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Progressive systemic sclerosis

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https://www.readbyqxmd.com/read/29785360/identical-patterns-of-cortico-efferent-tract-involvement-in-primary-lateral-sclerosis-and-amyotrophic-lateral-sclerosis-a-tract-of-interest-based-mri-study
#1
Hans-Peter Müller, Martin Gorges, Rebecca Kassubek, Johannes Dorst, Albert C Ludolph, Jan Kassubek
Background: There is an ongoing debate whether primary lateral sclerosis (PLS) should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis (ALS) or as a slowly progressive variant of ALS. Objective: The study was designed to investigate specific white matter alterations in diffusion tensor imaging (DTI) data from PLS patients by a hypothesis-guided tract-of-interest-based approach compared with 'classical' ALS patients and healthy controls, in order to identify microstructural changes according to the neuropathologically defined ALS affectation pattern in vivo...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29784250/update-on-pediatric-onset-multiple-sclerosis
#2
REVIEW
A Ruet
Pediatric-onset multiple sclerosis (POMS) has distinctive features compared with adult-onset multiple sclerosis (AOMS), and warrants caution despite being a rare form of MS. POMS diagnostic criteria are somewhat different from those used in AOMS, with acute disseminated encephalomyelitis being a key differential diagnosis of MS in children. Other differential diagnoses that have to be ruled out before diagnosing MS include demyelinating syndromes, autoimmune and systemic pathologies, and infectious, genetic, metabolic and neoplastic diseases...
May 18, 2018: Revue Neurologique
https://www.readbyqxmd.com/read/29784248/conventional-and-advanced-mri-in-multiple-sclerosis
#3
REVIEW
C Louapre
Magnetic resonance imaging (MRI) plays a central role in the management of patients with multiple sclerosis (MS). T2-weighted/FLAIR lesions have been included in the diagnostic criteria since 2001, and the importance of the technology has been expanded in each successive revision of the McDonald criteria. While the typical focal hyperintense lesions seen on T2 and FLAIR sequences in several areas of the central nervous system are key features for MS diagnosis, they can also be used to monitor disease activity, particularly in asymptomatic patients, and to evaluate therapeutic responses...
May 18, 2018: Revue Neurologique
https://www.readbyqxmd.com/read/29781588/smoking-in-systemic-sclerosis-a-longitudinal-european-scleroderma-trials-and-research-group-study
#4
Veronika K Jaeger, Gabriele Valentini, Eric Hachulla, Franco Cozzi, Oliver Distler, Paolo Airó, Laszlo Czirják, Yannick Allanore, Elise Siegert, Edoardo Rosato, Marco Matucci-Cerinic, Cristian Caimmi, Jörg Henes, Patricia E Carreira, Vanessa Smith, Francesco Del Galdo, Christopher P Denton, Susanne Ullman, Ellen De Langhe, Valeria Riccieri, Juan J Alegre-Sancho, Simona Rednic, Ulf Müller-Ladner, Ulrich A Walker
OBJECTIVES: Data on the role of tobacco exposure in systemic sclerosis (SSc) severity and progression are scarce. We aimed to assess the effects of smoking on the evolution of pulmonary and skin manifestations in the EUSTAR database. METHODS: Adult SSc patients with data on smoking history and a 12-24 months follow-up visit were included. Associations of severity and progression of organ involvement with smoking history and the comprehensive smoking index (CSI) were assessed using multivariable regression analyses...
May 21, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29780610/a-randomised-controlled-clinical-trial-of-narrowband-uvb-phototherapy-for-clinically-isolated-syndrome-the-phocis-study
#5
Prue H Hart, Anderson P Jones, Stephanie Trend, Lilian Cha, Marzena J Fabis-Pedrini, Matthew N Cooper, Catherine d'Este, Sian Geldenhuys, William M Carroll, Scott N Byrne, David R Booth, Judith M Cole, Robyn M Lucas, Allan G Kermode
Background: The natural history of multiple sclerosis (MS) typically presents with the clinically isolated syndrome (CIS), an episode of neurological symptoms caused by central nervous system inflammation or demyelination that does not fulfil the diagnostic criteria for MS. Objective: As preclinical studies have suggested that exposure to ultraviolet radiation (UVR) could regulate the development of MS, the Phototherapy for CIS (PhoCIS trial) was established to examine the effects of narrowband UVB phototherapy on patients with CIS, and their conversion to MS...
April 2018: Multiple Sclerosis Journal—Experimental, Translational and Clinical
https://www.readbyqxmd.com/read/29779597/amyotrophic-lateral-sclerosis-and-the-respiratory-system
#6
REVIEW
Andrew T Braun, Candelaria Caballero-Eraso, Noah Lechtzin
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder that always affects the respiratory muscles. It is characterized by degeneration of motor neurons in the brain and spinal cord. Respiratory complications are the most common causes of death in ALS and typically occur within 3 to 5 years of diagnosis. Because ALS affects both upper and lower motor neurons, it causes hyperreflexia, spasticity, muscle fasciculations, muscle atrophy, and weakness. It ultimately progresses to functional quadriplegia...
June 2018: Clinics in Chest Medicine
https://www.readbyqxmd.com/read/29776440/radiological-pleuroparenchymal-fibroelastosis-associated-to-limited-cutaneous-systemic-sclerosis-a-case-report
#7
D Hassoun, S Dirou, P P Arrigoni, C Durant, M Hamidou, A Néel, C Agard
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes...
May 18, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29774781/aging-and-lymphocyte-changes-by-immunomodulatory-therapies-impact-pml-risk-in-multiple-sclerosis-patients
#8
Elizabeth A Mills, Yang Mao-Draayer
New potent immunomodulatory therapies for multiple sclerosis (MS) are associated with increased risk for progressive multifocal leukoencephalopathy (PML). It is unclear why a subset of treated patients develops PML, but patient age has emerged as an important risk factor. PML is caused by the JC virus and aging is associated with immune senescence, which increases susceptibility to infection. With the goal of improving PML risk stratification, we here describe the lymphocyte changes that occur with disease-modifying therapies (DMTs) associated with high or moderate risk toward PML in MS patients, how these changes compare to immune aging, and which measures best correlate with risk...
May 1, 2018: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/29770132/differing-outcome-of-experimental-autoimmune-encephalitis-in-macrophage-neutrophil-and-t-cell-specific-gp130-deficient-mice
#9
Kristian Holz, Marco Prinz, Stefanie M Brendecke, Alexandra Hölscher, Fengyuan Deng, Hans-Willi Mitrücker, Stefan Rose-John, Christoph Hölscher
gp130 cytokines are differentially involved in regulating the T helper (H) 17-driven pathogenesis of experimental autoimmune encephalomyelitis (EAE), the animal model of human multiple sclerosis. Interleukin (IL)-6 directly promotes the development of TH17 cells through the gp130/IL-6R complex. By contrast, IL-27 has been shown to suppress a TH17 immune response by gp130/IL-27R-alpha (α) receptor ligation. The IL-27-dependent regulation of a TH17 development could be mediated on the level of CD4 T cells. However, because IL-27 also suppresses the secretion of the TH17-driving cytokines IL-6 and IL-12/23p40 in accessory cells, TH17 immune responses may also be controlled by IL-27 on the level of macrophages and/or neutrophils...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29769294/pulmonary-function-tests-as-outcomes-for-systemic-sclerosis-interstitial-lung-disease
#10
REVIEW
Melissa Caron, Sabrina Hoa, Marie Hudson, Kevin Schwartzman, Russell Steele
Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression ( i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD ( i...
June 30, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29764826/systemic-sclerosis-severe-pulmonary-arterial-hypertension-and-pericardial-effusion-at-diagnosis
#11
Patricia Moniz, Daniel Rei, Joana Rodrigues, Arturo Botella
A 51-year-old female patient with a recent hospital admission reported to the emergency room (ER) with progressive worsening of fatigue, dyspnoea and chest discomfort. She had been recently admitted and discharged with the diagnosis of pericarditis and medicated with non-steroidal anti-inflammatory drugs and diuretics. She returned to the ER with persisting symptoms. Echocardiography was repeated and showed signs of elevated right ventricular systolic pressure and a slightly increased moderate/severe pericardial effusion without signs of cardiac tamponade...
May 14, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29763902/prognostic-factors-of-renal-involvement-in-systemic-sclerosis
#12
Edoardo Rosato, Antonietta Gigante, Biagio Barbano, Maria Ludovica Gasperini, Rosario Cianci, Maurizio Muscaritoli
BACKGROUND/AIMS: Renal involvement is common in systemic sclerosis (SSc), including asymptomatic reduction of glomerular filtration rate (GFR), increased renal resistance indices, scleroderma renal crisis (SRC) and ANCA-associated vasculitis. The aim was to evaluate type and evolution of renal involvement for a period of five years. METHODS: 121 SSc patients (100 F, 21 M) with mean age of 54.9 ± 13.8, disease duration of 9 ± 6 years, of which 62 had a diffused form and 59 limited form were enrolled...
May 10, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29761344/beyond-the-magic-bullet-current-progress-of-therapeutic-vaccination-in-multiple-sclerosis
#13
Barbara Willekens, Nathalie Cools
Multiple sclerosis (MS) is a chronic immune-mediated disease of the central nervous system (CNS) characterized by neuroinflammation, neurodegeneration and impaired repair mechanisms that lead to neurological disability. The crux of MS is the patient's own immune cells attacking self-antigens in the CNS, namely the myelin sheath that protects nerve cells of the brain and spinal cord. Restoring antigen-specific tolerance via therapeutic vaccination is an innovative and exciting approach in MS therapy. Indeed, leveraging the body's attempt to prevent autoimmunity, i...
May 14, 2018: CNS Drugs
https://www.readbyqxmd.com/read/29760648/profile-of-arachidonic-acid-derived-inflammatory-markers-and-its-modulation-by-nitro-oleic-acid-in-an-inherited-model-of-amyotrophic-lateral-sclerosis
#14
Andrés Trostchansky, Mauricio Mastrogiovanni, Ernesto Miquel, Sebastián Rodríguez-Bottero, Laura Martínez-Palma, Patricia Cassina, Homero Rubbo
The lack of current treatments for amyotrophic lateral sclerosis (ALS) highlights the need of a comprehensive understanding of the biological mechanisms of the disease. A consistent neuropathological feature of ALS is the extensive inflammation around motor neurons and axonal degeneration, evidenced by accumulation of reactive astrocytes and activated microglia. Final products of inflammatory processes may be detected as a screening tool to identify treatment response. Herein, we focus on (a) detection of arachidonic acid (AA) metabolization products by lipoxygenase (LOX) and prostaglandin endoperoxide H synthase in SOD1G93A mice and (b) evaluate its response to the electrophilic nitro-oleic acid (NO2 -OA)...
2018: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29759135/the-levels-of-soluble-forms-of-cd21-and-cd83-in-multiple-sclerosis
#15
Sajad Karampoor, Hamid Zahednasab, Masoud Etemadifar, Hossein Keyvani
Multiple Sclerosis (MS) is a neuroinflammatory disease of the central nervous system (CNS) in which immune system plays a crucial role in progression of the disease. An enormous amount of research has been shown that immune mediators such as cytokines and chemokines are the culprits of MS propagation suggesting that modulation of such molecules may pave the path to hinder the disease development. It has been shown that both CD21 and CD83 contribute to the resolution of inflammation occurred in MS. CD21 and CD83 have also been ascribed to Epstein Barr virus (EBV) infection (the prime suspect of MS causality) and the levels of vitamin D, respectively...
July 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29759047/histological-predictors-of-renal-outcome-in-lupus-nephritis-the-importance-of-tubulointerstitial-lesions-and-scoring-of-glomerular-lesions
#16
B Obrișcă, R Jurubiță, A Andronesi, B Sorohan, C Achim, R Bobeica, M Gherghiceanu, E Mandache, G Ismail
Introduction Lupus nephritis (LN) affects nearly 60% of patients with systemic lupus erythematosus and up to 30% of them will progress to end-stage renal disease (ESRD), despite receiving aggressive immunosuppressive therapy. The prognostic value of ISN/RPS classification is controversial. Therefore, we aimed to identify clinical and pathological predictors of outcome in LN patients independent of this classification. Material and methods Thirty-seven patients with LN who underwent percutaneous kidney biopsy between 1997 and 2016 were included in this study...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29755712/lymphoproliferative-syndromes-associated-with-human-herpesvirus-6a-and-human-herpesvirus-6b
#17
REVIEW
Eva Eliassen, Gerhard Krueger, Mario Luppi, Dharam Ablashi
Human herpesvirus 6A and 6B (HHV-6A and HHV-6B) have been noted since their discovery for their T-lymphotropism. Although it has proven difficult to determine the extent to which HHV-6A and HHV-6B are involved in the pathogenesis of many diseases, evidence suggests that primary infection and reactivation of both viruses may induce or contribute to the progression of several lymphoproliferative disorders, ranging from benign to malignant and including infectious mononucleosis-like illness, drug induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS), and nodular sclerosis Hodgkin's lymphoma...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29755457/androgen-induced-immunosuppression
#18
REVIEW
Melanie R Gubbels Bupp, Trine N Jorgensen
In addition to determining biological sex, sex hormones are known to influence health and disease via regulation of immune cell activities and modulation of target-organ susceptibility to immune-mediated damage. Systemic autoimmune disorders, such as systemic lupus erythematosus, rheumatoid arthritis, and multiple sclerosis are more prevalent in females, while cancer shows the opposite pattern. Sex hormones have been repeatedly suggested to play a part in these biases. In this review, we will discuss how androgens and the expression of functional androgen receptor affect immune cells and how this may dampen or alter immune response(s) and affect autoimmune disease incidences and progression...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29755324/effect-of-chronic-oxidative-stress-on-neuroinflammatory-response-mediated-by-cd4-t-cells-in-neurodegenerative-diseases
#19
REVIEW
Helena Solleiro-Villavicencio, Selva Rivas-Arancibia
In a state of oxidative stress, there is an increase of reactive species, which induce an altered intracellular signaling, leading to dysregulation of the inflammatory response. The inability of the antioxidant defense systems to modulate the proinflammatory response is key to the onset and progression of neurodegenerative diseases. The aim of this work is to review the effect of the state of oxidative stress on the loss of regulation of the inflammatory response on the microglia and astrocytes, the induction of different CD4+ T cell populations in neuroinflammation, as well as its role in some neurodegenerative diseases...
2018: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/29755292/decoding-common-features-of-neurodegenerative-disorders-from-differentially-expressed-genes-to-pathways
#20
Rabia Habib, Nighat Noureen, Neha Nadeem
Background: Neurodegeneration is a progressive/irreversible loss of neurons, building blocks of our nervous system. Their degeneration gradually collapses the entire structural and functional system manifesting in myriads of clinical disorders categorized as Neurodegenerative Disorders (NDs) such as Alzheimer's Disease, (AD), Parkinson's Disease (PD), Frontotemporal Dementia (FTD) and Amyotrophic Lateral Sclerosis (ALS). NDs are characterized by a puzzling interplay of molecular and cellular defects affecting subset of neuronal populations in specific affected brain areas...
May 2018: Current Genomics
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