Abstracts tagged "Scleroderma"

Abstract Number: 0697 • ACR Convergence 2021
Detection of Autoantibodies Against Muscle-Specific Four-and-a-Half-LIM Domain 1 (FHL1) in Inflammatory Myopathies: Results from a Single-Center Cohort
Angeles Shunashy Galindo-Feria¹, Begum Horuluoglu¹, Jessica Day², Catia Cerqueira³, Edvard Wigren⁴, Susanne Gräslund⁴, Susanna Proudman⁵, Ingrid E Lundberg⁴ and Vidya Limaye⁶, ¹Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden, ²Rheumatology Unit, Royal Adelaide Hospital, Adelaide, Australia and Discipline of Medicine, University of Adelaide, Adelaide, Australia, ³4Dcell, Montreuil, France, ⁴Karolinska Institutet, Karolinska University Hospital, Division of Rheumatology, Department of Medicine Solna, Stockholm, Sweden, Stockholm, Sweden, ⁵University of Adelaide, Medindie, Australia, ⁶Royal Adelaide Hospital, Adelaide, Australia
Background/Purpose: Autoantibodies targeting a muscle-specific autoantigen, four-and-a-half-LIM-domain 1 (FHL1), have been previously identified in patients with idiopathic inflammatory myopathies (IIM) (1). The aim of this…
Abstract Number: 1848 • ACR Convergence 2021
Untangling the Gut: A Phenome-Wide Association Study of Drugs and Diseases with Gastrointestinal Dysfunction in Systemic Sclerosis
Rory Maclean¹, Fiza Ahmed², Voon Ong³, Charles Murray⁴ and Christopher Denton⁵, ¹Royal Free Hospital & University College London, London, United Kingdom, ²Royal Free Hospital, Ilford, United Kingdom, ³University College London Medical School Royal Free Campus, London, United Kingdom, ⁴Royal Free Hospital, London, United Kingdom, ⁵University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom
Background/Purpose: Gastrointestinal dysfunction (SSc-GI) is a significant burden to patients with systemic sclerosis (SSc), particularly in those with longstanding disease. The management of SSc-GI is…
Abstract Number: 0716 • ACR Convergence 2021
Marked Capillary Basement Membrane Reduplication Is the Hallmark Histopathological Feature of Scleromyositis
Benjamin Ellezam¹, Yves Troyanov², Valérie Leclair³, Imane Bersali⁴, Margherita Giannini⁴, Sabrina Hoa⁵, Josiane Bourré-Tessier⁵, Minoru Satoh⁶, Marvin Fritzler⁷, Béatrice Lannes⁸, Jean-Luc Senécal⁵, Marie Hudson⁹, Alain Meyer⁴ and Océane Landon-Cardinal⁵, ¹Division of Pathology, CHU Sainte-Justine; Department of Pathology and Cell Biology, Université de Montréal, Montréal, QC, Canada, ²Division of Rheumatology, Hôpital du Sacré-Coeur; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ³Division of Rheumatology, Jewish General Hospital; Department of Medicine, McGill University, Montréal, QC, Canada, ⁴Service de physiologie- explorations fonctionnelles musculaire, service de rhumatologie et Centre de références des maladies autoimmunes rares, Hôpitaux universitaires de Strasbourg., Strasbourg, France, ⁵Division of Rheumatology, Centre hospitalier de l'Université de Montréal; Department of Medicine, Université de Montréal, Montréal, QC, Canada, ⁶Department of Clinical Nursing, University of Occupational and Environmental Health, Kitakyushu, Japan, ⁷University of Calgary, Calgary, AB, Canada, ⁸Service de Pathologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, ⁹McGill University, Montréal, QC, Canada
Background/Purpose: Scleromyositis (SM) is an emerging subset of autoimmune myositis (AIM) in patients with features of systemic sclerosis (SSc). SM patients may present without characteristic…
Abstract Number: 1849 • ACR Convergence 2021
False Positive Anti-Topoisomerase I (Scl-70) Antibody Results: A Case Series from a Scleroderma Referral Center
Brian Lam¹, Rana Taherian¹, Julio Charles¹, Maureen Mayes², Shervin Assassi³ and Brian Skaug⁴, ¹Division of Rheumatology, University of Texas McGovern Medical School at Houston, Houston, TX, ²Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴University of Texas McGovern Medical School Houston, Houston, TX
Background/Purpose: Systemic sclerosis (SSc) is a progressive autoimmune disease with high morbidity and mortality, making early diagnosis and management critical. Anti-Topoisomerase I antibody (anti-Topo I,…
Abstract Number: L09 • ACR Convergence 2020
A Phase 2a Randomized, Double-blind, Placebo-controlled Study of Ziritaxestat in Early Diffuse Cutaneous Systemic Sclerosis (NOVESA)
Dinesh Khanna¹, Christopher Denton², Daniel Furst³, Maureen Mayes⁴, Marco Matucci-Cerinic⁵, Vanessa Smith⁶, Dick de Vries⁷, Liesbeth Deberdt⁸, Pieter‑Jan Stiers⁸, Niyati Prasad⁸ and Sohail Ahmed⁹, ¹University of Michigan, Ann Arbor, MI, ²Royal Free Hospital, University College London, London, United Kingdom, ³David Geffen School of Medicine at UCLA, Los Angeles, CA, ⁴University of Texas Health Science Center at Houston, Houston, TX, ⁵Università degli Studi di Firenze, Firenze, Italy, ⁶Ghent University Hospital, Ghent, Belgium, ⁷Galapagos BV, Leiden, Netherlands, ⁸Galapagos NV, Mechelen, Belgium, ⁹Ahmed Science Medicine, Basel, Switzerland
Background/Purpose: There is a high unmet need for systemic sclerosis (SSc) treatments. Ziritaxestat (ziri; GLPG1690) is an autotaxin inhibitor with a novel mechanism of action.…
Abstract Number: 0384 • ACR Convergence 2020
Serum Neutrophil Count Predicts Progression of Interstitial Lung Disease and Mortality in Patients with Systemic Sclerosis Related Interstitial Lung Disease
Nancy Wareing¹, Ning Li², Elizabeth Volkmann³, Marka Lyons⁴, Michael Roth², Donald Tashkin² and Shervin Assassi¹, ¹University of Texas Houston McGovern Medical School, Division of Rheumatology and Clinical Immunogenetics, Houston, TX, ²University of California, Los Angeles, Los Angeles, ³University of California, Los Angeles, Los Angeles, CA, ⁴University of Texas Houston, McGovern Medical School, Division of Rheumatology and Clinical Immunogenetics, Houston, TX
Background/Purpose: Systemic sclerosis (SSc) patients have a prominent neutrophil gene expression signature. However, investigations into the pathophysiologic role of neutrophils in SSc are lacking. This…
Abstract Number: 0916 • ACR Convergence 2020
Validating Autoantibody Associations and Clinical Impact of Severe Gastrointestinal Involvement in Systemic Sclerosis
Fiza Ahmed¹, Svetlana Nihtyanova¹, Stamatia Chatzinikolaou², Voon Ong¹, Charles Murray² and Christopher Denton³, ¹Centre for Rheumatology, Royal Free Campus, UCL Division of Medicine, UK, London, United Kingdom, ²Department of Gastroenterology, Royal Free London Foundation Trust, UK, London, United Kingdom, ³University College London, London, United Kingdom
Background/Purpose: Patients with systemic sclerosis (SSc) frequently experience gastrointestinal (GI) symptoms, ranging from mild to debilitating in severity. Better prediction of those most at risk…
Abstract Number: 1380 • ACR Convergence 2020
Digital Occlusive Arterial Disease on Laser Doppler Flowmetry Increases the Risk of Digital Ischemic Complications in Systemic Sclerosis: Results from a Single Center Referral Cohort (2001-2018)
Yasser Radwan¹, Tina Gunderson², Cynthia Crowson², David Liedl², Kenneth Warrington³, Paul Wennberg² and Ashima Makol², ¹Mayo Clinic, Rochester, Minnesota, USA, Lansing, MI, ²Mayo Clinic, Rochester, Minnesota, USA, Rochester, MN, ³Mayo Clinic, Rochester, MN
Background/Purpose: Vascular dysfunction is a key feature of systemic sclerosis (SSc), manifesting clinically as Raynaud’s phenomenon (RP) with or without digital ischemia. Laser doppler flowmetry…
Abstract Number: 2036 • ACR Convergence 2020
Damage Trajectories in Systemic Sclerosis Using Group-Based Trajectory Modeling
Ariane Barbacki¹, Murray Baron², Mianbo Wang³, Yuqing Zhang⁴, Mandana Nikpour⁵ and Ada Man⁶, ¹McGill University Health Center, Montreal, QC, Canada, ²Jewish General Hospital, Montreal, QC, Canada, ³Lady Davis institute for Medical Research, Montreal, QC, Canada, ⁴Massachusetts General Hospital, Harvard Medical School, Boston, ⁵The University of Melbourne at St. Vincent's Hospital, Melbourne, Victoria, Australia, ⁶University of Manitoba, Winnipeg, MB, Canada
Background/Purpose: Systemic sclerosis (SSc) is a rare systemic autoimmune disease associated with a high mortality and characterized by the accrual of organ damage over time.…
Abstract Number: 0385 • ACR Convergence 2020
A Pilot Study to Evaluate the Safety and Efficacy of Treprostinil in the Treatment of Calcinosis in Patients with Systemic Sclerosis
Melody Chung¹, Antonia Maria Valenzuela Vergara², Benjamin Catanese¹, Shufeng Li¹, Kate Stevens¹ and Lorinda Chung³, ¹Stanford University, Stanford, CA, ²Pontificia Universidad Catolica de Chile, Santiago de Chile, Chile, ³Stanford University School of Medicine and Palo Alto VA Health Care System, Palo Alto, CA
Background/Purpose: Calcinosis is characterized by calcium deposition in skin and subcutaneous tissues and progresses over one year in the majority of systemic sclerosis (SSc) patients. Since…
Abstract Number: 0922 • ACR Convergence 2020
Cancer in Systemic Sclerosis: Analysis of Antibodies Against Components of the Th/To Complex
Christopher Mecoli¹, Brittany Adler², Qingyuan Yang², Laura Hummers³, Antony Rosen², Livia Casciola-Rosen⁴ and Ami Shah⁵, ¹Johns Hopkins University School of Medicine, Baltimore, MD, ²Johns Hopkins University, Baltimore, MD, ³Johns Hopkins Univerisity, Ellicott City, MD, ⁴Johns Hopkins University, Johns Hopkins University, MD, ⁵Johns Hopkins University School of Medicine, Ellicott City, MD
Background/Purpose: The aim of this study is to describe four of the most common autoantibodies against components of the Th/To complex: hPOP1, RPP25, RPP30, and…
Abstract Number: 1383 • ACR Convergence 2020
Tocilizumab Shows Potential in Preserving Lung Function in Systemic Sclerosis with Positive anti-topoisomerase-1 (Scl-70): A Single Centre Cohort Study
Yasir Suleman¹, Kristina Clark², Svetlana Nihtyanova³, Voon Ong⁴ and Christopher Denton², ¹Royal Free Hospital London NHS Trust, London, United Kingdom, ²University College London, London, United Kingdom, ³Centre for Rheumatology, Royal Free Campus, UCL Division of Medicine, UK, London, United Kingdom, ⁴UCL, London, United Kingdom
Background/Purpose: Recent Phase II (faSScinate) and Phase III (focuSSced) clinical trials of tocilizumab versus placebo in early dcSSc highlighted the potential impact of tocilizumab on…
Abstract Number: 2039 • ACR Convergence 2020
Short Telomeres and Autoantibodies Targeting Telomere-Associated Proteins in Scleroderma
Britany Adler¹, Ami Shah², Francesco Boin³, Paul Wolters³, Livia Casciola-Rosen⁴ and Antony Rosen¹, ¹Johns Hopkins University, baltimore, MD, ²Johns Hopkins University School of Medicine, Ellicott City, MD, ³University of California, San Francisco, San Francisco, CA, ⁴Johns Hopkins University, Johns Hopkins University, MD
Background/Purpose: Scleroderma is a systemic fibrosing disease of unknown etiology that often manifests with interstitial lung disease (ILD). Prior studies have found an association between…
Abstract Number: 0386 • ACR Convergence 2020
Phase 1 Clinical Study of MGTA-145 in Combination with Plerixafor Shows Rapid Single-Day Mobilization and Collection of CD34+ Hematopoietic Stem Cells Without G-CSF
John DiPersio¹, Jonathan Hoggatt², Steven Devine³, David Scadden², Haley Howell⁴, Veit Schmelmer⁴, Glen Raffel⁴, Pat Falahee⁴, Kevin Goncalves⁴, William Savage⁴ and John Davis⁴, ¹Washington University, Saint Louis, ²Massachusetts General Hospital, Boston, ³National Marrow Donor Program, Minneapolis, ⁴Magenta Therapeutics, Cambridge
Background/Purpose: Autologous hematopoietic stem cell (HSC) transplantation is a recommended therapeutic option for selected patients with scleroderma and other autoimmune diseases. HSC transplant requires collection…
Abstract Number: 0926 • ACR Convergence 2020
Risk Factors for Fractures and Osteoporosis Among Patients with Systemic Sclerosis in a United States Cohort
Sina Famenini¹, Jamie Perin², Kristin Wipfler³, Kaleb Michaud⁴ and Zsuzsanna McMahan⁵, ¹Johns Hopkins School of Medicine, Baltimore, MD, ²JHUSPH, Baltimore, ³FORWARD, The National Databank for Rheumatic Diseases, Omaha, NE, ⁴University of Nebraska Medical Center, Omaha, NE, ⁵Johns Hopkins University, Baltimore
Background/Purpose:Systemic sclerosis (SSc) is associated with several risk factors for osteoporosis and fractures due to malabsorption or malnutrition, physical disability, chronic inflammation, and use of…

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