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Systemic sclerosis

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https://www.readbyqxmd.com/read/28822119/five-questions-answered-a-review-of-autologous-hematopoietic-stem-cell-transplantation-for-the-treatment-of-multiple-sclerosis
#1
REVIEW
Harold L Atkins, Mark S Freedman
Multiple sclerosis (MS) is thought to be an autoimmune disease targeting the central nervous system leading to demyelination, and axonal and neuronal damage, resulting in progressive disability. More intensive therapies such as immunodepletion with hematopoietic stem-cell rescue are being used at a time prior to patients becoming irreversibly disabled. Over the last 15 years, there has been a shift away from using autologous hematopoietic stem-cell transplants (aHSCT) to treat patients with progressive MS, towards treating those with active inflammation and relapses...
August 18, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28822061/co-ultramicronized-palmitoylethanolamide-luteolin-facilitates-the-development-of-differentiating-and-undifferentiated-rat-oligodendrocyte-progenitor-cells
#2
Stephen D Skaper, Massimo Barbierato, Laura Facci, Mila Borri, Gabriella Contarini, Morena Zusso, Pietro Giusti
Oligodendrocytes, the myelin-producing cells of the central nervous system (CNS), have limited capability to bring about repair in chronic CNS neuroinflammatory demyelinating disorders such as multiple sclerosis (MS). MS lesions are characterized by a compromised pool of undifferentiated oligodendrocyte progenitor cells (OPCs) unable to mature into myelin-producing oligodendrocytes. An attractive strategy may be to replace lost OLs and/or promote their maturation. N-palmitoylethanolamine (PEA) is an endogenous fatty acid amide signaling molecule with anti-inflammatory and neuroprotective actions...
August 18, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28821749/dna-methylation-in-demyelinated-multiple-sclerosis-hippocampus
#3
Anthony M Chomyk, Christina Volsko, Ajai Tripathi, Sadie A Deckard, Bruce D Trapp, Robert J Fox, Ranjan Dutta
Multiple Sclerosis (MS) is an immune-mediated demyelinating disease of the human central nervous system (CNS). Memory impairments and hippocampal demyelination are common features in MS patients. Our previous data have shown that demyelination alters neuronal gene expression in the hippocampus. DNA methylation is a common epigenetic modifier of gene expression. In this study, we investigated whether DNA methylation is altered in MS hippocampus following demyelination. Our results show that mRNA levels of DNA methyltransferase were increased in demyelinated MS hippocampus, while de-methylation enzymes were decreased...
August 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28821687/mri-substrates-of-sustained-attention-system-and-cognitive-impairment-in-pediatric-ms-patients
#4
Ermelinda De Meo, Lucia Moiola, Angelo Ghezzi, Pierangelo Veggiotti, Ruggero Capra, Maria Pia Amato, Elisabetta Pagani, Agnese Fiorino, Lorena Pippolo, Maria C Pera, Giancarlo Comi, Andrea Falini, Massimo Filippi, Maria A Rocca
OBJECTIVE: To explore the structural and functional integrity of the sustained attention system in patients with pediatric multiple sclerosis (MS) and its effect on cognitive impairment. METHODS: We enrolled 57 patients with pediatric MS and 14 age- and sex-matched healthy controls (HCs). Patients with >3 abnormal tests at neuropsychological evaluation were classified as cognitively impaired (CI). Sustained attention system activity was studied with fMRI during the Conners Continuous Performance Test (CCPT)...
August 18, 2017: Neurology
https://www.readbyqxmd.com/read/28819792/validation-of-the-acr-eular-classification-criteria-for-systemic-sclerosis-in-patients-with-early-scleroderma
#5
Farley Carvalho Araújo, Cíntia Zumstein Camargo, Cristiane Kayser
The aim of this study was to validate the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in patients with SSc, including patients with early SSc. Fifty-six consecutive patients with early SSc (2001 LeRoy and Medsger criteria), 122 patients with established SSc (1980 ACR classification criteria), and 141 patients with SSc-like disorders were included in this cross-sectional study. The diagnostic performance of the 2013 ACR/EULAR criteria was compared with the 1980 ACR criteria in several subsets of patients...
August 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#6
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28817964/understanding-coping-strategies-among-people-living-with-scleroderma-a-focus-group-study
#7
Stephanie T Gumuchian, Sandra Peláez, Vanessa C Delisle, Marie-Eve Carrier, Lisa R Jewett, Ghassan El-Baalbaki, Catherine Fortune, Marie Hudson, Annett Körner, Linda Kwakkenbos, Susan J Bartlett, Brett D Thombs
PURPOSE: Systemic sclerosis or scleroderma is a chronic, rare connective tissue disease with negative physical and psychological implications. Coping strategies used by scleroderma patients have not been studied in-depth. The objective of the present study was to gain a greater understanding of the coping strategies employed by people living with scleroderma. METHOD: Three semi-structured focus group discussions were conducted with a total of 22 people with scleroderma...
August 17, 2017: Disability and Rehabilitation
https://www.readbyqxmd.com/read/28817465/new-insights-into-the-recognition-classification-and-management-of-systemic-sclerosis-associated-pulmonary-hypertension
#8
Christopher J Mullin, Stephen C Mathai
PURPOSE OF REVIEW: Pulmonary hypertension is a common complication of systemic sclerosis (SSc), and remains a leading cause of morbidity and mortality. We will review recent developments in the recognition, classification and treatment of pulmonary hypertension in SSc. RECENT FINDINGS: Advances in screening for pulmonary arterial hypertension (PAH) and use of exercise haemodynamics may help to identify pulmonary vascular disease earlier in SSc. Recent studies have led to changes in recommendations for adjunct therapy and selection of pulmonary vasodilators for the treatment of SSc-associated PAH...
August 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28816034/cerebrospinal-fluid-cytology-in-nonmalignant-aseptic-meningeal-disorders
#9
REVIEW
Rafael Martínez-Girón, Liron Pantanowitz
Cerebrospinal fluid cytology examination is a common and reliable primary and/or complementary procedure for the diagnosis of central nervous system (CNS) disorders. This review provides an update of aseptic meningeal disorders that may be encountered in cytopathology practice. The article covers the cytological findings and helpful ancillary studies needed of nonmalignant aseptic CNS disorders such as viral, bacterial, fungal and parasitic infections, and other noninfectious diseases, such as Mollaret´s meningitis (recurrent benign lymphocytic meningitis), Guillain-Barré syndrome, multiple Sclerosis, subarachnoid haemorrhage, and drug-induced disorders...
August 16, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/28814429/nintedanib-macrophage-activation-and-ameliorates-vascular-and-fibrotic-manifestations-in-the-fra2-mouse-model-of-systemic-sclerosis
#10
Jingang Huang, Christiane Maier, Yun Zhang, Alina Soare, Clara Dees, Christian Beyer, Ulrike Harre, Chih-Wei Chen, Oliver Distler, Georg Schett, Lutz Wollin, Jörg H W Distler
BACKGROUND: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc). METHODS: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed...
August 16, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28813960/towards-free-3d-end-point-control-for-robotic-assisted-human-reaching-using-binocular-eye-tracking
#11
Roni O Maimon-Dror, Jorge Fernandez-Quesada, Giuseppe A Zito, Charalambos Konnaris, Sabine Dziemian, A Aldo Faisal
Eye-movements are the only directly observable behavioural signals that are highly correlated with actions at the task level, and proactive of body movements and thus reflect action intentions. Moreover, eye movements are preserved in many movement disorders leading to paralysis (or amputees) from stroke, spinal cord injury, Parkinson's disease, multiple sclerosis, and muscular dystrophy among others. Despite this benefit, eye tracking is not widely used as control interface for robotic interfaces in movement impaired patients due to poor human-robot interfaces...
July 2017: IEEE ... International Conference on Rehabilitation Robotics: [proceedings]
https://www.readbyqxmd.com/read/28812229/economics-and-cost-effectiveness-of-multiple-sclerosis-therapies-in-the-usa
#12
REVIEW
Daniel M Hartung
Multiple sclerosis (MS) is a disabling, chronic disease that imposes a significant economic burden on patients and the US healthcare system. The largest cost component for individuals with MS are prescription drugs, specifically disease-modifying therapies (DMTs). Despite an increase in the number and diversity of DMTs over the past 10 years, acquisition costs for all DMTs have escalated dramatically at rates substantially higher than medical inflation. Currently, costs for most DMTs exceed $70,000 a year. Recent cost-effectiveness studies suggest the cost for nearly all DMTs exceeds generally accepted thresholds for what is considered a good value in the USA, even after factoring expected rebates...
August 15, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28811529/disturbance-of-plasma-lipid-metabolic-profile-in-guillain-barre-syndrome
#13
Hsiang-Yu Tang, Daniel Tsun-Yee Chiu, Jui-Fen Lin, Cheng-Yu Huang, Kuo-Hsuan Chang, Rong-Kuo Lyu, Long-Sun Ro, Hung-Chou Kuo, Mei-Ling Cheng, Chiung-Mei Chen
Guillain-Barre Syndrome (GBS) is an inflammatory disease of the peripheral nervous system. Given that plasma metabolic profiles in GBS patients have never been explored, plasma samples of 38 GBS patients, 22 multiple sclerosis (MS) patients, and 40 healthy controls were analyzed by using untargeted and targeted metabolomics analysis. The untargeted analysis showed that levels of a set of plasma lipid metabolites were significantly decreased in GBS patients compared to the controls. Furthermore, the targeted analysis demonstrated that levels of 41 metabolites in GBS patients were significantly changed compared to either the controls or MS patients...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28811201/the-unmet-need-in-rheumatology-reports-from-the-targeted-therapies-meeting-2017
#14
Kevin L Winthrop, Vibeke Strand, Désirée van der Heijde, Philip Mease, Mary Crow, Michael Weinblatt, Joan Bathon, Gerd R Burmester, Maxime Dougados, Johnathan Kay, Xavier Mariette, Ronald Van Vollenhoven, Joachim Sieper, Fritz Melchers, Ferdinand C Breedfeld, Joachim Kalden, Josef S Smolen, Daniel E Furst
The 19th annual international Targeted Therapies meeting brought together over 100 leading basic scientists and clinical researchers from around the world in the field of immunology, molecular biology and rheumatology and other specialties. During the meeting, breakout sessions were held consisting of 5 disease-specific groups with 20-40 experts assigned to each group based on clinical or scientific expertise. Specific groups included: rheumatoid arthritis, psoriatic arthritis, axial spondyloarthritis, systemic lupus erythematous, connective tissue diseases (e...
August 12, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28811143/nadph-oxidases-as-drug-targets-and-biomarkers-in-neurodegenerative-diseases-what-is-the-evidence
#15
REVIEW
Silvia Sorce, Roland Stocker, Tamara Seredenina, Rikard Holmdahl, Adriano Aguzzi, Adriano Chio, Antoine Depaulis, Freddy Heitz, Peter Olofsson, Tomas Olsson, Venceslas Duveau, Despina Sanoudou, Sara Skosgater, Antonia Vlahou, Dominique Wasquel, Karl-Heinz Krause, Vincent Jaquet
Neurodegenerative disease are frequently characterized by microglia activation and/or leukocyte infiltration in the parenchyma of the central nervous system and at the molecular level by increased oxidative modifications of proteins, lipids and nucleic acids. NADPH oxidases (NOX) emerged as a novel promising class of pharmacological targets for the treatment of neurodegeneration due to their role in oxidant generation and presumably in regulating microglia activation. The unique function of NOX is the generation of superoxide anion (O2(•-)) and hydrogen peroxide (H2O2)...
August 12, 2017: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/28808237/coding-and-small-non-coding-transcriptional-landscape-of-tuberous-sclerosis-complex-cortical-tubers-implications-for-pathophysiology-and-treatment
#16
James D Mills, Anand M Iyer, Jackelien van Scheppingen, Anika Bongaarts, Jasper J Anink, Bart Janssen, Till S Zimmer, Wim G Spliet, Peter C van Rijen, Floor E Jansen, Martha Feucht, Johannes A Hainfellner, Pavel Krsek, Josef Zamecnik, Katarzyna Kotulska, Sergiusz Jozwiak, Anna Jansen, Lieven Lagae, Paolo Curatolo, David J Kwiatkowski, R Jeroen Pasterkamp, Ketharini Senthilkumar, Lars von Oerthel, Marco F Hoekman, Jan A Gorter, Peter B Crino, Angelika Mühlebner, Brendon P Scicluna, Eleonora Aronica
Tuberous Sclerosis Complex (TSC) is a rare genetic disorder that results from a mutation in the TSC1 or TSC2 genes leading to constitutive activation of the mechanistic target of rapamycin complex 1 (mTORC1). TSC is associated with autism, intellectual disability and severe epilepsy. Cortical tubers are believed to represent the neuropathological substrates of these disabling manifestations in TSC. In the presented study we used high-throughput RNA sequencing in combination with systems-based computational approaches to investigate the complexity of the TSC molecular network...
August 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28807667/inhibition-of-beta-catenin-signaling-in-the-skin-rescues-cutaneous-adipogenesis-in-systemic-sclerosis-a-randomized-double-blind-placebo-controlled-trial-of-c-82
#17
Robert Lafyatis, Julio C Mantero, Jessica Gordon, Nina Kishore, Mary Carns, Howard Dittrich, Robert Spiera, Robert W Simms, John Varga
Several studies have suggested that Wnts might contribute to skin fibrosis in systemic sclerosis (SSc) by affecting the differentiation of pluripotent dermal cells. We tested C-82, a therapeutic that inhibits canonical Wnt signaling by blocking the interaction of cAMP Response Element-Binding Protein with β-Catenin and inhibiting Wnt-activated genes. We utilized previously unreported trial design, formulating C-82 for topical application and conducting a placebo controlled, double-blinded clinical trial in which patients with diffuse cutaneous SSc were treated with C-82 or placebo on opposite forearms...
August 11, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28807589/differences-in-texture-analysis-parameters-between-active-alveolitis-and-lung-fibrosis-in-chest-ct-of-patients-with-systemic-sclerosis-a-feasibility-study
#18
Christopher Kloth, Anya C Blum, Wolfgang M Thaiss, Heike Preibsch, Hendrik Ditt, Rainer Grimmer, Jan Fritz, Konstantin Nikolaou, Hans Bösmüller, Marius Horger
RATIONALE AND OBJECTIVES: This study aimed to determine the diagnostic aid of computed tomography (CT) features for the differentiation of active alveolitis and fibrosis using a CT texture analysis (CTTA) prototype and CT densitometry in patients with systemic sclerosis (SSc) using ancillary high-resolution computed tomography (HRCT) features and their longitudinal course as standard of reference. MATERIALS AND METHODS: We retrospectively analyzed thin-slice noncontrast chest CT image data of 43 patients with SSc (18 men, mean age 51...
August 11, 2017: Academic Radiology
https://www.readbyqxmd.com/read/28807021/serological-and-morphological-prognostic-factors-in-patients-with-interstitial-pneumonia-with-autoimmune-features
#19
Yuhei Ito, Machiko Arita, Shogo Kumagai, Reoto Takei, Maki Noyama, Fumiaki Tokioka, Keisuke Nishimura, Takashi Koyama, Kenji Notohara, Tadashi Ishida
BACKGROUND: To identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria. METHODS: We retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20)...
August 14, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28806189/the-use-of-optical-coherence-tomography-in-neuro-ophthalmology
#20
Noel C Y Chan, Carmen K M Chan
PURPOSE OF REVIEW: In the last decade, with the advances of optical coherence tomography (OCT) technology, different imaging protocols and analysis algorithms have been introduced to maximize the potential of this diagnostic tool in the evaluation of different eye diseases. This review aims to provide an update on these additional features, with respect to the management of a diverse range of neuro-ophthalmologic conditions. RECENT FINDINGS: Macular ganglion cell complex (mGCC) analysis has been shown to be superior to peripapillary retinal nerve fiber layer (pRNFL) analysis in certain settings, such as differentiating Leber's hereditary optic neuropathy from functional visual loss; monitoring neurodegenerative diseases or multiple sclerosis; and predicting visual loss in nonarteritic ischemic optic neuropathy...
August 12, 2017: Current Opinion in Ophthalmology
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