-PIP). METHODS: We studied 11 patients with SSc-PIP who received combination therapy with PSL (0.5 mg/kg/day) and TAC (3 mg/day). RESULTS: Baseline Hugh-Jones grades were I, II, III, and IV in 2, 6, 2, and 1 patients, respectively. KL-6 values were elevated to 914 (300-2614) U/mL...
February 14, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29442260/esophageal-high-resolution-impedance-manometry-alterations-in-asymptomatic-patients-with-systemic-sclerosis-prevalence-associations-with-disease-features-and-prognostic-value
#4
Serena Vettori, Salvatore Tolone, Domenico Capocotta, Rossella Chieffo, Veronica Giacco, Gabriele Valentini, Ludovico Docimo
This study aims to investigate pre-clinical esophageal involvement in systemic sclerosis (SSc) by high-resolution impedance manometry (HRiM), its associations with disease features including lung involvement, and its predictivity of esophageal symptoms overtime. Charts of 45 asymptomatic (no heartburn/regurgitation/dysphagia) SSc patients (96% females; mean age 46 years) with at least one follow-up (FU) visit and complete clinical, serological, functional, and radiological assessment, including high-resolution computed tomography (HRCT) of the chest and lung function tests, that had undergone esophageal HRiM were retrospectively evaluated...
February 13, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29440425/bryostatin-1-alleviates-experimental-multiple-sclerosis
#5
Michael D Kornberg, Matthew D Smith, Hasti Atashi Shirazi, Peter A Calabresi, Solomon H Snyder, Paul M Kim
Multiple sclerosis (MS) is an inflammatory disorder targeting the central nervous system (CNS). The relapsing-remitting phase of MS is largely driven by peripheral activation of autoreactive T-helper (Th) 1 and Th17 lymphocytes. In contrast, compartmentalized inflammation within the CNS, including diffuse activation of innate myeloid cells, characterizes the progressive phase of MS, the most debilitating phase that currently lacks satisfactory treatments. Recently, bryostatin-1 (bryo-1), a naturally occurring, CNS-permeable compound with a favorable safety profile in humans, has been shown to act on antigen-presenting cells to promote differentiation of lymphocytes into Th2 cells, an action that might benefit Th1-driven inflammatory conditions such as MS...
February 12, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29440397/sex-bias-in-mhc-i-associated-shaping-of-the-adaptive-immune-system
#6
Tilman Schneider-Hohendorf, Dennis Görlich, Paula Savola, Tiina Kelkka, Satu Mustjoki, Catharina C Gross, Geoffrey C Owens, Luisa Klotz, Klaus Dornmair, Heinz Wiendl, Nicholas Schwab
HLA associations, T cell receptor (TCR) repertoire bias, and sex bias have independently been shown for many diseases. While some immunological differences between the sexes have been described, they do not fully explain bias in men toward many infections/cancers, and toward women in autoimmunity. Next-generation TCR variable beta chain (TCRBV) immunosequencing of 824 individuals was evaluated in a multiparametric analysis including HLA-A -B/MHC class I background, TCRBV usage, sex, age, ethnicity, and TCRBV selection/expansion dynamics...
February 12, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29439120/improved-visualization-of-cortical-lesions-in-multiple-sclerosis-using-7t-mp2rage
#7
E S Beck, P Sati, V Sethi, T Kober, B Dewey, P Bhargava, G Nair, I C Cortese, D S Reich
BACKGROUND AND PURPOSE: Cortical lesions are common and often extensive in multiple sclerosis but are difficult to visualize by MRI, leaving important questions about their clinical implications and response to therapy unanswered. Our aim was to determine whether cortical lesions are better visualized using magnetization prepared 2 rapid acquisition gradient echoes (MP2RAGE) than T2*-weighted imaging on 7T MR imaging. MATERIALS AND METHODS: Brain MR imaging using T1-weighted MP2RAGE at 500-μm isotropic resolution, T2*-weighted gradient-echo, and T2*-weighted segmented echo-planar imaging sequences were collected for 13 patients with MS and 5 age-matched neurologically healthy controls on a 7T research system...
February 8, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29438266/new-ways-of-seeing-the-mechanistic-heterogeneity-of-multiple-sclerosis-plaque-pathogenesis
#8
Ethan I Meltzer, Fiona E Costello, Elliot M Frohman, Teresa C Frohman
BACKGROUND: Over the past few decades, we have witnessed a transformation with respect to the principles and pathobiological underpinnings of multiple sclerosis (MS). From the traditional rubric of MS as an inflammatory and demyelinating disorder restricted to central nervous system (CNS) white matter, our contemporary view has evolved to encompass a broader understanding of the variable mechanisms that contribute to tissue injury, in a disorder now recognized to affect white and grey matter compartments...
March 2018: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/29438265/keep-your-eyes-wide-open-on-visual-and-vision-related-measurements-to-better-understand-multiple-sclerosis-pathophysiology
#9
Yael Backner, Netta Levin
BACKGROUND: Multiple sclerosis (MS), a demyelinating disease of the central nervous system, is multifaceted. It manifests as acute episodes as well as an accumulative chronic disability; myelin involvement as well as axonal damage; local as well as global effects; and disease load elements as well as compensatory mechanisms. The visual system, with its clear structural organization and relatively direct reflection of damage, may serve as an appropriate model to study MS. METHODS: In recent years, we have witnessed a blossoming in the field of visual measures in MS...
March 2018: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/29438164/the-autoimmune-ecology-an-update
#10
Juan-Manuel Anaya, Paula Restrepo-Jiménez, Carolina Ramírez-Santana
PURPOSE OF REVIEW: The autoimmune ecology refers to the interactions between individuals and their environment leading to a breakdown in immune tolerance and, therefore, to the development of one or more autoimmune diseases in such an individual. Herein, an update is offered on four specific factors associated with autoimmune diseases, namely, vitamin D, smoking, alcohol and coffee consumption from the perspective of exposome and metabolomics. RECENT FINDINGS: Smoking is associated with an increased risk for most of the autoimmune diseases...
February 12, 2018: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/29435762/establishment-of-a-myelinating-co-culture-system-with-a-motor-neuron-like-cell-line-nsc-34-and-an-adult-rat-schwann-cell-line-ifrs1
#11
Shizuka Takaku, Hideji Yako, Naoko Niimi, Tomoyo Akamine, Daiji Kawanami, Kazunori Utsunomiya, Kazunori Sango
Co-culture models of neurons and Schwann cells have been utilized for the study of myelination and demyelination in the peripheral nervous system; in most of the previous studies, however, these cells were obtained by primary culture with embryonic or neonatal animals. A spontaneously immortalized Schwann cell line IFRS1 from long-term cultures of adult Fischer rat peripheral nerves has been shown to retain fundamental ability to myelinate neurites in co-cultures with adult rat dorsal root ganglion neurons and nerve growth factor-primed PC12 cells...
February 12, 2018: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/29435755/surgical-management-of-gastroesophageal-reflux-disease-in-patients-with-systemic-sclerosis
#12
Jingliang Yan, Andrew T Strong, Gautam Sharma, Scott Gabbard, Prashanti Thota, John Rodriguez, Matthew Kroh
BACKGROUND: Systemic sclerosis (scleroderma) is frequently associated with both gastroesophageal reflux disease (GERD) and simultaneous esophageal dysmotility. Anti-reflux procedures in this patient population must account for the existing physiology of each patient and likely disease progression. We aim to compare perioperative and intermediate outcomes of fundoplication versus gastric bypass for the treatment of GERD. METHODS: After IRB approval, patients with systemic sclerosis undergoing fundoplication or gastric bypass for the treatment of GERD from 2004 to 2016 were identified...
February 12, 2018: Surgical Endoscopy
https://www.readbyqxmd.com/read/29435179/identifying-characteristic-mirnas-genes-and-risk-pathways-of-multiple-sclerosis-based-on-bioinformatics-analysis
#13
Deling Luo, Jin Fu
Multiple sclerosis is a chronic autoimmune disorder of the central nervous system. In MS, the genetic susceptibility is high and currently there is no effective treatment. MicroRNA, a small non-coding RNA, plays a vital role in immune responses. Aberrant or dysfunctional miRNAs may cause several diseases, including MS, thus miRNAs and miRNA related genes may be therapeutic weapons against MS. Here, we identified 21 miRNAs in peripheral blood mono-nuclear cells from over 600 persons, including healthy controls...
January 12, 2018: Oncotarget
https://www.readbyqxmd.com/read/29434586/bioactive-lipids-and-chronic-inflammation-managing-the-fire-within
#14
REVIEW
Valerio Chiurchiù, Alessandro Leuti, Mauro Maccarrone
Inflammation is an immune response that works as a contained fire that is pre-emptively sparked as a defensive process during infections or upon any kind of tissue insult, and that is spontaneously extinguished after elimination or termination of the damage. However, persistent and uncontrolled immune reactions act as a wildfire that promote chronic inflammation, unresolved tissue damage and, eventually, chronic diseases. A wide network of soluble mediators, among which endogenous bioactive lipids, governs all immune processes...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29433908/pro-fibrotic-phenotype-of-human-skin-fibroblasts-induced-by-periostin-via-modulating-tgf-%C3%AE-signaling
#15
Miwa Kanaoka, Yukie Yamaguchi, Noriko Komitsu, Carol A Feghali-Bostwick, Masahiro Ogawa, Kazuhiko Arima, Kenji Izuhara, Michiko Aihara
BACKGROUND: Periostin is a matricellular protein that belongs to a class of extracellular matrix (ECM)-related molecules defined by their ability to modulate cell-matrix interactions. We previously reported an elevated level of circulating periostin in patients with systemic sclerosis (SSc) and its association with the severity of skin sclerosis. OBJECTIVE: To examine the role of periostin in transforming growth factor (TGF)-β signaling involved in fibrosis. METHODS: Levels of periostin were examined in skin and lung fibroblasts obtained from SSc patients...
February 6, 2018: Journal of Dermatological Science
https://www.readbyqxmd.com/read/29433734/foxo-in-neural-cells-and-diseases-of-the-nervous-system
#16
Evan E Santo, Jihye Paik
The evolutionarily conserved FOXO family of transcription factors has emerged as a significant arbiter of neural cell fate and function in mammals. From the neural stem cell (NSC) state through mature neurons under both physiological and pathological conditions, they have been found to modulate neural cell survival, stress responses, lineage commitment, and neuronal signaling. Lineage-specific FOXO knockout mice have provided an invaluable tool for the dissection of FOXO biology in the nervous system. Within the NSC compartments of the brain, FOXOs are required for the maintenance of NSC quiescence and for the clearance of reactive oxygen species...
2018: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/29432239/neurocutaneous-disorders
#17
Tena Rosser
PURPOSE OF REVIEW: This article presents an up-to-date summary of the genetic etiology, diagnostic criteria, clinical features, and current management recommendations for the most common neurocutaneous disorders encountered in clinical adult and pediatric neurology practices. RECENT FINDINGS: The phakomatoses are a phenotypically and genetically diverse group of multisystem disorders that primarily affect the skin and central nervous system. A greater understanding of the genetic and biological underpinnings of numerous neurocutaneous disorders has led to better clinical characterization, more refined diagnostic criteria, and improved treatments in neurofibromatosis type 1, Legius syndrome, neurofibromatosis type 2, Noonan syndrome with multiple lentigines, tuberous sclerosis complex, Sturge-Weber syndrome, and incontinentia pigmenti...
February 2018: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29431122/poly-adp-ribose-polymerase-1-regulates-fibroblast-activation-in-systemic-sclerosis
#18
Yun Zhang, Sebastian Pötter, Chih-Wei Chen, Ruifang Liang, Kolja Gelse, Ingo Ludolph, Raymund E Horch, Oliver Distler, Georg Schett, Jörg H W Distler, Clara Dees
OBJECTIVES: The enzyme poly(ADP-ribose) polymerase-1 (PARP-1) transfers negatively charged ADP-ribose units to target proteins. This modification can have pronounced regulatory effects on target proteins. Recent studies showed that PARP-1 can poly(ADP-ribosyl)ate (PARylate) Smad proteins. However, the role of PARP-1 in the pathogenesis of systemic sclerosis (SSc) has not been investigated. METHODS: The expression of PARP-1 was determined by quantitative PCR and immunohistochemistry...
February 3, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29429076/cardiovascular-autonomic-dysfunction-link-between-multiple-sclerosis-osteoporosis-and-neurodegeneration
#19
REVIEW
Zohara Sternberg
The high prevalence of osteoporosis, observed in multiple sclerosis (MS) patients, has been attributed to reduced mobility and or the use of disease-modifying drugs. However, MS-impaired cardiovascular autonomic nervous system (ANS) function has the potential of reducing bone mass density (BMD) by altering the expression and/or function of the neuronal, systemic, and local mediators of bone remodeling. This review describes the complex regulation of bone homeostasis with a focus on MS, providing evidence that ANS dysfunction and low BMD are intertwined with MS inflammatory and neurodegenerative processes, and with other MS-related morbidities, including depression, fatigue, and migraine...
February 10, 2018: Neuromolecular Medicine
https://www.readbyqxmd.com/read/29428463/cardiac-repolarization-evolves-differently-during-the-course-of-benign-and-disabling-multiple-sclerosis
#20
Alma Mikkola, Aku Ojanen, Juha Ek Hartikainen, Anne M Remes, Sakari Simula
BACKGROUND: Cardiac repolarization is modulated by the autonomic nervous system. Even though multiple sclerosis associates with prolonged cardiac repolarization the physiology responsible for the phenomenon remains unknown. OBJECTIVE: To study in longitudinal setting whether the patients with confirmed benign and disabling outcome of relapsing-remitting multiple sclerosis (RRMS) differ in regard to changes of cardiac repolarization. METHODS: Total of 43 patients, 26% with benign (EDSS ≤2 at least 10y after onset symptom) and 74% with disabling (EDSS >2 at least 10y after onset symptom) RRMS, having 12-lead electrocardiogram (ECG) recorded at the time of onset symptom (ECG1) and for follow-up (ECG2), were studied...
February 1, 2018: Multiple Sclerosis and related Disorders
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