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esclerose sistemica

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41 papers 25 to 100 followers
https://www.readbyqxmd.com/read/29066464/safety-and-efficacy-of-subcutaneous-tocilizumab-in-systemic-sclerosis-results-from-the-open-label-period-of-a-phase-ii-randomised-controlled-trial-fasscinate
#1
Dinesh Khanna, Christopher P Denton, Celia Jf Lin, 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, Helen Spotswood, Laura Burke, Jeffrey Siegel, Angelika Jahreis, Daniel E Furst
OBJECTIVES: Assess the efficacy and safety of tocilizumab in patients with systemic sclerosis (SSc) in a phase II study. METHODS: Patients with SSc were treated for 48 weeks in an open-label extension phase of the faSScinate study with weekly 162 mg subcutaneous tocilizumab. Exploratory end points included modified Rodnan Skin Score (mRSS) and per cent predicted forced vital capacity (%pFVC) through week 96. RESULTS: Overall, 24/44 (55%) placebo-tocilizumab and 27/43 (63%) continuous-tocilizumab patients completed week 96...
October 24, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#2
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28835464/mapping-and-predicting-mortality-from-systemic-sclerosis
#3
Muriel Elhai, Christophe Meune, Marouane Boubaya, Jérôme Avouac, Eric Hachulla, Alexandra Balbir-Gurman, Gabriela Riemekasten, Paolo Airò, Beatriz Joven, Serena Vettori, Franco Cozzi, Susanne Ullman, László Czirják, Mohammed Tikly, Ulf Müller-Ladner, Paola Caramaschi, Oliver Distler, Florenzo Iannone, Lidia P Ananieva, Roger Hesselstrand, Radim Becvar, Armando Gabrielli, Nemanja Damjanov, Maria J Salvador, Valeria Riccieri, Carina Mihai, Gabriella Szücs, Ulrich A Walker, Nicolas Hunzelmann, Duska Martinovic, Vanessa Smith, Carolina de Souza Müller, Carlo Maurizio Montecucco, Daniela Opris, Francesca Ingegnoli, Panayiotis G Vlachoyiannopoulos, Bojana Stamenkovic, Edoardo Rosato, Stefan Heitmann, Jörg H W Distler, Thierry Zenone, Matthias Seidel, Alessandra Vacca, Ellen De Langhe, Srdan Novak, Maurizio Cutolo, Luc Mouthon, Jörg Henes, Carlo Chizzolini, Carlos Alberto von Mühlen, Kamal Solanki, Simona Rednic, Lisa Stamp, Branimir Anic, Vera Ortiz Santamaria, Maria De Santis, Sule Yavuz, Walter Alberto Sifuentes-Giraldo, Emmanuel Chatelus, Jiri Stork, Jacob van Laar, Esthela Loyo, Paloma García de la Peña Lefebvre, Kilian Eyerich, Vanesa Cosentino, Juan Jose Alegre-Sancho, Otylia Kowal-Bielecka, Grégoire Rey, Marco Matucci-Cerinic, Yannick Allanore
OBJECTIVES: To determine the causes of death and risk factors in systemic sclerosis (SSc). METHODS: Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres...
November 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28778710/how-i-treat-patients-with-systemic-sclerosis-in-clinical-practice
#4
REVIEW
Daniela Rossi, Elisabetta Zanatta, Piero Marson, Savino Sciascia, Pamela Polito, Dario Roccatello, Franco Cozzi
Systemic sclerosis (SSc) or scleroderma is a disorder of the connective tissue affecting the skin, and it is often associated with visceral involvement. The predominant pathological features of SSc are autoimmunity, vasculopathy, and fibrosis. Progressive fibrosis is associated with changes in the microcirculation of the involved organs. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management, reviewing available guidelines and recommendations and providing experts' insights...
August 1, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28680133/systemic-sclerosis-choosing-patients-wisely-when-treating-interstitial-lung-disease
#5
Richard M Silver
No abstract text is available yet for this article.
August 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28619061/rituximab-versus-cyclophosphamide-for-the-treatment-of-connective-tissue-disease-associated-interstitial-lung-disease-recital-study-protocol-for-a-randomised-controlled-trial
#6
Peter Saunders, Vicky Tsipouri, Gregory J Keir, Deborah Ashby, Marcus D Flather, Helen Parfrey, Daphne Babalis, Elisabetta A Renzoni, Christopher P Denton, Athol U Wells, Toby M Maher
BACKGROUND: Interstitial lung disease (ILD) frequently complicates systemic autoimmune disorders resulting in considerable morbidity and mortality. The connective tissue diseases (CTDs) most frequently resulting in ILD include: systemic sclerosis, idiopathic inflammatory myositis (including dermatomyositis, polymyositis and anti-synthetase syndrome) and mixed connective tissue disease. Despite the development, over the last two decades, of a range of biological therapies which have resulted in significant improvements in the treatment of the systemic manifestations of CTD, the management of CTD-associated ILD has changed little...
June 15, 2017: Trials
https://www.readbyqxmd.com/read/28544580/efficacy-of-mycophenolate-mofetil-and-oral-cyclophosphamide-on-skin-thickness-post-hoc-analyses-from-the-scleroderma-lung-study-i-and-ii
#7
Rajaie Namas, Donald P Tashkin, Daniel E Furst, Holly Wilhalme, Chi-Hong Tseng, Michael D Roth, Suzanne Kafaja, Elizabeth Volkmann, Philip J Clements, Dinesh Khanna
OBJECTIVES: To assess the efficacy of mycophenolate mofetil (MMF) and cyclophosphamide (CYC) on the modified Rodnan skin score (mRSS) in participants enrolled in the Scleroderma Lung Study (SLS)-I and II. METHODS: SLS-I participants received daily oral CYC or matching placebo for one year, whereas SLS-II participants received daily MMF for 2 years or daily oral CYC for 1 year followed by placebo for second year. We assessed the impact of MMF and CYC on the mRSS in SLS-II over 24-month period...
May 23, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28576600/the-role-of-nailfold-capillaroscopy-in-the-assessment-of-internal-organ-involvement-in-systemic-sclerosis-a-critical-review
#8
REVIEW
Stergios Soulaidopoulos, Eva Triantafyllidou, Alexandros Garyfallos, George D Kitas, Theodoros Dimitroulas
Endothelial dysfunction and microvascular damage constitute the hallmarks of systemic sclerosis (SSc), explaining much of the pathophysiology and clinical manifestations of the disease. Nailfold videocapillaroscopy (NVC) is an established method for the assessment of the microvasculature, aiding in distinguishing different types of structural vascular abnormalities. Until recently, NVC was used in the diagnosis of SSc as well as in the assessment and follow-up of peripheral digital vasculopathy. On the top of digital ulcers, internal organ involvement such as myocardial dysfunction, pulmonary vascular and/or parenchymal lung disease characterizes severe SSc imparting a high risk of mortality...
August 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28426895/short-term-pulmonary-function-trends-are-predictive-of-mortality-in-interstitial-lung-disease-associated-with-systemic-sclerosis
#9
Nicole S Goh, Rachel K Hoyles, Christopher P Denton, David M Hansell, Elisabetta A Renzoni, Toby M Maher, Andrew G Nicholson, Athol U Wells
OBJECTIVE: To determine the prognostic value of pulmonary function test (PFT) trends at 1 and 2 years in interstitial lung disease (ILD) associated with systemic sclerosis (SSc). METHODS: The prognostic significance of PFT trends at 1 year (n = 162) and 2 years (n = 140) was examined against 15-year survival in patients with SSc-associated ILD. PFT trends, expressed as continuous change and as categorical change in separate analyses, were examined against mortality in univariate and multivariate models...
August 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#10
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...
October 7, 2017: Lancet
https://www.readbyqxmd.com/read/28376288/mycophenolate-mofetil-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#11
COMPARATIVE STUDY
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 mofetil (MMF) with placebo for the treatment of systemic sclerosis (SSc)-related interstitial lung disease (ILD). METHODS: We included participants enrolled in the placebo arm of Scleroderma Lung Study (SLS) I and the MMF arm of SLS II. SLS I randomized participants to receive either oral cyclophosphamide (CYC) or placebo for 1 year, while SLS II randomized participants to receive either MMF for 2 years or oral CYC for 1 year followed by 1 year of placebo...
July 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28228650/haematopoietic-stem-cell-transplantation-for-autoimmune-diseases
#12
REVIEW
Joost F Swart, Eveline M Delemarre, Femke van Wijk, Jaap-Jan Boelens, Jürgen Kuball, Jacob M van Laar, Nico M Wulffraat
Autologous haematopoietic stem cell transplantation (HSCT) is the only treatment that is able to induce long-term, drug-free and symptom-free remission in several refractory autoimmune rheumatic diseases. Over 3,000 HSCT procedures for rheumatic and nonrheumatic severe autoimmune diseases have been performed worldwide. Specific conditioning regimens are currently used to eradicate the autoreactive immunological memory of patients. Although in vivo immune cell depletion with antithymocyte globulin or anti-CD52 is the norm for many regimens, ex vivo selection of CD34(+) stem cells from the graft is controversial...
April 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28232167/intravenous-immunoglobulins-in-systemic-sclerosis-data-from-a-french-nationwide-cohort-of-46-patients-and-review-of-the-literature
#13
REVIEW
Sébastien Sanges, Sébastien Rivière, Arsène Mekinian, Thierry Martin, Alain Le Quellec, Emmanuel Chatelus, Alain Lescoat, Patrick Jego, Claire Cazalets, Thomas Quéméneur, Noémie Le Gouellec, Patricia Senet, Camille Francès, Alban Deroux, Bernard Imbert, Olivier Fain, Latifatou Boukari, Thomas Sené, Christophe Deligny, Alexis Mathian, Christian Agard, Grégory Pugnet, Silvia Speca, Sylvain Dubucquoi, Pierre-Yves Hatron, Éric Hachulla, David Launay
BACKGROUND: As intravenous immunoglobulins (IVIG) exhibit immunomodulatory and antifibrotic properties, they may be a relevant treatment for systemic sclerosis (SSc). The objectives of this work were thus to report on the efficacy and safety of IVIG in a population of SSc patients and to review the available literature. METHODS: 46 patients from 19 French centers were retrospectively recruited. They were included if they had a diagnosis of SSc and received at least 1 IVIG infusion at a dosage >1g/kg/cycle...
April 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28188239/treatment-outcome-in-early-diffuse-cutaneous-systemic-sclerosis-the-european-scleroderma-observational-study-esos
#14
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'...
July 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28128745/endothelial-dysfunction-microvascular-damage-and-ischemic-peripheral-vasculopathy-in-systemic-sclerosis
#15
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
#16
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...
June 2017: Rheumatology International
https://www.readbyqxmd.com/read/28049126/comorbidities-in-interstitial-lung-diseases
#17
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
#18
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...
June 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/27941129/update-of-eular-recommendations-for-the-treatment-of-systemic-sclerosis
#19
Otylia Kowal-Bielecka, Jaap Fransen, Jerome Avouac, Mike Becker, Agnieszka Kulak, Yannick Allanore, Oliver Distler, Philip Clements, Maurizio Cutolo, Laszlo Czirjak, Nemanja Damjanov, Francesco Del Galdo, Christopher P Denton, Jörg H W Distler, Ivan Foeldvari, Kim Figelstone, Marc Frerix, Daniel E Furst, Serena Guiducci, Nicolas Hunzelmann, Dinesh Khanna, Marco Matucci-Cerinic, Ariane L Herrick, Frank van den Hoogen, Jacob M van Laar, Gabriela Riemekasten, Richard Silver, Vanessa Smith, Alberto Sulli, Ingo Tarner, Alan Tyndall, Joep Welling, Frederic Wigley, Gabriele Valentini, Ulrich A Walker, Francesco Zulian, Ulf Müller-Ladner
The aim was to update the 2009 European League against Rheumatism (EULAR) recommendations for the treatment of systemic sclerosis (SSc), with attention to new therapeutic questions. Update of the previous treatment recommendations was performed according to EULAR standard operating procedures. The task force consisted of 32 SSc clinical experts from Europe and the USA, 2 patients nominated by the pan-European patient association for SSc (Federation of European Scleroderma Associations (FESCA)), a clinical epidemiologist and 2 research fellows...
August 2017: 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
#20
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
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