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Systemic sclerosis

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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/26168983/update-on-biomarkers-in-systemic-sclerosis-tools-for-diagnosis-and-treatment
#2
REVIEW
Alsya J Affandi, Timothy R D J Radstake, Wioleta Marut
Systemic sclerosis (SSc) is a complex autoimmune disease in which immune activation, vasculopathy, and extensive fibrosis of the skin and internal organs are among the principal features. SSc is a heterogeneous disease with varying manifestations and clinical outcomes. Currently, patients' clinical evaluation often relies on subjective measures, non-quantitative methods, or requires invasive procedures as markers able to predict disease trajectory or response to therapy are lacking. Therefore, current research is focusing on the discovery of useful biomarkers reflecting ongoing inflammatory or fibrotic activity in the skin and internal organs, as well as being predictive of future disease course...
September 2015: Seminars in Immunopathology
https://www.readbyqxmd.com/read/28039187/initial-combination-therapy-with-ambrisentan-and-tadalafil-in-connective-tissue-disease-associated-pulmonary-arterial-hypertension-ctd-pah-subgroup-analysis-from-the-ambition-trial
#3
John Gerry Coghlan, Nazzareno Galiè, Joan Albert Barberà, Adaani E Frost, Hossein-Ardeschir Ghofrani, Marius M Hoeper, Masataka Kuwana, Vallerie V McLaughlin, Andrew J Peacock, Gérald Simonneau, Jean-Luc Vachiéry, Christiana Blair, Hunter Gillies, Karen L Miller, Julia H N Harris, Jonathan Langley, Lewis J Rubin
BACKGROUND: Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), in particular systemic sclerosis (SSc), had an attenuated response compared with idiopathic PAH in most trials. Thus, there is uncertainty regarding the benefit of PAH-targeted therapy in some forms of CTD-PAH. OBJECTIVE: To explore the safety and efficacy of initial combination therapy with ambrisentan and tadalafil versus ambrisentan or tadalafil monotherapy in patients with CTD-PAH and SSc-PAH enrolled in the AMBITION trial...
December 30, 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
#4
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/27330933/management-of-widespread-skin-thickening-in-diffuse-systemic-sclerosis
#5
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/27156934/safety-and-efficacy-of-subcutaneous-tocilizumab-in-adults-with-systemic-sclerosis-fasscinate-a-phase-2-randomised-controlled-trial
#6
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
#7
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/26782006/strategy-for-treatment-of-fibrosis-in-systemic-sclerosis-present-and-future
#8
REVIEW
Koichi Yanaba
Systemic sclerosis (SSc) is a generalized connective tissue disorder characterized by microvascular damage, autoimmunity, and excessive fibrosis of the skin and various internal organs. Regardless of the recent progress in medicine, no radical therapy for SSc has been developed, and the risk of mortality remains high. Therefore, diagnosis in the early disease stage, risk stratification for the development of serious organ involvement and therapeutic intervention with disease-modifying drugs can reduce the maximum degree of fibrosis, leading to improved long-term survival...
January 2016: Journal of Dermatology
https://www.readbyqxmd.com/read/26324802/interstitial-lung-disease-in-systemic-sclerosis-where-do-we-stand
#9
REVIEW
Susanna Cappelli, Silvia Bellando Randone, Gianna Camiciottoli, Amato De Paulis, Serena Guiducci, Marco Matucci-Cerinic
Interstitial lung disease (ILD) is common in systemic sclerosis (SSc) patients and despite recent advances in the treatment is, at present, the major cause of death. Today, an early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease. Pulmonary function tests and high-resolution computed tomography remain the mainstay for the diagnosis of SSc-ILD, but there is a growing interest in lung ultrasound. Recently, the correlation between severity of fibrosis and some peripheral blood biomarkers has been described...
September 2015: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/24122180/2013-classification-criteria-for-systemic-sclerosis-an-american-college-of-rheumatology-european-league-against-rheumatism-collaborative-initiative
#10
Frank van den Hoogen, Dinesh Khanna, Jaap Fransen, Sindhu R Johnson, Murray Baron, Alan Tyndall, Marco Matucci-Cerinic, Raymond P Naden, Thomas A Medsger, Patricia E Carreira, Gabriela Riemekasten, Philip J Clements, Christopher P Denton, Oliver Distler, Yannick Allanore, Daniel E Furst, Armando Gabrielli, Maureen D Mayes, Jacob M van Laar, James R Seibold, Laszlo Czirjak, Virginia D Steen, Murat Inanc, Otylia Kowal-Bielecka, Ulf Müller-Ladner, Gabriele Valentini, Douglas J Veale, Madelon C Vonk, Ulrich A Walker, Lorinda Chung, David H Collier, Mary Ellen Csuka, Barri J Fessler, Serena Guiducci, Ariane Herrick, Vivien M Hsu, Sergio Jimenez, Bashar Kahaleh, Peter A Merkel, Stanislav Sierakowski, Richard M Silver, Robert W Simms, John Varga, Janet E Pope
OBJECTIVE: The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc. METHODS: Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc...
November 2013: Arthritis and Rheumatism
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