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Diffuse Sclerosis

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Carbonic anhydrase IX as a novel candidate in liquid biopsy.

Ozen Ozensoy Guler, Claudiu T Supuran, Clemente Capasso,

Among the diagnostic techniques for the identification of tumour biomarkers, the liquid biopsy is considered one that offers future research on precision diagnosis and treatment of tumours in a non-invasive manner. The approach consists of isolating tumor-derived components, such as circulating tumour cells (CTC), tumour cell-free DNA (ctDNA), and extracellular ... Read more >>

J Enzyme Inhib Med Chem (Journal of enzyme inhibition and medicinal chemistry)
[2020, 35(1):255-260]

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Clinical course of Japanese patients with early systemic sclerosis: a multicenter, prospective, observational study.

Akira Utsunomiya, Minoru Hasegawa, Noritaka Oyama, Yoshihide Asano, Hirahito Endo, Manabu Fujimoto, Daisuke Goto, Osamu Ishikawa, Yasushi Kawaguchi, Masataka Kuwana, Fumihide Ogawa, Hiroki Takahashi, Sumiaki Tanaka, Shinichi Sato, Kazuhiko Takehara, Hironobu Ihn,

Objectives: To investigate the clinical course of Japanese patients with early diffuse cutaneous systemic sclerosis (dcSSc) and early SSc with interstitial lung disease (ILD).Methods: We prospectively analyzed the clinical features of 207 Japanese patients with early dcSSc (n = 150) and limited cutaneous SSc (lcSSc) with ILD (n = 57) in 10 medical centers ... Read more >>

Mod Rheumatol (Modern rheumatology)
[2020, :1-20]

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Management of systemic sclerosis: the first five years.

David Roofeh, Dinesh Khanna,

PURPOSE OF REVIEW:This review provides a risk-stratified and evidence-based management for subsets of systemic sclerosis (SSc) patients in the first five years from disease onset. RECENT FINDINGS:Cardiopulmonary disease remains the primary cause of mortality in SSc patients. Morbidity and mortality in SSc-associated pulmonary arterial hypertension have improved with combination treatment, ... Read more >>

Curr Opin Rheumatol (Current opinion in rheumatology)
[2020, 32(3):228-237]

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Successful treatment of systemic sclerosis complicated by ventricular tachycardia with a cardiac resynchronization therapy-defibrillator: A case report.

Yuan-Yuan Chen, Hui Yan, Jian-Hua Zhu,

BACKGROUNDSystemic sclerosis is a rare connective tissue disease characterized by localized or diffuse skin thickening and fibrosis, which usually accumulates in various organs throughout the body. Tachyarrhythmia is a common clinical manifestation of cardiovascular damage in systemic sclerosis patients. However, few studies have reported the use of catheter ablation and ... Read more >>

World J Clin Cases (World Journal of Clinical Cases)
[2020, 8(6):1129-1136]

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Drugs in phase I and phase II clinical trials for systemic sclerosis.

Melody P Chung, Lorinda Chung,

Introduction: Systemic sclerosis (SSc) is an autoimmune connective tissue disease that is characterized by excessive collagen deposition, vascular dysfunction, and fibrosis of cutaneous and visceral organs. Current therapeutic options are limited and provide only modest benefit.Areas covered: This review summarizes investigational agents in recent Phase I and II clinical trials ... Read more >>

Expert Opin Investig Drugs (Expert opinion on investigational drugs)
[2020, :1-14]

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Cognitive processing speed in multiple sclerosis clinical practice: association with patient-reported outcomes, employment, and MRI metrics.

Gabrielle Macaron, Laura E Baldassari, Kunio Nakamura, Stephen M Rao, Marisa P McGinley, Brandon P Moss, Hong Li, Deborah M Miller, Stephen E Jones, Robert A Bermel, Jeffrey A Cohen, Daniel Ontaneda, Devon S Conway,

OBJECTIVE:To analyze the relationship between cognitive processing speed, patient-reported outcome measures (PROMs), employment, and MRI metrics in a large multiple sclerosis cohort METHODS: Cross-sectional clinical data, PROMs, employment, and MRI studies within 90 days of completion of the Processing Speed Test (PST), a technology-enabled adaptation of the Symbol Digit Modalities ... Read more >>

Eur. J. Neurol. (European journal of neurology)
[2020, :]

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[Treatment of systemic sclerosis-associated interstitial lung disease].

A Prasse, F Bonella, U Müller-Ladner, T Witte, N Hunzelmann, J Distler,

BACKGROUND:Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In ... Read more >>

Z Rheumatol (Zeitschrift fur Rheumatologie)
[2020, 79(3):294-303]

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Therapeutic efficacy of combined glucocorticoid, intravenous cyclophosphamide, and double-filtration plasmapheresis for skin sclerosis in diffuse systemic sclerosis.

Kensuke Suga, Hiroyuki Yamashita, Yuko Takahashi, Daisuke Katagiri, Fumihiko Hinoshita, Hiroshi Kaneko,

We treated skin sclerosis with triple therapy consisting of a glucocorticoid, intravenous cyclophosphamide, and double-filtration plasmapheresis. The objective of this study was to analyze its effectiveness in a case series of patients who received triple therapy.We enrolled 8 patients with diffuse cutaneous systemic sclerosis (dcSSc) who received triple therapy at ... Read more >>

Medicine (Baltimore) (Medicine)
[2020, 99(10):e19301]

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Nintedanib in patients with systemic sclerosis associated interstitial lung disease: A Japanese population analysis of the SENSCIS trial.

Masataka Kuwana, Takashi Ogura, Shigeki Makino, Sakae Homma, Yasuhiro Kondoh, Aiko Saito, Hiroyuki Ugai, Martina Gahlemann, Kazuhiko Takehara, Arata Azuma,

Objective: We examined the efficacy and safety of nintedanib in Japanese patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) in the global Safety and Efficacy of Nintedanib in Systemic Sclerosis (SENSCIS) trial.Methods: Randomised patients received oral nintedanib 150 mg (N = 34) twice daily or placebo (N = 36) until the last patient reached 52 ... Read more >>

Mod Rheumatol (Modern rheumatology)
[2020, :1-26]

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Benzothiazole derivatives as anticancer agents.

Ali Irfan, Fozia Batool, Syeda Andleeb Zahra Naqvi, Amjad Islam, Sameh M Osman, Alessio Nocentini, Siham A Alissa, Claudiu T Supuran,

Benzothiazole (BTA) belongs to the heterocyclic class of bicyclic compounds. BTA derivatives possesses broad spectrum biological activities such as anticancer, antioxidant, anti-inflammatory, anti-tumour, antiviral, antibacterial, anti-proliferative, anti-diabetic, anti-convulsant, analgesic, anti-tubercular, antimalarial, anti-leishmanial, anti-histaminic and anti-fungal among others. The BTA scaffolds showed a crucial role in the inhibition of the metalloenzyme ... Read more >>

J Enzyme Inhib Med Chem (Journal of enzyme inhibition and medicinal chemistry)
[2020, 35(1):265-279]

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Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease-a randomised controlled trial.

Nupoor Acharya, Shefali Khanna Sharma, Debashish Mishra, Sahajal Dhooria, Varun Dhir, Sanjay Jain,

To assess the efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease (SSc-ILD). This was a double-blind, randomised, placebo-controlled, pilot study. Subjects with SSc-ILD and forced vital capacity (FVC) between 50 and 80% of the predicted (%pred) value were randomised in 1:1 ratio to receive either pirfenidone (2400 mg/day) ... Read more >>

Rheumatol. Int. (Rheumatology international)
[2020, :]

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Quantitative computed tomography assessment for systemic sclerosis-related interstitial lung disease: comparison of different methods.

Furkan Ufuk, Mahmut Demirci, Goksel Altinisik,

OBJECTIVES:To compare the previously defined six different histogram-based quantitative lung assessment (QLA) methods on high-resolution CT (HRCT) in patients with systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS:The HRCT images of SSc patients with ILD were reviewed, and the visual ILD score (semiquantitative) and the severity of ILD (limited or ... Read more >>

Eur Radiol (European Radiology)
[2020, :]

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Clinical significance of endothelial vasodilatory function evaluated by EndoPAT in patients with systemic sclerosis.

Naohiko Aozasa, Masaru Hatano, Ryosuke Saigusa, Kouki Nakamura, Takehiro Takahashi, Tetsuo Toyama, Hayakazu Sumida, Zenshiro Tamaki, Hisataka Maki, Shun Minatsuki, Issei Komuro, Shinichi Sato, Yoshihide Asano,

Endothelial dysfunction is a hallmark of vasculopathy associated with systemic sclerosis (SSc). Reactive hyperemia peripheral arterial tonometry is a rapid and non-invasive technique to assess peripheral microvascular endothelial function by measuring changes in digital pulse volume during reactive hyperemia. Low scores of the reactive hyperemia index (RHI) imply an impaired ... Read more >>

J. Dermatol. (The Journal of dermatology)
[2020, :]

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Patient and Physician Perspectives on Systemic Sclerosis-Associated Interstitial Lung Disease.

Tariq J Cheema, Meilin Young, Erica Rabold, Ashley N Barbieri, Nancy Baldwin, Virginia D Steen,

Systemic sclerosis-associated interstitial lung disease is challenging to diagnose and treat. Patients and physicians can perceive the disease differently and have different views on its management. Communication issues between them can lead to suboptimal disease management. Despite a clear need for improvement in the speed and accuracy of the diagnostic ... Read more >>

Clin Med Insights Circ Respir Pulm Med (Clinical medicine insights. Circulatory, respiratory and pulmonary medicine)
[2020, 14:1179548420913281]

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Gastrointestinal Emergencies.

Ashis Banerjee,

OropharyngealNeurological motility disorder: stroke; movement disorders (Parkinson’s disease, progressive supranuclear palsy); amyotrophic lateral sclerosis; multiple sclerosis; bulbar palsy; brainstem tumour; pseudobulbar palsyStriated muscle disease: myasthenia gravis; myotonic dystrophy; polymyositis; dermatomyositis; inflammatory myopathy; muscular dystrophyReduced salivary flow leading to dry mouth (xerostomia): Sjogren’s syndrome; anticholinergics; antihistamines; ACE inhibitors; alpha-adrenergic blockersStructural lesions: ... Read more >>

(Emergency Clinical Diagnosis)
[0, :235-260]

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Conversion of diffusely abnormal white matter to focal lesions is linked to progression in secondary progressive multiple sclerosis.

Mahsa Dadar, Sridar Narayanan, Douglas L Arnold, D Louis Collins, Josefina Maranzano,

BACKGROUND:Diffusely abnormal white matter (DAWM) regions are observed in magnetic resonance images of secondary progressive multiple sclerosis (SPMS) patients. However, their role in clinical progression is still not established. OBJECTIVES:To characterize the longitudinal volumetric and intensity evolution of DAWM and focal white matter lesions (FWML) and assess their associations with ... Read more >>

Mult. Scler. (Multiple sclerosis (Houndmills, Basingstoke, England))
[2020, :1352458520912172]

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Comment on: Skin improvement is a surrogate for favourable changes in other organ systems in early diffuse cutaneous systemic sclerosis.

Silvia Bosello, Elisa Gremese, Gianfranco Ferraccioli,

Rheumatology (Oxford) (Rheumatology (Oxford, England))
[2020, :]

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Usefulness of diffusion tensor imaging findings as biomarkers for amyotrophic lateral sclerosis.

Seol-Hee Baek, Jinseok Park, Yoo Hwan Kim, Hung Youl Seok, Ki-Wook Oh, Hee-Jin Kim, Ye-Ji Kwon, Youngbo Sim, Woo-Suk Tae, Seung Hyun Kim, Byung-Jo Kim,

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. However, no reliable biomarkers have been identified to represent the clinical status. This study aimed to investigate whether diffusion tensor imaging (DTI) findings are useful imaging biomarkers to indicate the clinical status of ALS patients. Ninety-six probable or definite ALS cases and ... Read more >>

Sci Rep (Scientific reports)
[2020, 10:]

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The profiles of miRNAs and lncRNAs in peripheral blood neutrophils exosomes of diffuse cutaneous systemic sclerosis.

Liya Li, Xiaoxia Zuo, Di Liu, Hui Luo, Honglin Zhu,

BACKGROUND:Diffuse cutaneous systemic sclerosis (dSSc) is a systemic autoimmune disease with skin fibrosis. Neutrophils display important roles in autoimmunity, inflammation, vasculopathy and fibrosis. Exosomes (EXOs) are cell-derived vesicles contained various noncoding RNAs, mRNA and proteins with biological roles. OBJECTIVE:To investigate the roles of miRNAs and lncRNAs from dSSc neutrophils EXOs. ... Read more >>

J. Dermatol. Sci. (Journal of dermatological science)
[2020, :]

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Treatment modalities and drug survival in a systemic sclerosis real-life patient cohort.

S. Panopoulos, Κ. Chatzidionysiou, M. G. Tektonidou, V. K. Bournia, A. A. Drosos, Stamatis-Nick C. Liossis, T. Dimitroulas, L. Sakkas, D. Boumpas, P. V. Voulgari, D. Daoussis, K. Thomas, G. Georgiopoulos, G. Vosvotekas, Α. Garyfallos, P. Sidiropoulos, G. Bertsias, D. Vassilopoulos, P. P. Sfikakis,

BackgroundEuropean data indicate that systemic sclerosis (SSc)-related death rates are increasing, thus raising concerns about SSc’s optimal management. Herein, we describe current treatment modalities and drug survival in a real-life SSc cohort.MethodsDetails on immunosuppressive/antiproliferative (methotrexate, mycophenolate, cyclophosphamide, azathioprine, rituximab, tocilizumab) and vasoactive agent [(endothelin receptor antagonists (ERAs), sildenafil, iloprost, and ... Read more >>

Arthritis Res. Ther. (Arthritis Research & Therapy)
[2020, 22:]

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Prevalence of GLA gene mutations and polymorphisms in patients with multiple sclerosis: A cross-sectional study.

Camilla Russo, Sirio Cocozza, Eleonora Riccio, Giuseppe Pontillo, Luigi Annicchiarico Petruzzelli, Roberta Lanzillo, Letizia Spinelli, Paolo Colomba, Giovanni Duro, Massimo Imbriaco, Cinzia Valeria Russo, Giulia De Riso, Teodolinda Di Risi, Enrico Tedeschi, Alberto Cuocolo, Arturo Brunetti, Vincenzo Brescia Morra, Sergio Cocozza, Antonio Pisani,

PURPOSE:Fabry Disease (FD) has been frequently proposed as possible underestimated differential diagnosis of Multiple Sclerosis (MS), but no study has been performed to test prevalence of GLA gene mutations in a population fulfilling diagnostic criteria of MS. Aim of this study is to determine the prevalence of GLA gene mutations ... Read more >>

J. Neurol. Sci. (Journal of the neurological sciences)
[2020, 412:116782]

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Comment on: Skin improvement is a surrogate for favourable changes in other organ systems in early diffuse systemic sclerosis: reply.

Tatiana Nevskaya, Boyang Zheng, Carl Baxter, Dena Ramey, Janet E Pope, Murray Baron,

Rheumatology (Oxford) (Rheumatology (Oxford, England))
[2020, :]

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Systemic sclerosis-associated interstitial lung disease.

Apostolos Perelas, Richard M Silver, Andrea V Arrossi, Kristin B Highland,

Systemic sclerosis is an autoimmune connective tissue disease, which is characterised by immune dysregulation and progressive fibrosis that typically affects the skin, with variable internal organ involvement. It is a rare condition that affects mostly young and middle-aged women, resulting in disproportionate morbidity and mortality. Currently, interstitial lung disease is ... Read more >>

Lancet Respir Med (The Lancet. Respiratory medicine)
[2020, 8(3):304-320]

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Distribution of Cortical Diffusion Tensor Imaging Changes in Multiple Sclerosis.

Benjamin Stock, Manoj Shrestha, Alexander Seiler, Christian Foerch, Elke Hattingen, Helmuth Steinmetz, Ralf Deichmann, Marlies Wagner, René-Maxime Gracien,

Purpose:Diffuse cortical damage in relapsing-remitting multiple sclerosis (RRMS) is clinically relevant but cannot be directly assessed with conventional MRI. In this study, it was aimed to use diffusion tensor imaging (DTI) techniques with optimized intrinsic eddy current compensation to quantify and characterize cortical mean diffusivity (MD) and fractional anisotropy (FA) ... Read more >>

Front Physiol (Frontiers in physiology)
[2020, 11:116]

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A Rare Presentation of Renal Impairment in Diffuse Cutaneous Systemic Sclerosis.

Kamal Kishor Solanki, Duncan Lamont, Douglas H N White,

J Clin Rheumatol (Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases)
[2020, :]

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