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esclerose sistemica

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28 papers 0 to 25 followers
https://www.readbyqxmd.com/read/28188239/treatment-outcome-in-early-diffuse-cutaneous-systemic-sclerosis-the-european-scleroderma-observational-study-esos
#1
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/28128745/endothelial-dysfunction-microvascular-damage-and-ischemic-peripheral-vasculopathy-in-systemic-sclerosis
#2
Ivone Fernandes Santos da Silva, Andreia Teixeira, José Oliveira, Rui Almeida, Carlos Vasconcelos
OBJECTIVE: To evaluate endothelial dysfunction and microvascular damage in secondary Raynaud Phenomenon (SRP) and Systemic sclerosis (SSc)-associated patients as possible predictors of ischemic fingertip digital ulcers (DU) in a 3-year clinical follow-up. METHODS: Flow-mediated dilatation (FMD), nailfold videocapillaroscopy (NVC), endothelin-1 (ET-1) and asymmetric dimethylarginine (ADMA) were analysed in a 3-year observational cohort study of 77 SRP patients with systemic sclerosis...
January 27, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/28063071/interstitial-lung-disease-in-systemic-sclerosis-current-and-future-treatment
#3
REVIEW
Roberto Giacomelli, Vasiliki Liakouli, Onorina Berardicurti, Piero Ruscitti, Paola Di Benedetto, Francesco Carubbi, Giuliana Guggino, Salvatore Di Bartolomeo, Francesco Ciccia, Giovanni Triolo, Paola Cipriani
Systemic sclerosis (SSc) has the highest fatality rate among connective tissue diseases and is characterized by vascular damage, inflammation and fibrosis of the skin and various internal organs. Interstitial lung disease (ILD) frequently complicates SSc and can be a debilitating disorder with a poor prognosis. ILD is the most frequent cause of death in SSc, and the management of SSc-ILD patients is a great challenge. Early detection of pulmonary involvement based on a recent decline of lung function tests and on the extent of lung involvement at high-resolution computed tomography is critical for the best management of these patients...
January 6, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28049126/comorbidities-in-interstitial-lung-diseases
#4
REVIEW
George A Margaritopoulos, Katerina M Antoniou, Athol U Wells
Fibrosing lung disorders include a large number of diseases with diverse behaviour. Patients can die because of the progression of their illness, remain stable or even improve after appropriate treatment has been instituted. Comorbidities, such as acute and chronic infection, gastro-oesophageal reflux, pulmonary hypertension, lung cancer, cardiovascular diseases, and obstructive sleep apnoea, can pre-exist or develop at any time during the course of the disease and, if unidentified and untreated, may impair quality of life, impact upon the respiratory status of the patients, and ultimately lead to disease progression and death...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/27940590/primary-myocardial-disease-in-scleroderma-a-comprehensive-review-of-the-literature-to-inform-the-uk-systemic-sclerosis-study-group-cardiac-working-group
#5
REVIEW
Lesley-Anne Bissell, Md Yuzaiful Md Yusof, Maya H Buch
Cardiac disease is prevalent in SSc and associated with a poor prognosis. Differentiating primary myocardial disease (SSc-cardiomyopathy) from ischaemic heart disease is difficult and the disease phenotype most at risk is unclear. A comprehensive literature review was performed to inform the UK Systemic Sclerosis Study Group for cardiac disease tasked with producing a best practice pathway for the management of cardiac disease in SSc. This review describes the prevalence of SSc-cardiomyopathy, its associated greater mortality and various manifestations (e...
December 10, 2016: Rheumatology
https://www.readbyqxmd.com/read/27941129/update-of-eular-recommendations-for-the-treatment-of-systemic-sclerosis
#6
(no author information available yet)
No abstract text is available yet for this article.
November 9, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27749244/uk-scleroderma-study-group-ukssg-guidelines-on-the-diagnosis-and-management-of-scleroderma-renal-crisis
#7
REVIEW
Bernadette M Lynch, Edward P Stern, Voon Ong, Mark Harber, Aine Burns, Christopher P Denton
The UK Scleroderma Study Group developed guidelines on the diagnosis and management of scleroderma renal crisis (SRC) based on best available evidence and clinical experience. SRC is characterised by the acute onset of severe hypertension and acute kidney injury. Current strategies to reduce the associated morbidity and mortality include identifying at risk patients to aid early diagnosis. ACE inhibitor therapy should be lifelong in all patients, regardless of whether they require renal replacement therapy...
September 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/26704522/predictors-of-lung-function-decline-in-scleroderma-related-interstitial-lung-disease-based-on-high-resolution-computed-tomography-implications-for-cohort-enrichment-in-systemic-sclerosis-associated-interstitial-lung-disease-trials
#8
RANDOMIZED CONTROLLED TRIAL
Dinesh Khanna, Vivek Nagaraja, Chi-Hong Tseng, Fereidoun Abtin, Robert Suh, Grace Kim, Athol Wells, Daniel E Furst, Philip J Clements, Michael D Roth, Donald P Tashkin, Jonathan Goldin
BACKGROUND: The extent of lung involvement visualized by high-resolution computed tomography (HRCT) is a predictor of decline in forced vital capacity (FVC) in scleroderma-interstitial lung disease. Our objective was to evaluate the performance of three different HRCT-defined staging systems in the Scleroderma Lung Study I (SLS I) over a 1-year period. METHODS: We assessed two visual semiquantitative scores: the maximum fibrosis score (MaxFib, the fibrosis score in the zone of maximal lung involvement) and visual assessment of total lung involvement (TLI) as proposed by Goh and Wells...
December 23, 2015: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/27548651/myopathy-in-scleroderma-and-in-other-connective-tissue-diseases
#9
Julie J Paik
PURPOSE OF REVIEW: This review discusses the most updated literature of myopathy in scleroderma and other connective tissue diseases. RECENT FINDINGS: In the past year, studies have demonstrated that myopathy in scleroderma is associated with poor outcomes such as disability and mortality. In addition, muscle histopathology in scleroderma continues to reveal that it is a heterogeneous entity, and that necrosis and acute neurogenic atrophy may be a more prevalent histopathologic feature in muscle biopsies than previously reported...
November 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27497912/pulmonary-involvement-in-systemic-sclerosis
#10
REVIEW
Adriana Morales-Cárdenas, Camila Pérez-Madrid, Liliana Arias, Paulina Ojeda, María Paula Mahecha, Adriana Rojas-Villarraga, Jorge A Carrillo-Bayona, Juan-Manuel Anaya
Systemic sclerosis (SSc) is a multi-systemic autoimmune disease that mainly affects the skin, lungs, gastrointestinal tract, heart and kidneys. Pulmonary disease in patients with SSc is strongly associated with mortality. The mechanisms involved into its pathophysiology include the activation of autoimmune cells and hyperplasia of fibroblasts with an increased capacity to produce collagen and diminished collagen breakdown. Although pulmonary biopsy is the gold standard for the diagnosis of interstitial lung disease in SSc, the most commonly used method is high-resolution computed tomography due to its high sensitivity and specificity...
November 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27593964/calcinosis-is-associated-with-digital-ischaemia-in-systemic-sclerosis-a-longitudinal-study
#11
Murray Baron, Janet Pope, David Robinson, Niall Jones, Nader Khalidi, Peter Docherty, Elzbieta Kaminska, Ariel Masetto, Evelyn Sutton, Jean-Pierre Mathieu, Sophie Ligier, Tamara Grodzicky, Sharon LeClercq, Carter Thorne, Geneviève Gyger, Douglas Smith, Paul R Fortin, Maggie Larché, Maysan Abu-Hakima, Tatiana S Rodriguez-Reyna, Antonio R Cabral-Castaneda, Marvin J Fritzler, Mianbo Wang, Marie Hudson
OBJECTIVE: To determine if ischaemia is a causal factor in the development of calcinosis in SSc. METHODS: Patients with SSc were assessed yearly. Physicians reported the presence of calcinosis, digital ischaemia (digital ulcers, digital necrosis/gangrene, loss of digital pulp on any digits and/or auto- or surgical digital amputation) and nailfold capillary dropout assessed using a dermatoscope. The number of digits with digital ischaemia was used as an assessment of the severity of digital ischaemia...
December 2016: Rheumatology
https://www.readbyqxmd.com/read/27387267/interstitial-lung-disease-in-systemic-sclerosis-progress-in-screening-and-early-diagnosis
#12
Øyvind Molberg, Anna-Maria Hoffmann-Vold
PURPOSE OF REVIEW: Interstitial lung disease (ILD) is the major determinant of morbidity and mortality in systemic sclerosis (SSc). In highly selected SSc patients, it was recently shown that stem cell therapy early in the disease course improved survival and reduced the extent of ILD, providing a rationale for early ILD detection strategies in this disease. Here, we review recent progress on ILD screening and early diagnosis in SSc. RECENT FINDINGS: Two studies showed that over 60% of unselected SSc cases with ILD by high-resolution computer tomography (HRCT) had normal range pulmonary function tests (PFTs); indicating poor performance of PFTs for ILD screening purposes...
November 2016: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/27255492/cyclophosphamide-versus-mycophenolate-mofetil-in-scleroderma-interstitial-lung-disease-ssc-ild-as-induction-therapy-a-single-centre-retrospective-analysis
#13
Padmanabha D Shenoy, Manish Bavaliya, Sujith Sashidharan, Kaveri Nalianda, Sreelakshmi Sreenath
BACKGROUND: Scleroderma is a systemic autoimmune disease characterized mainly by skin manifestations and involvement of various visceral organs, especially the lungs. Lung involvement is the leading cause of mortality in patients with scleroderma. There are data to suggest that cyclophosphamide (CYC) and mycophenolate mofetil (MMF) are effective in the management of scleroderma interstitial lung disease (SSc-ILD) but no head to head comparative data are available to date. METHODS: For the last 3 years, patients with SSc-ILD have been treated at our centre by protocol-based administration of intravenous CYC and MMF...
June 2, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/27330933/management-of-widespread-skin-thickening-in-diffuse-systemic-sclerosis
#14
Suzanne Kafaja, Philip Clements
Skin thickening is one of the early organ manifestations of systemic sclerosis (SSc) and has a great impact on quality of life (QOL) as well as overall daily living in patients with SSc. The dynamic changes that occur as the disease progresses and as other organs become further involved present the treating physician with therapeutic challenges. Hence, when considering drug therapy for skin disease, the treating physician should consider a number of factors including disease duration, the rate of skin thickening, the extent of disease progression, organ involvements, and patient-related outcome measures, all of which impact the type of treatments considered...
March 2016: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/27284161/bsr-and-bhpr-guideline-for-the-treatment-of-systemic-sclerosis
#15
REVIEW
Christopher P Denton, Michael Hughes, Nataliya Gak, Josephine Vila, Maya H Buch, Kuntal Chakravarty, Kim Fligelstone, Luke L Gompels, Bridget Griffiths, Ariane L Herrick, Jay Pang, Louise Parker, Anthony Redmond, Jacob van Laar, Louise Warburton, Voon H Ong
No abstract text is available yet for this article.
October 2016: Rheumatology
https://www.readbyqxmd.com/read/27273998/pulmonary-function-parameters-other-than-vital-capacity-should-be-considered-in-screening-for-interstitial-lung-disease-in-patients-with-systemic-sclerosis-comment-on-the-article-by-suliman-et-al
#16
LETTER
Bruno Degano, Thibaud Soumagne, Guillaume Eberst, Nadine Méaux-Ruault, Helder Gil, Nadine Magy-Bertrand
No abstract text is available yet for this article.
September 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27241706/calcinosis-in-systemic-sclerosis-subsets-distribution-and-complications
#17
Francesca Bartoli, Ginevra Fiori, Francesca Braschi, Laura Amanzi, Cosimo Bruni, Jelena Blagojevic, Silvia Bellando-Randone, Laura Cometi, Carolina de Souza Mueller, Serena Guiducci, Laura Rasero, Francesco Epifani, Daniel E Furst, Marco Matucci-Cerinic
OBJECTIVE: To retrospectively analyse the features of calcinosis in a cohort of SSc patients. METHODS: Charts of SSc patients attending the Ulcer Unit of the Rheumatology Department, University of Florence and presenting a clinical suspicion of calcinosis were considered in the study. Data on clinical history, including recent skin changes, and clinical examination of all areas with suspected calcinosis, radiological imaging of the calcinotic area, demographics and SSc-related organ involvement and pain measured by a visual analogue scale were recorded...
September 2016: Rheumatology
https://www.readbyqxmd.com/read/27156934/safety-and-efficacy-of-subcutaneous-tocilizumab-in-adults-with-systemic-sclerosis-fasscinate-a-phase-2-randomised-controlled-trial
#18
RANDOMIZED CONTROLLED TRIAL
Dinesh Khanna, Christopher P Denton, Angelika Jahreis, Jacob M van Laar, Tracy M Frech, Marina E Anderson, Murray Baron, Lorinda Chung, Gerhard Fierlbeck, Santhanam Lakshminarayanan, Yannick Allanore, Janet E Pope, Gabriela Riemekasten, Virginia Steen, Ulf Müller-Ladner, Robert Lafyatis, Giuseppina Stifano, Helen Spotswood, Haiyin Chen-Harris, Sebastian Dziadek, Alyssa Morimoto, Thierry Sornasse, Jeffrey Siegel, Daniel E Furst
BACKGROUND: Systemic sclerosis is a rare disabling autoimmune disease with few treatment options. The efficacy and safety of tocilizumab, an interleukin 6 receptor-α inhibitor, was assessed in the faSScinate phase 2 trial in patients with systemic sclerosis. METHODS: We did this double-blind, placebo-controlled study at 35 hospitals in Canada, France, Germany, the UK, and the USA. We enrolled adults with progressive systemic sclerosis of 5 or fewer years' duration from first non-Raynaud's sign or symptom...
June 25, 2016: Lancet
https://www.readbyqxmd.com/read/27016052/prediction-of-improvement-in-skin-fibrosis-in-diffuse-cutaneous-systemic-sclerosis-a-eustar-analysis
#19
Rucsandra Dobrota, Britta Maurer, Nicole Graf, Suzana Jordan, Carina Mihai, Otylia Kowal-Bielecka, Yannick Allanore, Oliver Distler
OBJECTIVES: Improvement of skin fibrosis is part of the natural course of diffuse cutaneous systemic sclerosis (dcSSc). Recognising those patients most likely to improve could help tailoring clinical management and cohort enrichment for clinical trials. In this study, we aimed to identify predictors for improvement of skin fibrosis in patients with dcSSc. METHODS: We performed a longitudinal analysis of the European Scleroderma Trials And Research (EUSTAR) registry including patients with dcSSc, fulfilling American College of Rheumatology criteria, baseline modified Rodnan skin score (mRSS) ≥7 and follow-up mRSS at 12±2 months...
October 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27094599/digital-ulcers-in-systemic-sclerosis
#20
REVIEW
Michael Hughes, Ariane L Herrick
Digital ulcers (DUs) are a common visible manifestation of the progressive vascular disease that characterizes the SSc disease process. DUs not only impact significantly on patients' quality of life and hand function, but are also a biomarker of internal organ involvement and of disease severity. The aetiology of (digital) vascular disease in SSc is multifactorial, and many of these factors are potentially amenable to therapeutic intervention. The management of DU disease in SSc is multifaceted. Patient education and non-pharmacological interventions (e...
January 2017: Rheumatology
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