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https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#1
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/28386330/geniposide-inhibited-endothelial-mesenchymal-transition-via-the-mtor-signaling-pathway-in-a-bleomycin-induced-scleroderma-mouse-model
#2
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/28376288/mycophenolate-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#3
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/28370513/high-dose-intravenous-immunoglobulin-therapy-for-scleromyxedema-a-prospective-open-label-clinical-trial-using-an-objective-score-of-clinical-evaluation-system
#4
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/28349063/thermography-improves-clinical-assessment-in-patients-with-systemic-sclerosis-treated-with-ozone-therapy
#5
Danuta Nowicka
Objective. Treatment of scleroderma is challenging and limited. The aim of our study was to evaluate the usefulness of thermography in assessment of the clinical condition (joints movability and skin thickness) in clinically advanced patients with systemic sclerosis before and after ozone therapy. Method. The study included 42 patients aged 32 to 73 years with advanced systemic sclerosis hospitalized in the university clinic between 2003 and 2006. Thermography and clinical examinations were conducted at baseline and after two series of bath in water with ozone...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28337853/long-term-efficacy-and-tolerability-of-mycophenolate-mofetil-therapy-in-diffuse-scleroderma-skin-disease
#6
Daniel Boulos, Gene-Siew Ngian, Anton Rajadurai, Kathleen Elford, Wendy Stevens, Susanna Proudman, Claire Owen, Janet Roddy, Mandana Nikpour, Peter Youssef, Catherine Hill, Joanne Sahhar
OBJECTIVES: To assess the long-term efficacy and tolerability of mycophenolate mofetil (MMF) in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: Patients enrolled in the Australian Scleroderma Cohort study with dcSSc and baseline modified Rodnan skin score (mRSS) ≥ 12 who were treated for a minimum of 12 months with MMF for the primary indication of skin disease were included and their prospectively collected data retrieved. Change in mRSS, the proportion with a clinically significant improvement (reduction in mRSS ≥ 5 from baseline) and adverse effects due to therapy were determined...
March 24, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28303481/morphea-and-eosinophilic-fasciitis-an-update
#7
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/28303284/-generalized-circumscribed-scleroderma-with-blisters
#8
G Wagner, V Meyer, M M Sachse
The patient suffered from a 20-year course of generalized circumscribed scleroderma and presented with blisters in circumscribed areas of the affected skin. The development of subepidermal blisters has been described in all clinical forms of circumscribed scleroderma. Aetiology and pathogenesis of blister formation have not yet been clarified. An obstruction of the lymphatic vessels due to the present sclerosis is favoured. Treatment of bullous circumscribed scleroderma is considered to be difficult. Oral steroids, methotrexate, hydroxychloroquine and PUVA methods have been used with varying success...
March 16, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28271552/localized-scleroderma-clinical-and-epidemiological-features-with-emphasis-on-adulthood-versus-childhood-onset-disease-differences
#9
REVIEW
A Lis-Święty, A Skrzypek-Salamon, I Ranosz-Janicka, L Brzezińska-Wcisło
BACKGROUND: Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given. OBJECTIVE: To identify these features which differentiate aLoS from JLS...
March 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28263294/increased-expression-of-latent-tgf-%C3%AE-binding-protein-4-affects-the-fibrotic-process-in-scleroderma-by-tgf-%C3%AE-smad-signaling
#10
Jiaying Lu, Qingmei Liu, Lei Wang, Wenzhen Tu, Haiyan Chu, Weifeng Ding, Shuai Jiang, Yanyun Ma, Xiangguang Shi, Weilin Pu, Xiaodong Zhou, Li Jin, Jiucun Wang, Wenyu Wu
Scleroderma is a fibrosis-related disorder characterized by cutaneous and internal organ fibrosis, and excessive collagen deposition in extracellular matrix (ECM) is a major cause of fibrosis. Transforming growth factor-β (TGF-β)/SMAD signaling has a central role in the pathogenesis of fibrosis by inducing abnormal collagen accumulation in ECM, and latent TGF-β-binding protein 4 (LTBP-4) affects the secretion of latent TGF-β to ECM. A previous study indicated that bleomycin (BLM) treatment increased LTBP-4 expression in lung fibroblasts of Thy-1 knockout mice with lung fibrosis, and LTBP-4 further promoted TGF-β bioavailability as well as SMAD3 phosphorylation...
March 6, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28261202/stretching-reduces-skin-thickness-and-improves-subcutaneous-tissue-mobility-in-a-murine-model-of-systemic-sclerosis
#11
Ying Xiong, Lisbeth Berrueta, Katia Urso, Sara Olenich, Igla Muskaj, Gary J Badger, Antonios Aliprantis, Robert Lafyatis, Helene M Langevin
OBJECTIVE: Although physical therapy can help preserve mobility in patients with systemic sclerosis (SSc), stretching has not been used systematically as a treatment to prevent or reverse the disease process. We previously showed in rodent models that stretching promotes the resolution of connective tissue inflammation and reduces new collagen formation after injury. Here, we tested the hypothesis that stretching would impact scleroderma development using a mouse sclerodermatous graft-versus-host disease (sclGvHD) model...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28254742/pirfenidone-ameliorates-murine-chronic-gvhd-through-inhibition-of-macrophage-infiltration-and-tgf-%C3%AE-production
#12
Jing Du, Katelyn Paz, Ryan Flynn, Ante Vulic, Tara M Robinson, Katie E Lineburg, Kylie A Alexander, Jingjing Meng, Sabita Roy, Angela Panoskaltsis-Mortari, Michael Loschi, Geoffrey R Hill, Jonathan S Serody, Ivan Maillard, David Miklos, John Koreth, Corey S Cutler, Joseph H Antin, Jerome Ritz, Kelli P MacDonald, Timothy W Schacker, Leo Luznik, Bruce R Blazar
Allogeneic hematopoietic stem cell transplantation is hampered by chronic graft-versus-host disease (cGVHD), resulting in multiorgan fibrosis and diminished function. Fibrosis in lung and skin leads to progressive bronchiolitis obliterans (BO) and scleroderma, respectively, for which new treatments are needed. We evaluated pirfenidone, a Food and Drug Administration (FDA)-approved drug for idiopathic pulmonary fibrosis, for its therapeutic effect in cGVHD mouse models with distinct pathophysiology. In a full major histocompatibility complex (MHC)-mismatched, multiorgan system model with BO, donor T-cell responses that support pathogenic antibody production are required for cGVHD development...
May 4, 2017: Blood
https://www.readbyqxmd.com/read/28249987/trpv4-ion-channel-is-a-novel-regulator-of-dermal-myofibroblast-differentiation
#13
Shweta Sharma, Rishov Goswami, Michael Merth, Jonathan Cohen, Kai Y Lei, David X Zhang, Shaik O Rahaman
Scleroderma is a multisystem fibroproliferative disease with no effective medical treatment. Myofibroblasts are critical to the fibrogenic tissue repair process in the skin and many internal organs. Emerging data support a role for both matrix stiffness, and transforming growth factor β1 (TGFβ1), in myofibroblast differentiation. Transient receptor potential vanilloid 4 (TRPV4) is a mechanosensitive ion channel activated by both mechanical and biochemical stimuli. The objective of this study was to determine the role of TRPV4 in TGFβ1- and matrix stiffness-induced differentiation of dermal fibroblasts...
March 1, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/28210455/morphea-en-coup-de-sabre-an-unusual-oral-presentation
#14
Sven Niklander, Constanza Marín, René Martínez, Alfredo Esguep
Morphea, or localized scleroderma, is an inflammatory disease that leads to sclerosis of the skin and underlying tissues due to excessive collagen deposition. Oral involvement is unusual and it may produce white linear fibrotic areas with a scar-like appearance, atrophy of tongue papillae, gingival recession and alveolar bone resorption. We report a case of a 13-year-old girl who consulted for progressive recession on the attached gingiva of her upper left incisors. She also presented a hypopigmented line on the left side skin of her upper lip, which continued through the vermilion and the lip mucosa, including the gingiva of the affected teeth...
February 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28188239/treatment-outcome-in-early-diffuse-cutaneous-systemic-sclerosis-the-european-scleroderma-observational-study-esos
#15
Ariane L Herrick, Xiaoyan Pan, Sébastien Peytrignet, Mark Lunt, Roger Hesselstrand, Luc Mouthon, Alan Silman, Edith Brown, László Czirják, Jörg H W Distler, Oliver Distler, Kim Fligelstone, William J Gregory, Rachel Ochiel, Madelon Vonk, Codrina Ancuţa, Voon H Ong, Dominique Farge, Marie Hudson, Marco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Alison C Jordan, Paresh Jobanputra, Wendy Stevens, Pia Moinzadeh, Frances C Hall, Christian Agard, Marina E Anderson, Elisabeth Diot, Rajan Madhok, Mohammed Akil, Maya H Buch, Lorinda Chung, Nemanja Damjanov, Harsha Gunawardena, Peter Lanyon, Yasmeen Ahmad, Kuntal Chakravarty, Søren Jacobsen, Alexander J MacGregor, Neil McHugh, Ulf Müller-Ladner, Gabriela Riemekasten, Michael Becker, Janet Roddy, Patricia E Carreira, Anne Laure Fauchais, Eric Hachulla, Jennifer Hamilton, Murat İnanç, John S McLaren, Jacob M van Laar, Sanjay Pathare, Susannah Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J Veale, Claire Grange, Georges-Selim Trad, Christopher P Denton
OBJECTIVES: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. We aimed to use an observational approach to compare effectiveness of currently used treatment approaches. METHODS: This was a prospective, observational cohort study of early dcSSc (within three years of onset of skin thickening). Clinicians selected one of four protocols for each patient: methotrexate, mycophenolate mofetil (MMF), cyclophosphamide or 'no immunosuppressant'...
February 10, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28138238/quantitative-nanohistological-investigation-of-scleroderma-an-atomic-force-microscopy-based-approach-to-disease-characterization
#16
Adam P Strange, Sebastian Aguayo, Tarek Ahmed, Nicola Mordan, Richard Stratton, Stephen R Porter, Susan Parekh, Laurent Bozec
Scleroderma (or systemic sclerosis, SSc) is a disease caused by excess crosslinking of collagen. The skin stiffens and becomes painful, while internally, organ function can be compromised by the less elastic collagen. Diagnosis of SSc is often only possible in advanced cases by which treatment time is limited. A more detailed analysis of SSc may provide better future treatment options and information of disease progression. Recently, the histological stain picrosirius red showing collagen register has been combined with atomic force microscopy (AFM) to study SSc...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28116278/calcinosis-cutis-circumscripta-of-knee-a-rare-presentation
#17
Vikram V Kadu, K A Saindane, Ninad Godghate, Neha Godghate
INTRODUCTION: Calcinosis circumscripta is a form of ectopic mineralization characterized by single or multiple cutaneous nodules containing calcium salts. Calcinosis cutis circumscripta is a very rare form of idiopathic calcinosis cutis arising in the second half oflife. It typically involves the extremities and is associated with prior trauma and scleroderma. We dealt with a very rare form of calcinosis cutis circumscripta in a healthy patient, for whom surgical excision revealed to be an effective and successful treatment...
July 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28107865/adipose-tissue-derived-stem-cells-ameliorates-dermal-fibrosis-in-a-mouse-model-of-scleroderma
#18
Wei Chen, Zhi-Kuan Xia, Man-Hui Zhang, Gui-Chun Ding, Xiao-Yan Zhang, Zheng-Xu Wang, Rong-Ya Yang
OBJECTIVE: To investigate the therapeutic potential of adipose-derived stem cells (ADSCs) for limited cutaneous scleroderma (LS) in mouse models. METHODS: ADSCs were isolated from pathogen-free female C57BL/6 mice and LS was induced in wild type (WT) C57BL/6 mice via daily injection of bleomycin (0.1 mL × 300 μg/mL) for 4 weeks; then the ADSCs were subcutaneously injected into the dorsal area in the model treatment group, and 100 μL of phosphate-buffered saline (PBS) solution was injected into the same site in the model control group...
January 2017: Asian Pacific Journal of Tropical Medicine
https://www.readbyqxmd.com/read/28065485/systemic-sclerosis-sine-scleroderma-in-mexican-patients-case-reports
#19
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/27939457/skin-fibrosis-models-and-mechanisms
#20
N N Do, S A Eming
Matrix synthesis, deposition and remodeling are complex biological processes that are critical in development, maintenance of tissue homeostasis and repair of injured tissues. Disturbances in the regulation of these processes can result in severe pathological conditions which are associated with tissue fibrosis as e.g. in Scleroderma, cutaneous Graft-versus-Host-Disease, excessive scarring after trauma or carcinogenesis. Therefore, finding efficient treatments to limit skin fibrosis is of major clinical importance...
October 2016: Current Research in Translational Medicine
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