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esclerose sistemica

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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
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...
2015: Arthritis Research & Therapy
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
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...
August 4, 2016: Autoimmunity Reviews
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...
September 4, 2016: Rheumatology
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
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...
2016: Arthritis Research & Therapy
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
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
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
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
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
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
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...
April 19, 2016: Rheumatology
Dilia Giuggioli, Federica Lumetti, Michele Colaci, Poupak Fallahi, Alessandro Antonelli, Clodoveo Ferri
BACKGROUND: The treatment of systemic sclerosis (SSc) represents a great clinical challenge because of the complex disease pathogenesis including vascular, fibrotic, and immune T- and B-lymphocyte-mediated alterations. Therefore, SSc should be treated by combined or sequential therapies according to prevalent clinico-pathogenetic phenotypes. Some preliminary data suggest that rituximab (RTX) may downregulate the B-cell over expression and correlated immunological abnormalities. METHODS: Here, we describe a series of 10 SSc patients (4M and 6F, mean age 46±13...
November 2015: Autoimmunity Reviews
Anne Claire Desbois, Patrice Cacoub
Systemic sclerosis (SSc) is a chronic immune disorder of unknown origin, dominated by excessive fibrosis responsible for cutaneous and pulmonary fibrosis, and by vascular endothelial dysfunction at the origin of skin ischemia, renal and pulmonary artery lesions. Renal and pulmonary complications are mainly responsible for the severity of the disease. Recent advances led to a better understanding of pathological mechanisms and a more accurate classification of patients according to clinical and biological (auto-antibodies) phenotype...
May 2016: Autoimmunity Reviews
Hyun J Kim, Donald P Tashkin, David W Gjertson, Matthew S Brown, Eric Kleerup, Semin Chong, John A Belperio, Michael D Roth, Fereidoun Abtin, Robert Elashoff, Chi-Hong Tseng, Dinesh Khanna, Jonathan G Goldin
OBJECTIVES: The aim is to investigate whether the 12-month quantitative changes in high-resolution CT (HRCT) measures of interstitial lung disease (ILD) are different, and to understand how they change, in patients with scleroderma-related ILD who receive drug therapy versus placebo. METHODS: HRCT images were acquired at baseline and at 12 months in 83 participants in Scleroderma Lung Study I, a clinical trial comparing treatment with oral cyclophosphamide versus placebo...
July 2016: Annals of the Rheumatic Diseases
Lazaros I Sakkas, Theodora Simopoulou, Christina Katsiari, Dimitrios Bogdanos, Ian C Chikanza
Systemic sclerosis (SSc) is characterized by microvasculopathy (Raynaud's phenomenon and fibrointimal proliferation), presence of autoantibodies and collagen deposition in skin (scleroderma) and internal organs. Microvasculopathy, detected by nailfold capillaroscopy, and disease-specific autoantibodies (anti-topoisomerase I, anti-centromere, anti-RNA polymerase III antibodies) usually appear earlier, even years before scleroderma. At that stage of the disease, immune activation with T cells and B cells promote fibrosis...
August 2015: Clinical Rheumatology
Michele Iudici, Gianluca Moroncini, Paola Cipriani, Roberto Giacomelli, Armando Gabrielli, Gabriele Valentini
Interstitial lung disease (ILD) affects about 90% of patients with systemic sclerosis (SSc). It is associated with a restrictive lung disease in only 30% of patients and is progressive in an even lower percentage. A low forced vital capacity at presentation, an extent of lung fibrosis >20% as detected by lung high-resolution computed tomography, high serum interleukin-6 levels, anti-topoisomerase I antibody positivity and diffuse cutaneous SSc are each associated with SSc-ILD progression. However, no such association is absolute...
July 2015: Autoimmunity Reviews
Alexandra Audemard, Nicolas Martin Silva, Jonathan Boutemy, Gwenola Maigné, Boris Bienvenu
No abstract text is available yet for this article.
February 2015: Journal of Rheumatology
2016-02-21 10:29:01
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