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Scleroderma

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https://www.readbyqxmd.com/read/28091808/occupational-and-environmental-scleroderma-systematic-review-and-meta-analysis
#1
Manuel Rubio-Rivas, Rafael Moreno, Xavier Corbella
The etiology of systemic sclerosis (SSc) remains unknown; however, several occupational and environmental factors have been implicated. Our objective was to perform a meta-analysis of all studies published on SSc associated with occupational and environmental exposure. The review was undertaken by means of MEDLINE and SCOPUS from 1960 to 2014 and using the terms: "systemic," "scleroderma," or "systemic sclerosis/chemically induced" [MesH]. The Newcastle-Ottawa Scale was used for the qualifying assessment. The inverse variance-weighted method was performed...
January 14, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28091699/musculoskeletal-mri-findings-of-juvenile-localized-scleroderma
#2
Eric P Eutsler, Daniel B Horton, Monica Epelman, Terri Finkel, Lauren W Averill
BACKGROUND: Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes. OBJECTIVE: There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children...
January 14, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28090307/hemodynamic-response-to-treatment-of-iron-deficiency-anemia-in-pulmonary-arterial-hypertension-longitudinal-insights-from-an-implantable-hemodynamic-monitor
#3
Muddassir Mehmood, Richa Agarwal, Amresh Raina, Priscilla Correa-Jaque, Raymond L Benza
Despite new therapeutic options, pulmonary arterial hypertension (PAH) remains a progressive disease associated with substantial morbidity and mortality. As such, additional strategies for monitoring and adjunctive management of this disease are important. A 59-year-old woman with scleroderma-associated PAH received an implantable hemodynamic monitor (IHM) as part of a research protocol at our institution. Pulmonary artery pressures, heart rate, and cardiac output (sensor-based algorithm) were measured on a daily basis, and parameters of right ventricular (RV) performance and afterload were calculated...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28089973/malignancies-in-patients-with-anti-rna-polymerase-iii-antibodies-and-systemic-sclerosis-analysis-of-the-eular-scleroderma-trials-and-research-cohort-and-possible-recommendations-for-screening
#4
Maria-Grazia Lazzaroni, Ilaria Cavazzana, Enrico Colombo, Rucsandra Dobrota, Jasmin Hernandez, Roger Hesselstrand, Cecilia Varju, Gabriella Nagy, Vanessa Smith, Paola Caramaschi, Valeria Riccieri, Eric Hachulla, Alexandra Balbir-Gurman, Emmanuel Chatelus, Katarzyna Romanowska-Próchnicka, Ana Carolina Araújo, Oliver Distler, Yannick Allanore, Paolo Airò
OBJECTIVE: To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)- positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients. METHODS: (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#5
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/28079847/relationship-between-abnormalities-on-high-resolution-computerized-tomography-pulmonary-function-and-bronchoalveolar-lavage-in-progressive-systemic-sclerosis
#6
Gulfidan Cakmak, Tuba Selcuk Can, Sule Gundogdu, Canan Akman, Hande Ikitimur, Benan Musellim, Gul Ongen
:  Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079844/serum-interleukin-6-in-systemic-sclerosis-and-its-correlation-with-disease-parameters-and-cardiopulmonary-involvement
#7
Rasha A Abdel-Magied, Shereen R Kamel, Azza Farag Said, Hazem M Ali, Ehab A Abdel Gawad, Mahmoud M Moussa
OBJECTIVE: To assess serum interleukin-6 (IL-6)level in patients with systemic sclerosis (SSc) and its correlations with European Scleroderma Study Group activity score (EUSTAR), Scleroderma Assessment Questionnaire (SAQ), disability index and cardiopulmonary involvement. METHODS: Twenty SSc patients and 10 matched healthy controls were included. Serum IL-6 was measured in patients and controls. Disease activity, status,and disability were assessed.Cardiopulmonary involvement was evaluated by pulmonary function tests (PFTs), six minute walk test, echocardiography, and high resolution computed tomography (HRCT) of chest...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28077400/differential-expression-of-hedgehog-and-snail-in-cutaneous-fibrosing-disorders-implications-for-targeted-inhibition
#8
Amrita Goyal, Katy R Linskey, Jonathan Kay, Lyn M Duncan, Rosalynn M Nazarian
OBJECTIVES: To examine Hedgehog signaling in cutaneous fibrosing disorders for which effective approved therapies are lacking, expand our knowledge of pathophysiology, and explore the rationale for targeted inhibition. METHODS: Stain intensity and percentage of cells staining for Sonic hedgehog (Shh), Indian hedgehog (Ihh), Patched (Ptch), glycogen synthase kinase 3 β (GSK3-β), β-catenin, and Snail were evaluated in human skin biopsy specimens of keloid, hypertrophic scar (Hscar), scleroderma, nephrogenic systemic fibrosis (NSF), scar, and normal skin using a tissue microarray...
December 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28068643/validation-of-the-body-concealment-scale-for-scleroderma-bcss-replication-in-the-scleroderma-patient-centered-intervention-network-spin-cohort
#9
Lisa R Jewett, Linda Kwakkenbos, Marie-Eve Carrier, Vanessa L Malcarne, Diana Harcourt, Nichola Rumsey, Maureen D Mayes, Shervin Assassi, Annett Körner, Rina S Fox, Shadi Gholizadeh, Sarah D Mills, Catherine Fortune, Brett D Thombs
Body concealment is an important component of appearance distress for individuals with disfiguring conditions, including scleroderma. The objective was to replicate the validation study of the Body Concealment Scale for Scleroderma (BCSS) among 897 scleroderma patients. The factor structure of the BCSS was evaluated using confirmatory factor analysis and the Multiple-Indicator Multiple-Cause model examined differential item functioning of SWAP items for sex and age. Internal consistency reliability was assessed via Cronbach's alpha...
January 6, 2017: Body Image
https://www.readbyqxmd.com/read/28068631/microrna-202-3p-regulates-scleroderma-fibrosis-by-targeting-matrix-metalloproteinase-1
#10
Bin Zhou, Honglin Zhu, Hui Luo, Siming Gao, Xiaodan Dai, Yisha Li, Xiaoxia Zuo
The leading cause of death in systemic sclerosis (SSc) is the uncontrolled fibrosis in multiple organs. The exact mechanism of fibrosis is not fully clear. Our previous studies using miRNA array analysis indicated that miR-202-3p was increased in SSc lesion skin tissues. Bioinformatics analysis suggested matrix metallopeptidase (MMP) 1 is the target gene of miR-202-3p. Here we confirmed that miR-202-3p was upregulated, and the mRNA and protein expression of MMP1 were significantly decreased in SSc skin tissues and primary fibroblast compared with normal skin...
January 6, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28065485/systemic-sclerosis-sine-scleroderma-in-mexican-patients-case-reports
#11
Olga Vera-Lastra, Christian Alexis Sauceda-Casas, María Del Pilar Cruz Domínguez, Sergio Alberto Mendoza Alvarez, Jesús Sepulceda-Delgado
: Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10. CONCLUSION: In patients with RP, esophageal dysmotility, interstitial lung disease and pulmonary arterial hypertension should be tested for ACA in order to establish a prompt diagnosis and treatment of ssSSc...
January 3, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28058708/platelet-rich-gel-for-the-adjuvant-treatment-of-wound-healing-of-transposed-flap-for-arteriovenous-fistula-in-systemic-scleroderma
#12
Salvatore Buscemi, Salvatore De Luca, Salvatore Fazzotta, Attilio Ignazio Lo Monte
No abstract text is available yet for this article.
January 3, 2017: Journal of Vascular Access
https://www.readbyqxmd.com/read/28058539/validation-of-the-ucla-scleroderma-clinical-trial-consortium-gastrointestinal-tract-instrument-2-0-in-english-and-chinese-speaking-patients-in-a-multi-ethnic-singapore-systemic-sclerosis-cohort
#13
Andrea Hsiu Ling Low, Xiaohui Xin, Weng Giap Law, Gim Gee Teng, Amelia Santosa, Anita Lim, Grace Chan, Swee Cheng Ng, Julian Thumboo
The aim of this study was to (1) translate the Gastrointestinal Tract Instrument (GIT) 2.0 from English to Chinese and (2) validate both versions in a multi-ethnic systemic sclerosis cohort in Singapore (SCORE). The English GIT2.0 was translated to Chinese using a standard forward-backward translation approach. Psychometric evaluation of the GIT2.0 included internal consistency reliability (using Cronbach's alpha), test-retest reliability (using intra-class correlation coefficient (ICC)), scale level factor analysis, and construct validity (using Spearman correlation) against the modified Scleroderma Health Assessment Questionnaire (S-HAQ) and the SF-36 v2...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28056626/nailfold-capillaroscopic-changes-in-patients-with-systemic-lupus-erythematosus-correlations-with-disease-activity-skin-manifestation-and-nephritis
#14
S Shenavandeh, S Habibi
Introduction The clinical expression of systemic lupus erythematosus (SLE) is the consequence of endothelial cell damage leading to serious multiple organ dysfunction. The aim of this study was to assess the association between nailfold capillaroscopic changes and disease activity, skin and renal involvement in patients with SLE. Methods Demographic variables, clinical manifestations and laboratory data of 108 patients with SLE were investigated. Nailfold capillaroscopy (NFC) was performed in all patients. Result Morphological changes in NFC were observed in 102 out of 108 (94...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28052367/morphoea-with-prominent-plasma-cell-endoneuritis
#15
M Singh, N Farquharson, C Owen, A J Howat, S Singh, N Francis, E Calonje
Morphoea (localized scleroderma) is a cutaneous inflammatory condition characterized by the development of indurated and discoloured plaques. The histological features of morphoea typically include a superficial and deep perivascular and periadnexal chronic inflammatory infiltrate associated with variable degrees of dermal and/or subcutaneous sclerosis. The infiltrate is typically composed of lymphocytes, macrophages and conspicuous plasma cells. The early stages of morphoea may have a very prominent inflammatory infiltrate associated with subtle sclerosis...
January 4, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28038680/changes-in-plasma-cxcl4-levels-are-associated-with-improvements-in-lung-function-in-patients-receiving-immunosuppressive-therapy-for-systemic-sclerosis-related-interstitial-lung-disease
#16
Elizabeth R Volkmann, Donald P Tashkin, Michael D Roth, Philip J Clements, Dinesh Khanna, Daniel E Furst, Maureen Mayes, Julio Charles, Chi-Hong Tseng, Robert M Elashoff, Shervin Assassi
BACKGROUND: Increased circulatory levels of the chemokine CXCL4 have been associated with the presence of interstitial lung disease (ILD) in an observational study of patients with systemic sclerosis (SSc). The purpose of the present study was to evaluate the relationship between baseline CXCL4 level and extent of ILD in the context of a randomized controlled trial and to determine whether changes in CXCL4 levels in response to immunosuppression are associated with future progression of SSc-ILD...
December 30, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28033278/delayed-onset-of-posterior-reversible-encephalopathy-syndrome-in-a-case-of-scleroderma-renal-crisis-with-maintenance-hemodialysis-case-report-and-literature-review
#17
Ching-Yang Chen, Shin-Yuan Hung, Yi-Jer Lee, Yi-Chan Lin, Chu-Cheng Pai
INTRODUCTION: In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28033115/quantification-of-differences-between-nailfold-capillaroscopy-images-with-a-scleroderma-pattern-and-normal-pattern-using-measures-of-geometric-and-algorithmic-complexity
#18
Samuel George Urwin, Bridget Griffiths, John Allen
This study aimed to quantify and investigate differences in the geometric and algorithmic complexity of the microvasculature in nailfold capillaroscopy (NFC) images displaying a scleroderma pattern and those displaying a 'normal' pattern. 11 NFC images were qualitatively classified by a capillary specialist as indicative of 'clear microangiopathy' (CM), i.e. a scleroderma pattern, and 11 as 'not clear microangiopathy' (NCM), i.e. a 'normal' pattern. Pre-processing was performed, and fractal dimension (FD) and Kolmogorov complexity (KC) were calculated following image binarisation...
December 29, 2016: Physiological Measurement
https://www.readbyqxmd.com/read/28029745/early-mortality-in-a-multinational-systemic-sclerosis-inception-cohort
#19
Yanjie Hao, Marie Hudson, Murray Baron, Patricia Carreira, Wendy Stevens, Candice Rabusa, Solene Tatibouet, Loreto Carmona, Beatriz E Joven, Molla Huq, Susanna Proudman, Mandana Nikpour
OBJECTIVE: To determine mortality and causes of death in a multinational inception systemic sclerosis (SSc) cohort. METHODS: We quantified mortality as Standardized Mortality Ratio (SMR), Years of Life Lost (YLL) and percentage mortality in the first decade of disease. The inception cohort was comprised of patients recruited within 4 years of disease onset. For comparison, we used a prevalent cohort, which included all patients irrespective of disease duration at recruitment...
December 28, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28026802/ethosuximide-induced-lupus-scleroderma-syndrome-with-disease-specific-autoantibodies
#20
Tetsuo Toyama, Yoshihide Asano, Takashi Taniguchi, Takehiro Takahashi, Yohei Ichimura, Zenshiro Tamaki, Shinji Kagami, Hiroshi Mitsui, Shinichi Sato
No abstract text is available yet for this article.
December 23, 2016: European Journal of Dermatology: EJD
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