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Scleroderma

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https://www.readbyqxmd.com/read/29340325/end-stage-kidney-disease-from-scleroderma-in-the-united-states-1996-to-2012
#1
Donal J Sexton, Scott Reule, Robert N Foley
Introduction: Although the management of scleroderma continues to evolve, it is unknown whether the burden of end-stage kidney disease (ESKD) treated with maintenance renal replacement therapy from SD has changed. Methods: We examined United States Renal Data System data (n = 1,677,303) for the years 1996 to 2012 to quantify the incidence and outcomes of ESKD from scleroderma treated with renal replacement therapy (n = 2398). Outcomes assessed through demography-matched scleroderma-positive/scleroderma-negative comparisons included recovery of kidney function, mortality, listing for transplant, renal transplantations, and graft failure...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29339444/light-hvem-signaling-in-keratinocytes-controls-development-of-dermatitis
#2
Rana Herro, Jr-Wen Shui, Sonja Zahner, Daniel Sidler, Yuko Kawakami, Toshiaki Kawakami, Koji Tamada, Mitchell Kronenberg, Michael Croft
Dermatitis is often associated with an allergic reaction characterized by excessive type 2 responses leading to epidermal acanthosis, hyperkeratosis, and dermal inflammation. Although factors like IL-4, IL-13, and thymic stromal lymphopoietin (TSLP) are thought to be instrumental for the development of this type of skin disorder, other cytokines may be critical. Here, we show that the tumor necrosis factor (TNF) superfamily protein LIGHT (homologous to lymphotoxin, exhibits inducible expression, and competes with HSV glycoprotein D for binding to HVEM, a receptor expressed on T lymphocytes) is required for experimental atopic dermatitis, and LIGHT directly controls keratinocyte hyperplasia, and production of periostin, a matricellular protein that contributes to the clinical features of atopic dermatitis as well as other skin diseases such as scleroderma...
January 16, 2018: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29336866/regulatory-mechanisms-of-collagen-expression-by-interleukin-22-signaling-in-scleroderma-fibroblasts
#3
Soichiro Sawamura, Masatoshi Jinnin, Kuniko Inoue, Keitaro Yamane, Noritoshi Honda, Ikko Kajihara, Takamitsu Makino, Shinichi Masuguchi, Satoshi Fukushima, Hironobu Ihn
BACKGROUND: Various cytokines have been indicated to be involved in the pathogenesis of systemic sclerosis (SSc). IL-22 is one of the member of IL-10 cytokine family, and several studies have implicated IL-22 signaling in the pathogenesis of autoimmune diseases. OBJECTIVES: To clarify the role of IL-22 in the regulatory mechanism of ECM expression and to determine the contribution of IL-22 to the phenotype of SSc. METHODS: The effect of IL-22 on ECM expression in normal fibroblasts was determined by using PCR array, real-time PCR and immunoblotting...
December 29, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/29333938/structure-based-engineering-to-restore-high-affinity-binding-of-an-isoform-selective-anti-tgf%C3%AE-1-antibody
#4
Dana M Lord, Julie J Bird, Denise M Honey, Annie Best, Anna Park, Ronnie R Wei, Huawei Qiu
Metelimumab (CAT192) is a human IgG4 monoclonal antibody developed as a TGFβ1-specific antagonist. It was tested in clinical trials for the treatment of scleroderma but later terminated due to lack of efficacy. Subsequent characterization of CAT192 indicated that its TGFβ1 binding affinity was reduced by ∼50-fold upon conversion from the parental single-chain variable fragment (scFv) to IgG4. We hypothesized this result was due to decreased conformational flexibility of the IgG that could be altered via engineering...
January 15, 2018: MAbs
https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-rheumatology-research-alliance-consensus-treatment-plans-towards-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#5
REVIEW
Sarah Ringold, Peter A Nigrovic, Brian M Feldman, George A Tomlinson, Emily von Scheven, Carol A Wallace, Adam M Huber, Laura E Schanberg, Suzanne C Li, Pamela F Weiss, Robert C Fuhlbrigge, Esi M Morgan, Yukiko Kimura
The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes...
January 15, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29324934/stem-cells-therapy-the-future-in-the-management-of-systemic-sclerosis-a-case-report
#6
Jung In Song, Silvanie Volz, Maria Eirini Liodaki, Peter Mailänder, Konstantinos Kalousis
OBJECTIVE: Systemic sclerosis (SSc) is a connective tissue disorder of unknown etiology, with heterogeneous clinical manifestations and chronic and often progressive course. The diffuse cutaneous form of SSc (dcSSc) is characterized by thickening of the skin (scleroderma) and distinctive involvement of multiple internal organs. Patients with limited cutaneous SSc (lcSSc) generally have long-standing Raynaud's phenomenon before other manifestations of SSc appear. Over the last decade the Interest of adipose-derived cell therapy in regenerative medicine has increased continuously...
September 2017: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29324586/ten-year-experience-of-transjugular-intrahepatic-portosystemic-shunt-for-noncirrhotic-portal-hypertension
#7
David Regnault, Louis d'Alteroche, Charlotte Nicolas, Fanny Dujardin, Jean Ayoub, Jean Marc Perarnau
BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) is considered to be well suited for the treatment of noncirrhotic portal hypertension (NCPHT) because of a usually severe portal hypertension (PHT) and a mild liver failure, but very less data are available. PATIENTS AND METHODS: Records of patients referred for TIPS between 2004 and 2015 for NCPHT were reviewed. No patient should have clinical or biological or histological features of cirrhosis. RESULTS: Twenty-five patients with a wide variety of histological lesions (sinusoidal dilatations, granulomatosis, regenerative nodular hyperplasia, obliterative portal venopathy, or subnormal liver) and a wide variety of associated diseases (thrombophilia, sarcoidosis, common variable immunodeficiency, scleroderma, Castleman's disease, early primitive biliary cirrhosis, congenital liver fibrosis, chemotherapy, purinethol intake, and congenital varices) were included...
January 10, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29322341/interstitial-lung-disease-in-systemic-sclerosis-data-from-the-spanish-scleroderma-study-group
#8
D Sánchez-Cano, N Ortego-Centeno, J L Callejas, V Fonollosa Plá, R Ríos-Fernández, C Tolosa-Vilella, G Espinosa-Garriga, D Colunga-Argüelles, M V Egurbide-Arberas, M Rubio-Rivas, M Freire, J J Ríos-Blanco, L Trapiella-Martínez, M Rodríguez-Carballeira, A Marín-Ballvé, X Pla-Salas, C P Simeón-Aznar
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group...
January 10, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29321259/plasmacytoid-dendritic-cells-promote-systemic-sclerosis-with-a-key-role-for-tlr8
#9
Marie Dominique Ah Kioon, Claudio Tripodo, David Fernandez, Kyriakos A Kirou, Robert F Spiera, Mary K Crow, Jessica K Gordon, Franck J Barrat
Systemic sclerosis (SSc) is a multisystem life-threatening fibrosing disorder that lacks effective treatment. The link between the inflammation observed in organs such as the skin and profibrotic mechanisms is not well understood. The plasmacytoid dendritic cell (pDC) is a key cell type mediating Toll-like receptor (TLR)-induced inflammation in autoimmune disease patients, including lupus and skin diseases with interface dermatitis. However, the role of pDCs in fibrosis is less clear. We show that pDCs infiltrate the skin of SSc patients and are chronically activated, leading to secretion of interferon-α (IFN-α) and CXCL4, which are both hallmarks of the disease...
January 10, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29316366/impact-of-radiation-therapy-on-scleroderma-and-cancer-outcomes-in-scleroderma-patients-with-breast-cancer
#10
Dhaval J Shah, Ram Hirpara, Corrie L Poelman, Adrianne Woods, Laura K Hummers, Fredrick M Wigley, Jean L Wright, Arti Parekh, Virginia D Steen, Robyn T Domsic, Ami A Shah
OBJECTIVES: We examined SSc patients with breast cancer to 1) identify the prevalence of radiation complications and 2) examine SSc outcomes in SSc patients who received radiation therapy (RT) as part of their cancer treatment. METHODS: Patients with SSc and breast cancer were identified from the Johns Hopkins and University of Pittsburgh Scleroderma Center databases. We examined whether erythema, blistering, ulceration, or thickening of the skin developed in the RT port...
January 5, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29313516/scleroderma-in-hospital-settings-in-lom%C3%A3-50%C3%A2-cases
#11
A S Akakpo, J N Teclessou, A Mouhari-Touré, B Saka, H Matakloe, K Kakpovi, K Kombate, P Pitché
The aim of this study was to document the epidemiological and clinical profile, treatment used, and outcome of patients with scleroderma in hospital settings in Lomé. This descriptive study examined the records of all patients seen as outpatients or admitted for scleroderma in hospital dermatology and rheumatology departments in Lomé during the 20-year period of 1993-2012. During the study period, 50 (0.04%) of the 121,021 patients seen in these departments had scleroderma. There were 29 cases of localized scleroderma and 21 systemic cases, predominantly women (sex-ratio=0...
November 1, 2017: Médecine et Santé Tropicales
https://www.readbyqxmd.com/read/29313055/abatacept-improves-skin-score-and-reduces-lesions-in-patients-with-localized-scleroderma-a-case-series
#12
Simon Wehner Fage, Kristian Bakke Arvesen, Anne Braae Olesen
No abstract text is available yet for this article.
January 9, 2018: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29310332/quantifying-the-direct-public-health-care-cost-of-systemic-sclerosis-a-comprehensive-data-linkage-study
#13
Kathleen Morrisroe, Wendy Stevens, Joanne Sahhar, Gene-Siew Ngian, Candice Rabusa, Nava Ferdowsi, Catherine Hill, Susanna Proudman, Mandana Nikpour
To quantify the direct healthcare cost of systemic sclerosis (SSc) and identify its determinants. Healthcare use was captured through data linkage, wherein clinical and medication data for SSc patients from the state of Victoria enrolled in the Australian Scleroderma Cohort Study were linked with the Victorian hospital admissions and emergency presentations data sets, and the Medicare Benefits Schedule which contains all government subsidized ambulatory care services, for the period 2011-2015. Medication cost was determined from the Pharmaceutical Benefits Scheme...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29308290/cutaneous-calcinosis-in-a-patient-with-limited-scleroderma-crest-syndrome
#14
Nurşen Düzgün
No abstract text is available yet for this article.
December 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/29306872/patterns-and-predictors-of-skin-score-change-in-early-diffuse-systemic-sclerosis-from-the-european-scleroderma-observational-study
#15
Ariane L Herrick, Sebastien Peytrignet, Mark Lunt, Xiaoyan Pan, Roger Hesselstrand, Luc Mouthon, Alan J Silman, Graham Dinsdale, Edith Brown, László Czirják, Jörg H W Distler, Oliver Distler, Kim Fligelstone, William J Gregory, Rachel Ochiel, Madelon C Vonk, Codrina Ancuţa, Voon H Ong, Dominique Farge, Marie Hudson, Marco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Paresh Jobanputra, Alison C Jordan, Wendy Stevens, Pia Moinzadeh, Frances C Hall, Christian Agard, Marina E Anderson, Elisabeth Diot, Rajan Madhok, Mohammed Akil, Maya H Buch, Lorinda Chung, Nemanja S 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, Susanna M Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J Veale, Claire Grange, Georges-Selim Trad, Christopher P Denton
OBJECTIVES: Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). METHODS: The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months)...
January 6, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29300294/calcinosis-cutis-woc-nurse-management
#16
Debra Netsch
BACKGROUND: Calcinosis cutis is characterized by deposition of calcium in the dermis and the subcutaneous tissue. This condition may be initially identified by the WOC nurse, and its management requires a team approach. Calcinosis cutis is a debilitating and painful condition; it is difficult to manage, and widely agreed-upon standards for treatment have not been established. CASES: Two patients who presented with calcium deposits in the dermis and the subcutaneous tissue are discussed...
January 2018: Journal of Wound, Ostomy, and Continence Nursing
https://www.readbyqxmd.com/read/29298697/cone-beam-computed-tomography-for-the-assessment-of-linear-scleroderma-of-the-face
#17
C Di Giovanni, S Puggina, A Meneghel, F Vittadello, G Martini, F Zulian
BACKGROUND: To date, standardized methods for assessing the disease progression of linear scleroderma of the face (LSF) are lacking. OBJECTIVES: We investigated whether Cone Beam Computed Tomography (CBCT) may represent a reliable tool for assessing linear scleroderma of the face (LSF). METHODS: Ten patients with LSF and five age-matched controls underwent CBCT assessment. The transverse sections at three anatomic levels of the maxillofacial bones were analyzed...
January 3, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29298160/myeloablative-autologous-stem-cell-transplantation-for-severe-scleroderma
#18
Keith M Sullivan, Ellen A Goldmuntz, Lynette Keyes-Elstein, Peter A McSweeney, Ashley Pinckney, Beverly Welch, Maureen D Mayes, Richard A Nash, Leslie J Crofford, Barry Eggleston, Sharon Castina, Linda M Griffith, Julia S Goldstein, Dennis Wallace, Oana Craciunescu, Dinesh Khanna, Rodney J Folz, Jonathan Goldin, E William St Clair, James R Seibold, Kristine Phillips, Shin Mineishi, Robert W Simms, Karen Ballen, Mark H Wener, George E Georges, Shelly Heimfeld, Chitra Hosing, Stephen Forman, Suzanne Kafaja, Richard M Silver, Leroy Griffing, Jan Storek, Sharon LeClercq, Richard Brasington, Mary E Csuka, Christopher Bredeson, Carolyn Keever-Taylor, Robyn T Domsic, M Bashar Kahaleh, Thomas Medsger, Daniel E Furst
BACKGROUND: Despite current therapies, diffuse cutaneous systemic sclerosis (scleroderma) often has a devastating outcome. We compared myeloablative CD34+ selected autologous hematopoietic stem-cell transplantation with immunosuppression by means of 12 monthly infusions of cyclophosphamide in patients with scleroderma. METHODS: We randomly assigned adults (18 to 69 years of age) with severe scleroderma to undergo myeloablative autologous stem-cell transplantation (36 participants) or to receive cyclophosphamide (39 participants)...
January 4, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29297205/cyclophosphamide-for-connective-tissue-disease-associated-interstitial-lung-disease
#19
REVIEW
Hayley Barnes, Anne E Holland, Glen P Westall, Nicole Sl Goh, Ian N Glaspole
BACKGROUND: Approximately one-third of individuals with interstitial lung disease (ILD) have associated connective tissue disease (CTD). The connective tissue disorders most commonly associated with ILD include scleroderma/systemic sclerosis (SSc), rheumatoid arthritis, polymyositis/dermatomyositis, and Sjögren's syndrome. Although many people with CTD-ILD do not develop progressive lung disease, a significant proportion do progress, leading to reduced physical function, decreased quality of life, and death...
January 3, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29280826/an-unusual-cause-of-dysphagia
#20
Soumya Chatterjee, Brian J Hedman, Donald F Kirby
Scleredema adultorum of Buschke is a rare scleroderma mimic characterized by woody induration of the skin involving the back of the neck, interscapular region, upper and mid back, and sometimes the face, upper chest, and upper arms. Dysphagia is rare and not well characterized. We describe a case of scleredema that developed significant dysphagia affecting both the oropharyngeal phase and the esophageal phase of deglutition. Diagnostic evaluation revealed mild-moderate oropharyngeal and severe esophageal dysmotility...
December 27, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
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