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https://www.readbyqxmd.com/read/28431140/performance-of-the-patient-reported-outcomes-measurement-information-system-29-in-scleroderma-a-scleroderma-patient-centered-intervention-network-cohort-study
#1
Linda Kwakkenbos, Brett D Thombs, Dinesh Khanna, Marie-Eve Carrier, Murray Baron, Daniel E Furst, Karen Gottesman, Frank van den Hoogen, Vanessa L Malcarne, Maureen D Mayes, Luc Mouthon, Warren R Nielson, Serge Poiraudeau, Robert Riggs, Maureen Sauvé, Fredrick Wigley, Marie Hudson, Susan J Bartlett
Objective.: The Patient-Reported Outcomes Measurement Information System (PROMIS)-29 assesses seven health-related quality of life domains plus pain intensity. The objective was to examine PROMIS-29v2 validity and explore clinical associations in patients with SSc. Methods.: English-speaking SSc patients in the Scleroderma Patient-centered Intervention Network Cohort from 26 sites in Canada, the USA and the UK completed the PROMIS-29v2 between July 2014 and November 2015...
April 18, 2017: Rheumatology
https://www.readbyqxmd.com/read/28429663/finding-the-groove-the-groove-sign-in-scleroderma-and-related-disorders
#2
Alejandro Gómez Gómez, Alberto Cecconi
No abstract text is available yet for this article.
April 2017: Vascular Medicine
https://www.readbyqxmd.com/read/28426903/the-therapeutic-efficacy-of-botulinum-toxin-in-treating-scleroderma-associated-raynaud-s-phenomenon-a-randomized-double-blind-placebo-controlled-clinical-trial-assessing
#3
Ricardo J Bello, Carisa M Cooney, Eitan Melamed, Keith Follmar, Gayane Yenokyan, Gwendolyn Leatherman, Ami A Shah, Fredrick M Wigley, Laura K Hummers, Scott D Lifchez
Objective To assess the therapeutic efficacy of local injections with Botulinum Toxin Type-A (Btx-A) in improving blood flow to the hands of patients with Raynaud's Phenomenon (RP) secondary to scleroderma. Methods In this randomized, double-blind, placebo-controlled clinical trial (http://ClinicalTrials.gov #NCT02165111), patients with scleroderma-associated RP received Btx-A (50 units in 2.5 mL) in one randomly selected hand and sterile saline (2.5 mL) in the opposite hand. Follow-up at 1 and 4 months post-injection included Laser Doppler Imaging (LDI) of hands, patient-reported outcomes, and physical exam...
April 20, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28424250/unifying-mechanism-for-different-fibrotic-diseases
#4
Gerlinde Wernig, Shih-Yu Chen, Lu Cui, Camille Van Neste, Jonathan M Tsai, Neeraja Kambham, Hannes Vogel, Yaso Natkunam, D Gary Gilliland, Garry Nolan, Irving L Weissman
Fibrotic diseases are not well-understood. They represent a number of different diseases that are characterized by the development of severe organ fibrosis without any obvious cause, such as the devastating diseases idiopathic pulmonary fibrosis (IPF) and scleroderma. These diseases have a poor prognosis comparable with endstage cancer and are uncurable. Given the phenotypic differences, it was assumed that the different fibrotic diseases also have different pathomechanisms. Here, we demonstrate that many endstage fibrotic diseases, including IPF; scleroderma; myelofibrosis; kidney-, pancreas-, and heart-fibrosis; and nonalcoholic steatohepatosis converge in the activation of the AP1 transcription factor c-JUN in the pathologic fibroblasts...
April 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28420992/deficient-adipogenesis-of-scleroderma-patient-and-healthy-african-american-monocytes
#5
Rebecca Lee, Charles Reese, Gustavo Carmen-Lopez, Beth Perry, Michael Bonner, Marina Zemskova, Carole L Wilson, Kristi L Helke, Richard M Silver, Stanley Hoffman, Elena Tourkina
Monocytes from systemic sclerosis (SSc, scleroderma) patients and healthy African Americans (AA) are deficient in the regulatory protein caveolin-1 leading to enhanced migration toward chemokines and fibrogenic differentiation. While dermal fibrosis is the hallmark of SSc, loss of subcutaneous adipose tissue is a lesser-known feature. To better understand the etiology of SSc and the predisposition of AA to SSc, we studied the adipogenic potential of SSc and healthy AA monocytes. The ability of SSc and healthy AA monocytes to differentiate into adipocyte-like cells (ALC) is inhibited compared to healthy Caucasian (C) monocytes...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28418584/transforming-growth-factor-%C3%AE-inhibitor-repsox-down-regulates-collagen-expression-of-scleroderma-dermal-fibroblasts-and-prevents-bleomycin-induced-mice-skin-fibrosis
#6
Maho Ide, Masatoshi Jinnin, Yukiko Tomizawa, Zhongzhi Wang, Ikko Kajihara, Satoshi Fukushima, Yoshinobu Hashizume, Yoshihide Asano, Hironobu Ihn
Inhibition of transforming growth factor (TGF)-β1 signaling may be one of the most reliable approaches to treat skin fibrosis of scleroderma. Although there have been a lot of basic researches of TGF-β blockade reagents, few of them was proved to have inhibitory effects on fibrosis both in vitro and in vivo. In the present study, we randomly chose four commercially available low molecular compounds (Repsox, LY2109761, LY364947, and K02288) from TGF-β1 inhibitor library, and compared their anti-fibrotic effects in vitro and in vivo...
April 18, 2017: Experimental Dermatology
https://www.readbyqxmd.com/read/28414153/inflammatory-myopathy-associated-with-myasthenia-gravis-with-and-without-thymic-pathology-report-of-four-cases-and-literature-review
#7
REVIEW
Ernestina Santos, Ester Coutinho, Ana Martins da Silva, António Marinho, Carlos Vasconcelos, Ricardo Taipa, Manuel Melo Pires, Guilherme Gonçalves, Carlos Lopes, Maria Isabel Leite
INTRODUCTION: the association of myasthenia gravis (MG) and inflammatory myopathy is rare and often only one of the diseases is diagnosed. Thymus pathology may be in the origin of such disease association. METHODS: we described four patients with both MG and inflammatory myopathy. RESULTS: these cases correspond to 2.3% of our MG cohort. Case 1: MG, polymyositis and thymolipoma; case 2: MG and necrotizing myopathy without thymic pathology on a background of scleroderma, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia (CREST); case 3: MG and dermatomyositis without thymic pathology; case 4: MG and dermatomyositis with type C thymoma...
April 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#8
REVIEW
Christopher P Denton, Dinesh Khanna
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease that is characterised by fibrosis of the skin and internal organs and vasculopathy. Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved understanding of systemic sclerosis has allowed better management of the disease, including improved classification and more systematic assessment and follow-up. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis...
April 13, 2017: Lancet
https://www.readbyqxmd.com/read/28412704/structural-validity-of-the-rheumatology-attitudes-index-in-systemic-sclerosis-analysis-from-the-ucla-scleroderma-quality-of-life-study
#9
Shadi Gholizadeh, Sarah D Mills, Rina S Fox, Erin L Merz, Scott C Roesch, Philip J Clements, Suzanne Kafaja, Daniel E Furst, Dinesh Khanna, Vanessa L Malcarne
OBJECTIVE: To evaluate the structural validity of the Rheumatology Attitudes Index (RAI), a widely used measure of rheumatic disease-related helplessness in patients with systemic sclerosis (SSc). METHODS: Patients with physician-confirmed SSc from the University of California, Los Angeles (UCLA) Scleroderma Quality of Life Study (n = 208) received clinical examinations and completed self-report questionnaires. The structural validity of the RAI was examined through confirmatory and exploratory factor analysis (CFA/EFA)...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28412076/original-article-varicella-vaccination-elicits-a-humoral-and-cellular-response-in-children-with-rheumatic-diseases-using-immune-suppressive-treatment
#10
Noortje Groot, Gecilmara Pileggi, Cleonice B Sandoval, Ingrid Grein, Guy Berbers, Virginia Paes Leme Ferriani, Nico Wulffraat, Sytze de Roock
OBJECTIVE: To assess humoral and cellular responses to live-attenuated varicella zoster virus (VZV) vaccination of patients with juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) or juvenile scleroderma (JScle) compared to those of healthy controls (HC). METHODS: Before, 4-6weeks and one year after VZV vaccination, blood samples of patients and HC were collected. VZV-specific antibody concentrations were measured by ELISA and multiplex immune-assay...
April 12, 2017: Vaccine
https://www.readbyqxmd.com/read/28406085/a-single-center-kidney-transplant-experience-among-ten-caucasian-females-with-end-stage-renal-disease-due-to-scleroderma%C3%A2
#11
Nishkarsh Saxena, Arjang Djamali, Brad C Astor, Maha Mohamed, Didier Mandelbrot, Sandesh Parajuli
There is limited information on kidney transplant recipients with end-stage renal disease (ESRD) due to scleroderma. We conducted an observational study on kidney transplant recipients with ESRD due to scleroderma who received kidney transplant at our center between 01/1994 and 06/2013. During the study period, there were 10 kidney transplant recipients, all of whom were Caucasian females. Seven of them were living-donor kidney transplant recipients, and the mean age at time of transplant was 56.6 ± 11.99 years...
April 13, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28393380/th1-and-th17-polarized-immune-infiltrates-in-eosinophilic-fasciitis-a-potential-marker-for-histopathologic-distinction-from-morphea
#12
Andrea Primiani Moy, Elena Maryamchik, Olga V Nikolskaia, Rosalynn M Nazarian
Morphea (localized scleroderma) and eosinophilic fasciitis (EF) are rare fibrosing disorders which may present a diagnostic challenge. While histopathologic features are often distinct, in some cases there may be overlap. T-cells contribute to etiopathogenesis of both autoimmune conditions. We sought to determine whether T-cell immune polarization enables histopathologic distinction. We retrospectively examined clinicopathologically confirmed cases of morphea (n = 12) and EF (n = 8) using immunohistochemistry for CD3, CD8, and dual staining for CD4 with T-bet, GATA-3, STAT-3, or BNC-2 (transcription factors reported to be specific and mutually exclusive for Th1, Th2, Th17 and Th22 cells, respectively) to characterize the T-cell infiltrate...
April 10, 2017: Journal of Cutaneous Pathology
https://www.readbyqxmd.com/read/28386760/lung-transplantation-in-patients-with-systemic-sclerosis
#13
REVIEW
Rupal J Shah, Francesco Boin
PURPOSE OF REVIEW: The purpose of this study was to highlight recent findings on evaluation for lung transplantation and outcomes after transplantation in patients with systemic sclerosis (scleroderma). RECENT FINDINGS: The recognition that extra-pulmonary disease manifestations can seriously compromise post-transplant outcomes has changed the way patients are screened for lung transplant candidacy. Reluctance to transplant subjects affected by scleroderma has been driven by the complexity and multisystem nature of this disease...
May 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28386330/geniposide-inhibited-endothelial-mesenchymal-transition-via-the-mtor-signaling-pathway-in-a-bleomycin-induced-scleroderma-mouse-model
#14
Qing Qi, Yueping Mao, Yongzhen Tian, Ke Zhu, Xushan Cha, Minghua Wu, Xiaodong Zhou
AIM: Geniposide is an iridoid glycoside isolated from the gardenia plant. It has multiple biological activities. The roles of geniposide in systemic sclerosis (SSc) and in endothelial-to-mesenchymal transition (EndMT) are unclear. We investigated the protective effects of geniposide in a bleomycin-induced SSc mouse model, and its potential mechanisms. METHODS: The effects of geniposide were evaluated as follows: (1) histological and immunochemical changes in mouse skin tissue; (2) changes in cellular morphology of human umbilical vein endothelial cells (HUVECs); (3) expression of endothelial cell biomarkers (E-Cadherin, CD31, and CD34), mesenchymal cell markers (FSP1, Collagen, and α-SMA), and key factors of EndMT (Slug, Snail, and Twist) using real time PCR, Western blot, and immunofluorescence; (4) tube formation in HUVECs; (5) mTOR signaling pathway transcription factors using Western blot analysis...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28386138/nailfold-digital-capillaroscopic-findings-in-patients-with-diffuse-and-limited-cutaneous-systemic-sclerosis
#15
Saeedeh Shenavandeh, Mahyar Yousefipour Haghighi, Mohammad Ali Nazarinia
BACKGROUND: Systemic sclerosis (SSc) is a chronic disease with microvascular damage. Nailfold capillaroscopy is a non-invasive method used for evaluating capillaries in SSc. Its findings could be related to the internal organ involvement and SSc course. In this study, we aimed to determine the association of the capillaroscopic patterns of nailfold capillaries with the disease subtypes of SSc, disease duration, and clinical manifestations. MATERIAL AND METHODS: Seventy patients with SSc (15 cases with diffuse cutaneous SSc [DcSSc] and 55 patients with limited SSc [LcSSc]) were studied...
2017: Reumatologia
https://www.readbyqxmd.com/read/28376288/mycophenolate-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#16
Elizabeth R Volkmann, Donald P Tashkin, Ning Li, Michael D Roth, Dinesh Khanna, Anna-Maria Hoffmann-Vold, Grace Kim, Jonathan Goldin, Philip J Clements, Daniel E Furst, Robert M Elashoff
OBJECTIVE: To compare mycophenolate (MMF) with placebo for the treatment of systemic sclerosis-related interstitial lung disease (SSc-ILD). METHODS: Participants enrolled in the placebo arm of Scleroderma Lung Study (SLS) I and the MMF arm of SLS II were included. SLS I randomized participants to oral cyclophosphamide (CYC) versus placebo for 1 year, while SLS II randomized participants to MMF for 2 years versus oral CYC for 1 year followed by 1 year of placebo...
April 4, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28375832/fatigue-in-patients-with-systemic-sclerosis-and-hypothyroidism-a-review-of-the-literature-and-report-of-our-experience
#17
Alessandro Antonelli, Poupak Fallahi, Flavia Di Bari, Dilia Giuggioli, Silvia Martina Ferrari, Clodoveo Ferri
Persistent fatigue (defined as ongoing exhaustion, disproportionate to exertion and not adequately alleviated by rest) reduces health-related quality of life of systemic sclerosis (SSc) patients. Fatigue in SSc is associated with reduced capacity to carry out daily activities, work disability and impaired physical function. Clinical studies demonstrated a high prevalence of autoimmune thyroiditis and hypothyroidism in patients with SSc. Since hypothyroidism and the associated fatigue symptoms could be cured by L-thyroxine (L-T4) substitutive therapy, the evolution of fatigue symptoms in SSc hypothyroid patients treated with substitutive therapy has been recently evaluated, showing an amelioration of the fatigue symptoms...
April 4, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28374002/a-case-of-orf-disease-in-a-patient-with-scleroderma
#18
Danit Maor, Lawrence L Yu, Roland Brand
No abstract text is available yet for this article.
March 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28370513/high-dose-intravenous-immunoglobulin-therapy-for-scleromyxedema-a-prospective-open-label-clinical-trial-using-an-objective-score-of-clinical-evaluation-system
#19
A Guarneri, M Cioni, F Rongioletti
BACKGROUND: Scleromyxedema is a primary fibro-mucinosis whose therapy is still challenging. OBJECTIVE: To evaluate the safety and efficacy of high-dose intravenous immunoglobulin (IVIg) for the management of scleromyxedema prospectically using an objective score. METHODS: In a prospective open-label study, IVIg was administered to 8 patients with scleromyxedema in a dose of 2 gr/Kg per month. The patients were followed up to a minimum of 6 months and their disease activity and response to treatment were assessed using the Physician's Global Assessment of disease severity (PGA) and a modified objective skin scoring system for patients with scleroderma (modified Rodnan score system for scleromyxedema or mRSSS)...
March 29, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28370352/serum-levels-of-genomic-dna-of-%C3%AE-1-i-collagen-are-elevated-in-scleroderma-patients
#20
Soichiro Sawamura, Masatoshi Jinnin, Miki Shimbara, Kayo Nakamura, Hideo Kudo, Kuniko Inoue, Wakana Nakayama, Ikko Kajihara, Satoshi Fukushima, Hironobu Ihn
Recent studies have indicated that various nucleic acids are present in human sera, and attracted attention for their potential as novel disease markers in many human diseases. In this study, we tried to evaluate the possibility that DNA and RNA of collagens exist in human sera, and determined whether their serum levels can be useful biomarkers in scleroderma patients. The RNA or DNA of collagens were purified from sera, and detected by polymerase chain reaction or quantitated by real-time polymerase chain reaction...
March 31, 2017: Journal of Dermatology
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