Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial
Marco Matucci-Cerinic,Christopher P. Denton,Daniel E. Furst,Maureen D. Mayes,Vivien Hsu,Patrick H. Carpentier,Fredrick M. Wigley,Carol M. Black,Barri J. Fessler,Peter A. Merkel,Janet E. Pope,Nadera J. Sweiss,Mittie K. Doyle,Mittie K. Doyle,Bernhard Hellmich,Thomas A. Medsger,Adele Morganti,Fabrice Kramer,Joseph H. Korn,James R. Seibold +19 more
TLDR
Bosentan treatment reduced the occurrence of new DUs in patients with SSc but had no effect on DU healing and may be a useful adjunct in the management of patients withSSc with recurrent DUs.Citations
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Update of EULAR recommendations for the treatment of systemic sclerosis
Otylia Kowal-Bielecka,Jaap Fransen,Jérôme Avouac,Mike O Becker,Mike O Becker,Agnieszka Kulak,Yannick Allanore,Oliver Distler,Philip J. Clements,Maurizio Cutolo,László Czirják,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 J. A. van den Hoogen,Jacob M van Laar,Gabriela Riemekasten,Richard M. Silver,Vanessa Smith,Vanessa Smith,Alberto Sulli,Ingo H. Tarner,Alan Tyndall,Joep Welling,Frederic Wigley,Gabriele Valentini,Ulrich A Walker,Francesco Zulian,Ulf Müller-Ladner +38 more
TL;DR: In this article, the European League against Rheumatism (EULAR) developed a set of recommendations for the treatment of systemic sclerosis (SSc) with attention to new therapeutic questions.
Journal ArticleDOI
Raynaud's Phenomenon
TL;DR: This review updates the understanding of the pathogenesis, the approach to management, and current approaches to drug therapy of Raynaud's phenomenon.
Journal ArticleDOI
Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities:
TL;DR: This review features recent insights of the pathogenic process of SSc, highlighting three major characteristics of S Sc, microvasculopathy, excessive fibrosis, and immune dysregulation, and sheds new light on the understanding of molecular and cellular mechanisms contributing to the pathogenesis of S sc and providing novel avenues for targeted therapies.
Journal ArticleDOI
The pathogenesis, diagnosis and treatment of Raynaud phenomenon
TL;DR: An update of the pathogenesis, diagnosis and treatment of Raynaud phenomenon is provided, and current and future treatment approaches are discussed, and some key unanswered questions are highlighted.
Journal ArticleDOI
Safety, tolerability and potential efficacy of injection of autologous adipose-derived stromal vascular fraction in the fingers of patients with systemic sclerosis: an open-label phase I trial
B. Granel,Aurélie Daumas,Elisabeth Jouve,Jean-Robert Harlé,Pierre-Sébastien Nguyen,Christian Chabannon,Nathalie Colavolpe,Jean-Charles Reynier,Romain Truillet,Stéphanie Mallet,Antoine Baiada,Dominique Casanova,Laurent Giraudo,Laurent Arnaud,Julie Veran,Florence Sabatier,Guy Magalon +16 more
TL;DR: A significant improvement in hand disability and pain, Raynaud's phenomenon, finger oedema and quality of life was observed and preliminary assessments at 6 months suggest potential efficacy needing confirmation in a randomised placebo-controlled trial on a larger population.
References
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Preliminary criteria for the classification of systemic sclerosis (scleroderma)
TL;DR: A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus erythematosus, polymyositis/dermatomyositis, and Raynaud's phenomenon was conducted in order to develop classification criteria for systemic sclerosis.
Journal Article
Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee.
C Leroy,Roy D. Altman,J.B. Kirsner,Jonathan A. Myers,Dennis J. McShane,D. Masi,Gordon C. Sharp,Gerald P. Rodnan,A. MacKenzie-Graham,Thomas A. Medsger,James F. Fries +10 more
TL;DR: Proposed classification criteria for systemic sclerosis had a 97% sensitivity for definite systemic sclerosis and 98% specificity when applied to the case and comparison patients included in this study.
Journal ArticleDOI
Bosentan therapy for pulmonary arterial hypertension.
Lewis J. Rubin,David B. Badesch,Robyn J. Barst,Nazzareno Galiè,Carol M. Black,Anne Keogh,Tomás Pulido,Adaani E. Frost,Sébastien Roux,Isabelle Leconte,Michael J. Landzberg,Gérald Simonneau +11 more
TL;DR: The endothelin-receptor antagonist bosentan is beneficial in patients with pulmonary arterial hypertension and is well tolerated at a dose of 125 mg twice daily.
Journal ArticleDOI
Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial.
David B. Badesch,Victor F. Tapson,Michael D. McGoon,Bruce H. Brundage,Lewis J. Rubin,Fredrick M. Wigley,Stuart Rich,Robyn J. Barst,Pamela S. Barrett,Kenneth M. Kral,Maria M. Jöbsis,James E. Loyd,Srinivas Murali,Adaani E. Frost,Reda E. Girgis,Robert C. Bourge,David D. Ralph,C. Gregory Elliott,Nicholas S. Hill,David Langleben,Robert Schilz,Vallerie V. McLaughlin,Ivan M. Robbins,Bertron M. Groves,Shelley Shapiro,Thomas A. Medsger,Sean Gaine,Evelyn M. Horn,James C. Decker,Katharine Knobil +29 more
TL;DR: Continuous epoprostenol therapy improves exercise capacity and cardiopulmonary hemodynamics in patients with pulmonary hypertension due to the scleroderma spectrum of disease as discussed by the authors.
Annals of Internal Medicine Continuous Intravenous Epoprostenol for Pulmonary Hypertension Due to the Scleroderma Spectrum of Disease
David B. Badesch,Victor F. Tapson,Michael D. McGoon,Bruce H. Brundage,Lewis J. Rubin,Fredrick M. Wigley,Stuart Rich,Robyn J. Barst,Pamela S. Barrett,Kenneth M. Kral,James E. Loyd,Srinivas Murali,A. Frost,Reda E. Girgis,Robert C. Bourge,David D. Ralph,C. Gregory Elliott,Nicholas Hill,David Langleben,Robert J. Schilz,Vallerie V. McLaughlin,Ivan M. Robbins,Bertron M. Groves,Shelley M. Shapiro,Sean Gaine,Evelyn M. Horn,James C. Decker,Katharine Knobil +27 more
TL;DR: Results from a single-center, uncontrolled study suggest that long-term, continuously infused epoprostenol therapy can produce hemodynamic and symptomatic responses in patients with connective tissue disease who have severe pulmonary hypertension that is refractory to conventional medical therapy.
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Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist.
Joseph H. Korn,Maureen D. Mayes,M. Matucci Cerinic,Maurizio Rainisio,Janet E. Pope,Eric Hachulla,Eric Rich,Patrick H. Carpentier,Jerry A. Molitor,James R. Seibold,Vivien Hsu,Loïc Guillevin,Sharmila Chatterjee,Hans-Hartmut Peter,J. Coppock,Ariane L. Herrick,Peter A. Merkel,Robert W. Simms,Christopher P. Denton,Daniel E. Furst,N. Nguyen,M. Gaitonde,Carol M. Black +22 more
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