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Scleroderma

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https://www.readbyqxmd.com/read/29168920/a-case-report-of-open-craniofacial-sutures-a-novel-feature-of-systemic-sclerosis
#1
Patricia Gorecki, John Rout
Scleroderma is an uncommon connective-tissue disease, its key feature being excessive collagen deposition resulting in fibrosis of the skin and internal organs. There are different types that can vary in severity from localized scleroderma to systemic sclerosis. Various clinical and radiographic findings can be attributed to the disease, which arise due to the progressive nature of microvascular changes and collagen deposition. These include limited mouth opening, xerostomia, periodontal disease and cardiac and pulmonary disease...
November 23, 2017: Dento Maxillo Facial Radiology
https://www.readbyqxmd.com/read/29166498/evaluation-of-mean-platelet-volume-in-localized-scleroderma
#2
Anil Gulsel Bahali, Ozlem Su, Nazan Emiroglu, Fatma Pelin Cengiz, Mehmet Onur Kaya, Nahide Onsun
BACKGROUND: Localized scleroderma is a chronic inflammatory skin disease characterized by sclerosis of the dermis and subcutaneous tissue. Platelets play an important role in inflammation. Following activation, platelets rapidly release numerous mediators and cytokines, which contribute to inflammation. OBJECTIVES: To evaluate whether there was any relation between localized scleroderma and platelet parameters. METHODS: Forty-one patients with localized scleroderma were enrolled in the study...
September 2017: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/29166431/coexistence-of-morphea-and-granuloma-annulare-a-rare-case-report
#3
Şenay Ağırgöl, Özge Yöntem, Cem Leblebici, Tuğba Özkök Akbulut, Filiz Topaloğlu Demir, Zafer Türkoğlu
CONTEXT: Localized scleroderma (morphea) is characterized by fibrosis of skin and subcutaneous tissue. Granuloma annulare is a relatively common disease that is characterized by dermal papules and arciform plaques. CASE REPORT: Here, we present the case of a 42-year-old woman who developed granuloma annulare on the dorsum of her feet and abdominal region, and morphea on the anterior side of her lower limbs. We also discuss the etiological and pathogenetic processes that may cause the rare coexistence of these two diseases...
November 17, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/29164837/serum-surfactant-protein-d-and-exhaled-nitric-oxide-as-biomarkers-of-early-lung-damage-in-systemic-sclerosis
#4
Alida Benfante, Riccardo Messina, Alessandra Paternò, Nicola Scichilone
BACKGROUND: Interstitial lung disease (ILD) complicates the course of systemic sclerosis (SSc), representing the main cause of death in these patients. The identification of parameters that can predict the early onset and progression of ILD in SSc represents an unmet need in clinical practice. The study was designed to explore whether the surfactant proteins (SP) A and D may be used as noninvasive tools for the early identification of ILD in SSc. Alveolar exhaled nitric oxide (NO) was investigated as a surrogate marker of distal inflammation...
November 21, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29164584/cardiac-magnetic-resonance-in-systemic-sclerosis-patients-with-cardiac-symptoms
#5
A Meduri, D V Di Molfetta, L Natale, R Manfredi
OBJECTIVE: Systemic sclerosis (SSc) is characterized by widespread vascular lesions and skin and internal organs fibrosis, including the heart; all cardiac layers, endocardium, myocardium, and pericardium, may be involved. We report the relevance of cardiac MRI findings in scleroderma patients with cardiac symptoms. PATIENTS AND METHODS: 50 patients, all fulfilling the ACR SSc criteria (19 with limited and 31 with diffused skin involvement) were evaluated using a 1...
November 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29160213/cutaneous-metastases-of-internal-malignancies-an-experience-from-a-single-institution
#6
Elena Guanziroli, Antonella Coggi, Luigia Venegoni, Daniele Fanoni, Giulia Ercoli, Francesca Boggio, Stefano Veraldi, Emilio Berti, Raffaele Gianotti, Stefano Ferrero, Alessandro Del Gobbo
BACKGROUND: Cutaneous metastases represent 2% of all skin tumours. Their recognition can be challenging, as they may present with different clinical features, with consequent frequent delay and failure in diagnosis. OBJECTIVES: To review our series of cutaneous metastatic lesions, analyse their frequency according to patient gender, histotype, localization of the primary tumour, and site of cutaneous metastasis, and correlate this data with clinicopathological parameters...
November 21, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29155994/work-productivity-in-systemic-sclerosis-its-economic-burden-and-association-with-health-related-quality-of-life
#7
Kathleen Morrisroe, Vijaya Sudararajan, Wendy Stevens, Joanne Sahhar, Jane Zochling, Janet Roddy, Susanna Proudman, Mandana Nikpour
Objective: To evaluate work productivity and its economic burden in SSc patients. Methods: Consecutive SSc patients enrolled in the Australian Scleroderma Cohort Study were mailed questionnaires assessing employment (Workers' Productivity and Activity Impairment Questionnaire and a custom-made questionnaire) and health-related quality of life (HRQoL) (36-item Short Form Health Survey and Patient-Reported Outcomes Measurement Information System 29). Linear regression methods were used to determine factors associated with work productivity...
October 9, 2017: Rheumatology
https://www.readbyqxmd.com/read/29155097/14-3-3z-sequesters-cytosolic-t-bet-up-regulating-il-13-in-tc2-and-scleroderma-cd8-lymphocytes
#8
Sandra Cascio, Thomas A Medsger, William F Hawse, Simon C Watkins, Christine Milcarek, Larry W Moreland, Robert A Lafyatis, Patrizia Fuschiotti
BACKGROUND: Interleukin(IL)-13-producing CD8+ T cells have been implicated in the pathogenesis of type-2 driven inflammatory human conditions. We have shown that CD8+IL-13+ cells play a critical role in cutaneous fibrosis, the most characteristic feature of systemic sclerosis (scleroderma; SSc). However, the molecular mechanisms underlying IL-13 and other type-2 cytokine production by CD8+ T cells remain unclear. OBJECTIVE: Establish the molecular basis of IL-13 over-production by SSc CD8+ T cells, focusing on T-bet modulation of GATA-3 activity, which we showed to underlie IL-13 over-production in SSc CD8+IL-13+ cells...
November 15, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29151520/dropped-head-syndrome-and-the-presence-of-rimmed-vacuoles-in-a-muscle-biopsy-in-scleroderma-polymyositis-overlap-syndrome-associated-with-anti-ku-antibody
#9
Yoshida Takeshi, Yoshida Mai, Mitsuyo Kinjo, Jonosono Manabu, Higuchi Itsuro
A 66-year-old woman with a history of interstitial lung disease presented with a 3-month history of dropped head syndrome (DHS), followed by camptocormia and extremity weakness. A clinical examination revealed Raynaud phenomenon, arthralgia, distal skin sclerosis, and microbleeds in the nailfold capillaries. An anti-Ku antibody test was positive. A muscle biopsy revealed inflammatory myopathy with rimmed vacuoles (RVs). The diagnosis of scleroderma-polymyositis (SSc-PM) overlap syndrome was made. RVs on a muscle biopsy in a patient with inflammatory myositis involving axial muscles may be seen either in inclusion body myositis or SSc-PM overlap syndrome...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29151332/the-relationship-between-the-degree-of-skin-fibrosis-by-sonoelastography-and-the-degree-of-pulmonary-involvement-in-scleroderma
#10
Songül Çildağ, Mehmet Burak Çildağ
Background/aim: This study aimed to assess the relationship between skin fibrosis as determined by sonoelastography and the degree of pulmonary involvement as determined by high-resolution computed tomography (HRCT) in patients with diffuse cutaneous systemic sclerosis (dcSSc).Materials and methods: This prospective study included 40 patients with dcSSc. All patients with HRCT scans underwent conventional ultrasonography and sonoelastography to determine skin thickness and degree of fibrosis. The degree of fibrosis was classified according to color-scale sonoelastography...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29151128/physical-activity-in-patients-with-systemic-sclerosis
#11
S I E Liem, J M T A Meessen, R Wolterbeek, N Ajmone Marsan, M K Ninaber, T P M Vliet Vlieland, J K de Vries-Bouwstra
OBJECTIVES: To compare self-reported levels of physical activity (PA) of systemic sclerosis (SSc) patients with the general population. To evaluate in SSc patients factors associated with PA levels and needs and preferences regarding PA. METHODS: Fifty nine SSc patients completed the Short QUestionnaire to ASsess Health-Enhancing PA. The proportion of patients meeting the Dutch Recommendation for PA (= moderate PA for 30 min on ≥ 5 days/week) and total minutes of PA per week were calculated and compared with similar data from the Dutch population...
November 18, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29145709/defining-a-unified-vascular-phenotype-in-systemic-sclerosis
#12
REVIEW
Yannick Allanore, Oliver Distler, Marco Matucci-Cerinic, Christopher P Denton
Microcirculation impairment and related vasculopathy is a hallmark of systemic sclerosis. Digital ulcers are second only to Raynaud's phenomenon as a vascular complication occurring in scleroderma patients. Digital ulcers are painful and generate disability. Furthermore, patients may develop recurrent digital ulcers and one may ask whether the outcomes of such patients might be different to those of unaffected ones. Recently several registries have provided relevant information about digital ulcers. Male sex and severe skin disease seem to be the main associated factors observed in several registries...
November 16, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29142074/stat3-controls-col1a2-enhancer-activation-cooperatively-with-junb-regulates-type-i-collagen-synthesis-post-transcriptionally-and-is-essential-for-lung-myofibroblast-differentiation
#13
Ioannis Papaioannou, Shiwen Xu, Christopher P Denton, David J Abraham, Markella Ponticos
Fibroblast differentiation is key cellular process that underlies the process of fibrosis, a deadly complication of fibrotic diseases like Scleroderma (SSc). This transition coincides with the overproduction of Collagen type I (COL1) and other extracellular matrix proteins. High level expression of the collagen type 1α2 subunit (COL1A2), requires the engagement of a far upstream enhancer, whose activation is strongly dependent on the AP1 factor JunB. We now report that STAT3 also binds the COL1A2 enhancer and is essential for RNA polymerase recruitment, without affecting JunB binding...
November 15, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/29141862/the-activation-of-human-dermal-microvascular-cells-by-poly-i-c-lipopolysaccharide-imiquimod-and-odn2395-is-mediated-by-the-fli1-foxo3a-pathway
#14
Lukasz Stawski, Grace Marden, Maria Trojanowska
Endothelial cell (EC) dysfunction has been associated with inflammatory and autoimmune diseases; however, the factors contributing to this dysfunction have not been fully explored. Because activation of TLRs has been implicated in autoimmune diseases, the goal of this study was to determine the effects of TLR ligands on EC function. Human dermal microvascular ECs (HDMECs) treated with TLR3 [Poly(I:C)], TLR4 (LPS), and TLR7 (imiquimod) agonists showed decreased proliferation and a reduced total number of branching tubules in three-dimensional human dermal organoid ex vivo culture...
November 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29140482/estimating-autoantibody-signatures-to-detect-autoimmune-disease-patient-subsets
#15
Zhenke Wu, Livia Casciola-Rosen, Ami A Shah, Antony Rosen, Scott L Zeger
Autoimmune diseases are characterized by highly specific immune responses against molecules in self-tissues. Different autoimmune diseases are characterized by distinct immune responses, making autoantibodies useful for diagnosis and prediction. In many diseases, the targets of autoantibodies are incompletely defined. Although the technologies for autoantibody discovery have advanced dramatically over the past decade, each of these techniques generates hundreds of possibilities, which are onerous and expensive to validate...
November 13, 2017: Biostatistics
https://www.readbyqxmd.com/read/29136260/localized-scleroderma-systemic-sclerosis-and-cardiovascular-risk-a-danish-nationwide-cohort-study
#16
Jeanette Halskou Hesselvig, Kristian Kofoed, Jashin J Wu, Lene Dreyer, Gunnar Gislason, Ole Ahlehoff
Recent findings indicate that patients with systemic sclerosis have an increased risk of cardiovascular disease. To determine whether patients with systemic sclerosis or localized scleroderma are at increased risk of cardiovascular disease, a cohort study of the entire Danish population aged ≥ 18 and ≤ 100 years was conducted, followed from 1997 to 2011 by individual-level linkage of nationwide registries. Multivariable adjusted Cox regression models were used to estimate the hazard ratios (HRs) for a composite cardiovascular disease endpoint...
November 14, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29135399/-investigation-of-parameters-related-to-prognosis-in-diffuse-parenchymal-lung-diseases-prognosis-in-interstitial-lung-diseases
#17
Coşkun Canıvar, Züleyha Bingöl, Zeki Kılıçaslan, Tülin Çağatay, N Gülfer Okumuş
Introduction: Parameters related to prognosis in diffuse parenchymal lung disease (DPLD) have a decisive influence on treatment and follow-up processes. We aimed to define baseline characteristics and factors that effect the mortality of the group of patients with DPLD and to determine distinctions between subgroups. Materials and Methods: Demographic characteristics, complaints, comorbidity, treatment, pulmonary function tests, echocardiographic findings, six minute walking test (6MWT), arterial blood gases analysis, radiological findings and survival time were collected from outpatient clinics database...
September 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/29121455/bench-to-bedside-and-back-again-finding-the-goldilocks-zone-within-the-scleroderma-universe
#18
EDITORIAL
Janet E Pope, Jason J Lee, Christopher P Denton
Systemic sclerosis (SSc) is a complex inflammatory connective tissue disease characterized by skin thickening, organ fibrosis and vasculopathy [1, 2]. The underlying disease mechanisms and pathophysiology are not fully understood. However, it appears to be, at least in part, driven by autoimmunity and inflammation associated with microvascular dysfunction ultimately resulting in excess extracellular matrix deposition in target organs [2-4]. Clinical heterogeneity coupled with our lack of understanding of the disease pathogenesis means that current treatment strategies are mostly organ based therapies using anti-rheumatic drugs that were originally approved for other indications [5]...
November 9, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29120857/the-safety-and-efficacy-of-light-emitting-diodes-based-ultraviolet-a1-phototherapy-in-bleomycin-induced-scleroderma-in-mice
#19
Diana Karpec, Romualdas Rudys, Laima Leonaviciene, Zygmunt Mackiewicz, Ruta Bradunaite, Gailute Kirdaite, Algirdas Venalis
PURPOSE: To define the efficacy and safety of narrowband ultraviolet A1 (UVA1) for the treatment of dermal fibrosis in bleomycin-induced mouse model of scleroderma. MATERIALS AND METHODS: 42 DBA/2 strain mice were included in the study: healthy mice and mice with established scleroderma, treated with high or medium dose of UVA1. Non-treated groups served as control. The equipment emitting 365±5nm UVA1 radiation was used in the study. The average cumulative doses were 1200J/cm(2) for high and 600J/cm(2) for medium dose course...
November 6, 2017: Advances in Medical Sciences
https://www.readbyqxmd.com/read/29120812/imaging-findings-in-systemic-childhood-diseases-presenting-with-dermatologic-manifestations
#20
REVIEW
Adam Z Fink, Julia K Gittler, Radhika N Nakrani, Jonathan Alis, Einat Blumfield, Terry L Levin
PURPOSE: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. METHODS: We review the imaging findings in childhood diseases associated with dermatologic manifestations. FINDINGS: Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura)...
October 31, 2017: Clinical Imaging
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