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systemic sclerosis update

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https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#1
REVIEW
Jorre S Mertens, Marieke M B Seyger, Rogier M Thurlings, Timothy R D J Radstake, Elke M G J de Jong
Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated...
March 16, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28282924/autophagy-and-microglia-novel-partners-in-neurodegeneration-and-aging
#2
REVIEW
Ainhoa Plaza-Zabala, Virginia Sierra-Torre, Amanda Sierra
Autophagy is emerging as a core regulator of Central Nervous System (CNS) aging and neurodegeneration. In the brain, it has mostly been studied in neurons, where the delivery of toxic molecules and organelles to the lysosome by autophagy is crucial for neuronal health and survival. However, we propose that the (dys)regulation of autophagy in microglia also affects innate immune functions such as phagocytosis and inflammation, which in turn contribute to the pathophysiology of aging and neurodegenerative diseases...
March 9, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28235778/clinical-evoked-potentials-in-neurology-a-review-of-techniques-and-indications
#3
REVIEW
Agustina M Lascano, Patrice H Lalive, Martin Hardmeier, Peter Fuhr, Margitta Seeck
Evoked potentials (EPs) are a powerful and cost-effective tool for evaluating the integrity and function of the central nervous system. Although imaging techniques, such as MRI, have recently become increasingly important in the diagnosis of neurological diseases, over the past 30 years, many neurologists have continued to employ EPs in specific clinical applications. This review presents an overview of the recent evolution of 'classical' clinical applications of EPs in terms of early diagnosis and disease monitoring and is an extension of a previous review published in this journal in 2005 by Walsh and collaborators...
February 24, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28205373/pulmonary-hypertension-in-connective-tissue-diseases-an-update
#4
REVIEW
Ramya Aithala, Anoop G Alex, Debashish Danda
Pulmonary hypertension (PH) is a relatively commoner complication of systemic sclerosis (SSc) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases (CTD). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD-associated PH belongs to group 1 PH, also known as pulmonary arterial hypertension (PAH). Around 30% of scleroderma-related deaths are due to PAH. Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels...
February 16, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28131211/potential-pathophysiological-pathways-that-can-explain-the-positive-effects-of-exercise-on-fatigue-in-multiple-sclerosis-a-scoping-review
#5
REVIEW
Martin Langeskov-Christensen, Etienne J Bisson, Marcia L Finlayson, Ulrik Dalgas
BACKGROUND: Fatigue is one of the most common and most disabling symptoms of multiple sclerosis (MS). It is a multidimensional and complex symptom with multifaceted origins, involving both central and peripheral fatigue mechanisms. Exercise has proven to be safe for people with MS, with cumulating evidence supporting significant reductions in fatigue. However, the potential pathophysiological pathways that can explain the positive effects of exercise on fatigue in MS remain elusive. OBJECTIVES: The objectives were, in PwMS (1) to update the knowledge on the pathophysiology underlying primary and secondary fatigue, and (2) to discuss potential pathophysiological pathways that can explain the positive effects of exercise on MS fatigue...
February 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28130412/update-on-disease-modifying-therapies-for-multiple-sclerosis
#6
Diana L Vargas, William R Tyor
Multiple sclerosis (MS) is an autoimmune, demyelinating disease of the central nervous system (CNS). It predominantly affects young women and is one of the most common causes of disability in young adults. MS is characterized by formation of white matter lesions in the CNS as a result of inflammation, demyelination, and axonal loss. Treatment has been a focus of neurological research for over 60 years. A number of disease-modifying therapies (DMTs) have become available making MS a treatable disease. These compounds target the inflammatory response in MS...
January 27, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#7
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
December 9, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28058863/what-should-clinicians-know-about-the-use-of-glucocorticoids-in-systemic-sclerosis
#8
Michele Iudici
Glucocorticoids (GC) are drugs largely used by rheumatologists, but their utilization in systemic sclerosis (SSc) is still controversial for both the lack of evidence of their usefulness and the physicians and patients' concern about their related adverse events. Due to the rarity and the complexity of the disease, few studies investigating the role of GC in SSc have been performed. Thus, a huge variability in corticosteroid prescriptive practice among physicians involved in the care of SSc patients still exists worldwide...
January 6, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/27988845/an-updated-histological-classification-system-for-multiple-sclerosis-lesions
#9
Tanja Kuhlmann, Samuel Ludwin, Alexandre Prat, Jack Antel, Wolfgang Brück, Hans Lassmann
Multiple sclerosis is a complex and heterogeneous, most likely autoimmune, demyelinating disease of the central nervous system (CNS). Although a number of histological classification systems for CNS lesions have been used by different groups in recent years, no uniform classification exists. In this paper, we propose a simple and unifying classification of MS lesions incorporating many elements of earlier histological systems that aims to provide guidelines for neuropathologists and researchers studying MS lesions to allow for better comparison of different studies performed with MS tissue, and to aid in understanding the pathogenesis of the disease...
January 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/27941129/update-of-eular-recommendations-for-the-treatment-of-systemic-sclerosis
#10
(no author information available yet)
No abstract text is available yet for this article.
November 9, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27911122/an-update-on-the-use-of-cerebrospinal-fluid-analysis-as-a-diagnostic-tool-in-multiple-sclerosis
#11
Matteo Gastaldi, Elisabetta Zardini, Diego Franciotta
Intrathecal B-lymphocyte activation is a hallmark of multiple sclerosis (MS), a multi-factorial inflammatory-demyelinating disease of the central nervous system. Such activation has a counterpart in the cerebrospinal fluid (CSF) oligoclonal IgG bands (OCB), whose diagnostic role in MS has been downgraded within the current McDonald's criteria. With a theoretico-practical approach, the authors review the physiopathological basis of the CSF dynamics, and the state-of-the-art of routine CSF analysis and CSF biomarkers in MS...
January 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/27881062/antihypertensive-treatments-in-patients-affected-by-aortic-valve-stenosis
#12
Paolo Poggio, Gianluca Folesani, Giuseppe M Raffa, Paola Songia, Vincenzo Valenti, Veronika Myasoedova, Alessandro Parolari
Systemic hypertension and aortic valve stenosis (AVS) are both age-related diseases. Severe aortic stenosis affects ≈2% to 5% of adults age > 65 years. Systemic hypertension (HTN) is a frequent comorbidity in patients with AVS and is coexistent for a longer period of time before AVS is treated. Essential systemic hypertension, per se, plays an important role in the creation of lesions on the aortic side of the valve, the region of higher exposure to tension stress. The subsequent endothelial defect represents the principal site of inflammatory process and oxidative stress, leading to aortic sclerosis and calcification...
November 23, 2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27773488/update-on-infections-with-human-herpesviruses-6a-6b-and-7
#13
REVIEW
H Agut, P Bonnafous, A Gautheret-Dejean
Human herpesviruses 6A, 6B, and 7 (HHV-6A, HHV-6B, HHV-7) are genetically related to cytomegalovirus. They belong to the Roseolovirus genus and to the Betaherpesvirinae subfamily. They infect T cells, monocytes-macrophages, epithelial cells, and central nervous system cells. These viruses are ubiquitous and are responsible for lifelong chronic infections, most often asymptomatic, in the vast majority of the general adult population. HHV-6B is responsible for exanthema subitum, which is a benign disease of infants...
October 20, 2016: Médecine et Maladies Infectieuses
https://www.readbyqxmd.com/read/27757048/medical-cannabis-the-canadian-perspective
#14
REVIEW
Gordon D Ko, Sara L Bober, Sean Mindra, Jason M Moreau
Cannabis has been widely used as a medicinal agent in Eastern medicine with earliest evidence in ancient Chinese practice dating back to 2700 BC. Over time, the use of medical cannabis has been increasingly adopted by Western medicine and is thus a rapidly emerging field that all pain physicians need to be aware of. Several randomized controlled trials have shown a significant and dose-dependent relationship between neuropathic pain relief and tetrahydrocannabinol - the principal psychoactive component of cannabis...
2016: Journal of Pain Research
https://www.readbyqxmd.com/read/27658514/-non-criteria-neurologic-manifestations-of-antiphospholipid-syndrome-a-hidden-kingdom-to-be-discovered
#15
Md Asiful Islam, Fahmida Alam, Mohammad Amjad Kamal, Kah Keng Wong, Teguh Haryo Sasongko, Siew Hua Gan
Neurological manifestations or disorders associated with central nervous system (CNS) are one of the most common as well as important clinical characteristics of antiphospholipid syndrome (APS). Although in the last updated (2006) classification criteria of APS its neurological manifestations encompassed only transient ischemic attack (TIA) and stroke, diverse 'non-criteria' neurological disorders or manifestations (headache, migraine, bipolar disorder, transverse myelitis, dementia, chorea, epileptic seizures, multiple sclerosis, psychosis, cognitive impairment, Tourette's syndrome, parkinsonism, dystonia, transient global amnesia, obsessive compulsive disorder and leukoencephalopathy) have been observed in APS patients...
September 20, 2016: CNS & Neurological Disorders Drug Targets
https://www.readbyqxmd.com/read/27527809/progranulin-a-key-player-in-autoimmune-diseases
#16
REVIEW
Jinlong Jian, Guangfei Li, Aubryanna Hettinghouse, Chuanju Liu
Autoimmune disease encompasses an array of conditions with a variety of presentations and the involvement of multiple organs. Though the etiologies of many autoimmune conditions are unclear, uncontrolled inflammatory immune response is believed to be a major cause of disease development and progression. Progranulin (PGRN), an anti-inflammatory molecule with therapeutic effect in inflammatory arthritis, was identified as an endogenous antagonist of TNFα by competitively binding to TNFR. PGRN exerts its anti-inflammatory activity through multiple pathways, including induction of Treg differentiation and IL-10 expression and inhibition of chemokine release from macrophages...
August 12, 2016: Cytokine
https://www.readbyqxmd.com/read/27464769/the-influence-of-different-solvents-on-systemic-sclerosis-an-updated-meta-analysis-of-14-case-control-studies
#17
Jiu-Hua Zhao, Yu Duan, Yu-Jie Wang, Xiao-Lei Huang, Guo-Jun Yang, Jing Wang
BACKGROUND: Several studies have collected detailed data to examine which specific solvents account for the association between solvents and risk of systemic sclerosis (SSc). These studies generally reported elevated risks associated with many of the specific solvents examined, such as toluene, xylene, and trichloroethylene. The previous meta-analysis was not able to conduct separate analyses for specific solvent subtypes. OBJECTIVE: The aims of the new meta-analysis were to investigate a more comprehensive estimate and to consider the effect of different solvents on SSc...
August 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/27432676/multiple-sclerosis
#18
REVIEW
Massimo Filippi, Paolo Preziosa, Maria A Rocca
Due to its sensitivity to the different multiple sclerosis (MS)-related abnormalities, magnetic resonance imaging (MRI) has become an established tool to diagnose MS and to monitor its evolution. MRI has been included in the diagnostic workup of patients with clinically isolated syndromes suggestive of MS, and ad hoc criteria have been proposed and are regularly updated. In patients with definite MS, the ability of conventional MRI techniques to explain patients' clinical status and progression of disability is still suboptimal...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27228639/vitamin-d-and-systemic-lupus-erythematosus-myth-or-reality
#19
REVIEW
Abdulla Watad, Shana G Neumann, Alessandra Soriano, Howard Amital, Yehuda Shoenfeld
There is growing interest in the contribution of vitamin D deficiency to autoimmunity. Several studies have shown an association between low levels of vitamin D and autoimmune disorders, including multiple sclerosis, rheumatoid arthritis, type 1 diabetes, autoimmune thyroid diseases, celiac disease, and systemic lupus erythematosus (SLE). Vitamin D receptor ligands can mediate immunosuppressive effects. It has been suggested that low levels of this hormone contribute to the immune activation in lupus and other autoimmune diseases...
March 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/27192495/the-keap1-nrf2-are-pathway-as-a-potential-preventive-and-therapeutic-target-an-update
#20
REVIEW
Meng-Chen Lu, Jian-Ai Ji, Zheng-Yu Jiang, Qi-Dong You
The Keap1-Nrf2-ARE ((Kelch-like ECH-Associating protein 1) nuclear factor erythroid 2 related factor 2-antioxidant response element) pathway is one of the most important defense mechanisms against oxidative and/or electrophilic stresses, and it is closely associated with inflammatory diseases, including cancer, neurodegenerative diseases, cardiovascular diseases, and aging. In recent years, progress has been made in strategies aimed at modulating the Keap1-Nrf2-ARE pathway. The Nrf2 activator DMF (Dimethylfumarates) has been approved by the FDA as a new first-line oral drug to treat patients with relapsing forms of multiple sclerosis, while a phase 3 study of another promising candidate, CDDO-Me, was terminated for safety reasons...
September 2016: Medicinal Research Reviews
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