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esclerose sistemica

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36 papers 0 to 25 followers
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
#1
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
#2
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-nailfoldcapillaroscopy-in-the-assessment-of-internal-organ-involvement-in-systemic-sclerosis-a-critical-review
#3
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...
May 30, 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
#4
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 one and two years in interstitial lung disease associated with systemic sclerosis (SSc-ILD). METHODS: The prognostic significance of PFT trends at one year (n=162), and two years (n=140) was examined against 15 year survival. PFT trends, expressed as continuous and categorical change in separate analyses, were examined against mortality in univariate and multivariate models. SSc-ILD was defined at presentation as limited lung fibrosis or extensive lung fibrosis using the UKRSA staging system...
April 20, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28413064/systemic-sclerosis
#5
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/28376288/mycophenolate-mofetil-versus-placebo-for-systemic-sclerosis-related-interstitial-lung-disease-an-analysis-of-scleroderma-lung-studies-i-and-ii
#6
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...
April 4, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28228650/haematopoietic-stem-cell-transplantation-for-autoimmune-diseases
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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...
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
#15
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
#16
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
#17
REVIEW
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
#18
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
#19
MULTICENTER STUDY
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
#20
REVIEW
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
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