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  • Abstract Number: 844 • 2016 ACR/ARHP Annual Meeting

    Bardoxolone Methyl Produces Durable Benefits in Participants with Pulmonary Arterial Hypertension: Data from an Open-Label Extension Study

    Ron Oudiz1, Colin Meyer2, Melanie Chin2, Jeremy Feldman3, Angie Goldsberry2, John McConnell4, Peter A. McCullough5, Megan O'Grady2, Victor Tapson6, Fernando Torres7, Aaron B. Waxman8 and R. James White9, 1Los Angeles Biomedical Research Inst. at Harbor-UCLA Medical Center, Torrance, CA, 2Reata Pharmaceuticals, Irving, TX, 3Arizona Pulmonary Specialists, Phoenix, AZ, 4Kentuckiana Pulmonary Associates, Louisville, KY, 5Baylor University Medical Center, Dallas, TX, 6Cedars-Sinai Medical Center, Los Angeles, CA, 7University of Texas, Southwestern Medical Center, Dallas, TX, 8Harvard Medical School, Boston, MA, 9University of Rochester Medical Center, Rochester, NY

    Background/Purpose:   Bardoxolone methyl (BARD), an activator of the transcription factor Nrf2, and inhibitor of NF-kB, targets dysfunctional inflammatory, metabolic, and bioenergetic pathways. In an initial…
  • Abstract Number: 845 • 2016 ACR/ARHP Annual Meeting

    Current Use of Off-Label Therapies in Systemic Sclerosis-Associated Interstitial Lung Disease

    Elise Siegert1, Dörte Huscher2, Ulf Müller-Ladner3, Veronika K. Jaeger4, Ulrich A. Walker4, Marc Frerix5, László Czirják6, Francesco Del Galdo7, Gabriele Valentini8, Marco Matucci-Cerinic9, Yannick Allanore10, Oliver Distler11, Christopher Denton12, Gabriela Riemekasten13 and EUSTAR co-authors, 1Rheumatology and Clinical Immunology, Charité – University Medicine Berlin, Berlin, Germany, 2Epidemiology, German Rheumatism Research Centre, Berlin, Germany, 3Department of Internal Medicine and Rheumatology, Justus-Liebig-University Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, 4Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 5Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, 6Department of Rheumatology and Immunology, University of Pécs, Faculty of Medicine, Pécs, Hungary, 7Musculoskeletal Diseases, Scleroderma Research Program, Leeds Institute of Molecular Medicine, Division of Musculoskeletal Diseases, University of Leeds, Leeds, United Kingdom, 8Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, 9Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy, 10Rheumatology, Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France, 11Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 12Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 13Department of Rheumatology, Universitatsklinikum Schleswig-Holstein, Lubeck, Germany

    Background/Purpose: Systemic Sclerosis (SSc) is a connective tissue disease that is often complicated by secondary interstitial lung disease (SSc-ILD). Due to its high morbidity and…
  • Abstract Number: 846 • 2016 ACR/ARHP Annual Meeting

    Progression of Left Ventricular Myocardial Dysfunction in Systemic Sclerosis: Using Speckle Tracking Strain Echocardiography to Identify Patients at Risk

    Susanne Van Wijngaarden1, Samira Ben Said- Bouyeri2, Maarten K. Ninaber3, J.J. Bax1, V. Delgado1, Jeska K. de Vries-Bouwstra4 and Nina Ajmone Marsan1, 1Heart and Lung Center, Leiden University Medical Center, Leiden, Netherlands, 2Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 3Heart and Lung Center; Pulmonology, Leiden University Medical Center, Leiden, Netherlands, 4Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: Cardiac involvement is a main cause of mortality in systemic sclerosis, although reported prevalence of cardiac involvement is low and detection of cardiac involvement…
  • Abstract Number: 847 • 2016 ACR/ARHP Annual Meeting

    Forced Vital Capacity Predicts Lung Fibrosis Progression and Mortality in Systemic Sclerosis

    Anna Hoffmann-Vold1, Elizabeth R. Volkmann2, Oyvind Midtvedt3, Torhild Garen3, Anders Heiervang Tennøe3, Trond Mogens Aalokken4, May Brit Lund5 and Øyvind Molberg3, 1Division of Rheumatology, Oslo University Hospital, Oslo, Norway, 2University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, 3Rheumatology, Oslo University Hospital, Oslo, Norway, 4Radiology, Oslo University Hospital, Oslo, Norway, 5Respiratory Medicine, Oslo University Hospital, Oslo, Norway

    Background/Purpose:  Systemic sclerosis (SSc) carries high risk for progressive interstitial lung disease (ILD), but there are no valid methods for early detection of SSc-ILD or…
  • Abstract Number: 848 • 2016 ACR/ARHP Annual Meeting

    Efficacy of Prucalopride in the Treatment of Systemic Sclerosis-Related Intestinal Involvement: Results from an Open Label Cross-over Study

    Barbara Vigone1, Monica Caronni1, Adriana Severino1, Chiara Bellocchi2, Anna Rita Baldassarri3, Gaia Montanelli4, Alessandro Santaniello1 and Lorenzo Beretta1, 1Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 2Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases,, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 3Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milan, Italy, 4Scleroderma Unit, Referral Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy

    Background/Purpose: The gastrointestinal tract (GIT) is frequently affected in SSc patients as a consequence of a reduction in enteric propulsive forces. Due to intestinal involvement,…
  • Abstract Number: 849 • 2016 ACR/ARHP Annual Meeting

    Collagen Formation/Degradation Neoepitopes Are Promising Biomarkers for Systemic Sclerosis

    Rucsandra Dobrota1, Suzana Jordan1, Pernille Juhl2, Lukas Wildi1, Britta Maurer1, Anne C. Bay-Jensen3, Morten Asser Karsdal3, Anne Sofie Siebuhr3 and Oliver Distler1, 1Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, 2Biomarkers & Research, Nordic Bioscience, Herlev, Denmark, 3Rheumatology, Nordic Bioscience, Herlev, Denmark

    Background/Purpose: Systemic sclerosis (SSc) is a complex autoimmune disease with extensive fibrosis of the skin and internal organs in which extracellular matrix (ECM) remodeling is…
  • Abstract Number: 850 • 2016 ACR/ARHP Annual Meeting

    A Multicentre Reliability Study of Laser Speckle Contrast Imaging and Thermography in Patients with Raynaud’s Phenomenon Secondary to Systemic Sclerosis

    Andrea Murray1, Joanne Manning2, Tonia Moore3,4, Jack Wilkinson5, Elizabeth J. Marjanovic6, Sarah Leggett7, Chris Roberts8, John Allen9, Jason Britton10, Maya H. Buch11, Francesco Del Galdo12, Christopher Denton13, Tracey Drayton14, Anita Furlong14, Bridget Griffiths15, Frances Hall16, Darren Hart17, Kevin Howell18, Audrey MacDonald19, Neil J. McHugh20, John D. Pauling21, Jacqueline Shipley22 and Ariane L. Herrick23, 1University of Manchester, Salford Royal Hospital, Salford, United Kingdom, 2Rheumatology Department, Salford Royal NHS Foundation Trust, Salford, United Kingdom, 3Salford Royal Hospital NHS Foundation Trust, Salford, United Kingdom, 4Centre for Musculoskeletal Research, University of Manchester, MAHSC, Salford Royal Hospital, Salford, United Kingdom, 5Research and Development, Salford Royal NHS Foundation Trust, Salford, United Kingdom, 6Institute of Inflammation and Repair, University of Manchester, Manchester, United Kingdom, 7Arthritis Research UK Centre for Epidemiology, The University of Manchester, Manchester, United Kingdom, 8Centre for Biostatistics, The University of Manchester, Manchester, United Kingdom, 9Regional Medical Physics Department, Freeman Hospital, Newcastle upon Tyne, United Kingdom, 10Medical Physics Department, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, 11NIHR-Leeds Musculoskeletal Biomedical Research Unit, Leeds, United Kingdom, 12Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds and NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, 13Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 14Addenbrookes Hospital, Cambridge, United Kingdom, 15Rheumatology, Freeman Hospital, Newcastle Upon Tyne, United Kingdom, 16School of Clinical Medicine, University of Cambridge, Cambridge, United Kingdom, 17Clinical Measurement Department, Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, 18Institute of Immunity and Transplantation, University College London, Royal Free Campus, London, United Kingdom, 19The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, United Kingdom, 20Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, 21Upper Borough Walls, Royal National Hospital for Rheumatic Disease, Bath, United Kingdom, 22Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, 23Centre for Musculoskeletal Research, University of Manchester, MAHSC, Salford Royal Hospital, Manchester, United Kingdom

    Background/Purpose: Effective treatments for systemic sclerosis (SSc) related Raynaud’s phenomenon (RP) are badly needed but clinical trials have been hampered by the lack of objective…
  • Abstract Number: 851 • 2016 ACR/ARHP Annual Meeting

    Perioral Autologous FAT Transplantation  Is More Effective THAN Hyaluronic Acid Filler on Scleroderma Skin Fibrosis: Results from a LONG TERM Controlled Study

    Nicoletta Del Papa1, Eleonora Zaccara1, Romina Andracco1, Wanda Maglione1, Francesca Pignataro1, Fabio Caviggioli2, Gabriele Di Luca3, Antonina Prafioriti4 and Claudio Vitali5, 1Dept. Rheumatology, G. Pini Hospital, Milano, Italy, 2UOC Chirurgia Plastica, UOC Chirurgia Plastica, Multimedica Holding SpA, Milano, Italy, 3UOS Chirurgia Vascolare,, Osp. G. Pini, Milano, Italy, 4Pathology Unit, Istituto G.Pini, Milan, Italy, 5Rheumatology Section, Istituto San Giuseppe, Como, Italy

    Background/Purpose: Autologous fat tissue grafting (AFTG) has been successfully used in the treatment of different sclerotic conditions, including scleroderma. We evaluated in patients with SSc…
  • Abstract Number: 852 • 2016 ACR/ARHP Annual Meeting

    Targeting Fibroblastoid-like Cells By Drug Loaded Engineered Gold Nanoparticles As a Novel Approach for ILD-SSc Treatment

    Veronica Codullo1, Emanuela Cova2, Simona Inghilleri2, Miriam Colombo3, Davide Prosperi3, Federica Meloni4 and Carlomaurizio Montecucco5, 1Division of Rheumatology, University of Pavia, IRCCS Foundation Policlinico S. Matteo, Pavia, Italy, 2Clinica di Malattie dell’Apparato Respiratorio, IRCCS Fondazione Policlinico S Matteo, Pavia, Italy, Pavia, Italy, 3Dipartimento di Biotecnologie e Bioscienze, Università di Milano-Bicocca, Milan, Italy, 4Dipartimento di Medicina Interna, Unità di Pneumologia e UOC di Reumatologia, Università di Pavia, Pavia, Italy, 5Rheumatology Unit of the University Hospital of Pavia, Pavia, Italy

    Background/Purpose:  Systemic Sclerosis (SSc) is an autoimmune fibrotic disorder characterised by collagen and extracellular matrix deposition in the skin and internal organs, such as the…
  • Abstract Number: 853 • 2016 ACR/ARHP Annual Meeting

    Clinical Associations of Anti-Eukaryotic Initiation Factor 2B (anti-eIF2B) Antibodies in a Large Cohort of Patients with Anti-Nuclear Antibody Negative Systemic Sclerosis

    John Pauling1,2, Hui Lu1, Zoe Betteridge1,2, Gloria Salazar3, Shervin Assassi3, Maureen D Mayes3 and Neil J. McHugh1,4, 1Department of Pharmacy and Pharmacology, University of Bath, Bath, United Kingdom, 2Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, 3Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 4Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom

    Background/Purpose:  Autoantibodies can be characterized in up to 95% of patients with systemic sclerosis (SSc) and provide enormous diagnostic and prognostic value in the clinical…
  • Abstract Number: 854 • 2016 ACR/ARHP Annual Meeting

    The Ducas: Proposal for a Digital Ulcer Assessment Score in Scleroderma

    Cosimo Bruni1, Tanaka Ngcozana2, Francesca Braschi3, Guya Piemonte4, Laura Benelli4, Serena Guiducci5, Silvia Bellando-Randone3, Jonathan Grotts6, Christopher Denton7, Daniel E. Furst8 and Marco Matucci-Cerinic5, 1Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Firenze, Italy, 2Rheumatology Department, Lower, Royal Free hospital, London, United Kingdom, 3Department of Clinical and Experimental Medicine, Division of Rheumatology, University of Florence, Firenze, Italy, 4University of Florence, Florence, Italy, 5Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy, 6Biostatistics, University of California Los Angeles, Los Angeles, CA, 7Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, 8Division of Rheumatology, Department of Internal Medicine, University of California Los Angeles, David Geffen School of Medicine, Los Angeles, CA

    Background/Purpose: No objective measure is presently available to assess digital ulcer (DU) in SSc patients apart from “healed/non healed” and experience-based clinical judgment. The aim…
  • Abstract Number: 855 • 2016 ACR/ARHP Annual Meeting

    The Relationship Between Systemic Sclerosis and Breast Cancer and the Effects on Treatment Outcome

    Pichaya O-charoen1, Katherine Glass2, William Messner3 and Soumya Chatterjee4, 1Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, 2Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, 3Quantitative Health Sciences, Cleveland Clinic, Cleveland, OH, 4Rheumatic and Immunologic Ds, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Breast cancer has been shown to be more prevalent in patients with systemic sclerosis (SSc) compared to that in the general population. A close…
  • Abstract Number: 856 • 2016 ACR/ARHP Annual Meeting

    Rho Kinase Ezpression in Giant Cell Arteritis: Validating Perm Intensity Score As a Method of Increasing Sensitivity of Temporal Artery Biopsy

    Lindsay Lally1, Navneet Narula2, Nicola Goodfellow3, Raashid Luqmani4 and Robert F. Spiera5, 1Rheumatology, Hospital for Special Surgery, New York, NY, 2Pathology, Weill Cornell Medical College, New York, NY, 3Oxford, Oxofrd, United Kingdom, 4Oxford, Oxford, United Kingdom, 5Hospital for Special Surgery, Cornell, New York, NY

    Background/Purpose: Aberrant rho-kinase (ROCK) activity is implicated in pathogenesis of several vascular and immunologic disorders. We previously demonstrated evidence of increased ROCK activity in histopathologically…
  • Abstract Number: 857 • 2016 ACR/ARHP Annual Meeting

    Ultrasound Cut-Off Values for Intima-Media Thickness of Temporal, Facial and Axillary Arteries in Giant Cell Arteritis

    Valentin S. Schäfer1, Aaron Juche2, Sofia Ramiro3, Andreas Krause2 and Wolfgang A. Schmidt4, 1Immanuel Krankenhaus Berlin, Medical Center for Rheumatology Berlin-Buch, Berlin, Germany, 2Medical Centre for Rheumatology Berlin-Buch, Immanuel Krankenhaus Berlin, Berlin, Germany, 3Department of Rheumatology, Leiden University Medical Centre, Leiden, Netherlands, 4Medical Center for Rheumatology and Clinical Immunology Berlin-Buch, Immanuel Krankenhaus Berlin, Berlin, Germany

    Abstract Background/Purpose: Ultrasound (US) is increasingly used in the diagnosis of giant cell arteritis (GCA). US findings mainly rely on morphology (“halo-sign” and “compression sign”).…
  • Abstract Number: 858 • 2016 ACR/ARHP Annual Meeting

    Is There a Place for Repetitive 18f-Fluorodeoxyglucose Positron Emission Tomography in Giant-Cell Arteritis with Large-Vessel Involvement?

    Hubert de Boysson1, Eric Liozon2, Marc Lambert3, Jonathan Boutemy1, Gwénola Maigné1, Nicolas Martin Silva1, Alain Manrique4, Boris Bienvenu5 and Achille Aouba1, 1Department of Internal Medicine, Caen University Hospital, Caen, France, 2Departement of Internal Medicine, Limoges University Hospital, Limoges, France, 3Department of Internal Medicine, Lille University Hospital, Lille, France, 4Department of Nuclear Medicine, Caen University Hospital, Caen, France, 5Caen University Hospital, Caen, France

    Background/Purpose:  Positron emission tomography combined with computed tomography (PET/CT) with 18F-fluorodeoxyglucose (FDG) can be used to assess large-vessel inflammation in giant-cell arteritis (GCA) and has demonstrated…
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