Abstracts tagged "Systemic sclerosis"

Abstract Number: 0410 • ACR Convergence 2021
Long-term Outcomes of Vascular Grafting of Medium Sized Vessels of the Wrist and Hand in Patients with Medication Refractory Systemic Sclerosis Related Raynaud’s Phenomenon at the University of Pennsylvania from 2009-2019
Nora Sandorfi¹, Chris Derk² and Nora Hajnoczky³, ¹University of Pennsylvania, Philadelphia, ²University of Pennsylvania, Philadelphia, PA, ³Albert Einstein Healthcare Network, Wallingford, PA
Background/Purpose: Raynaud’s phenomenon (RP) seen in patients with an underlying autoimmune rheumatic disorder have both functional and structural dysfunction at the microvascular level and in…
Abstract Number: 0559 • ACR Convergence 2021
Targeting NNMT as a Novel Metabolic Approach to Treat Systemic Sclerosis
M.Asif Amin¹, Bo Shi², Pei-Suen Tsou¹, Swarna Bale¹, Phillip Campbell¹, Mikel Gurrea-Rubio¹, Mark Eckert³, Ernst Lengyel³, Johann Gudjonsson⁴ and Varga John¹, ¹University of Michigan, Ann Arbor, MI, ²Nothwestern university, Chicago, IL, ³University of chicago, Chicago, IL, ⁴Department of Dermatology, University of Michigan, Ann Arbor, MI
Background/Purpose: Fibrosis, the hallmark of systemic sclerosis (SSc), is associated with metabolic and epigenetic alterations that are incompletely characterized. Our laboratory had previously implicated dysregulated…
Abstract Number: 1365 • ACR Convergence 2021
Release of High-Mobility Group Box-1 After an Raynaud’s Attack Potentially Leads to Fibroblast Activation and Interferon-γ Induced Protein-10 Production in Systemic Sclerosis
Isabella Atzeni¹, Yehya Al-Adwi², Berber Doornbos-van der Meer², Amel Eman Abdulle², Anniek Van Roon², Alja J. Stel², Harry Van Goor², Andries Smit², Johanna Westra² and Douwe J Mulder², ¹University of Groningen, University Medical Centre Groningen, Groningen, Groningen, Netherlands, ²University of Groningen, University Medical Centre Groningen, Groningen, Netherlands
Background/Purpose: Raynaud’s Phenomenon (RP) leading to repetitive ischaemia and reperfusion (IR) stress, is the first recognisable sign of systemic sclerosis (SSc). Although RP has been…
Abstract Number: 1434 • ACR Convergence 2021
Multiomic Study of Skin, Peripheral Blood and Serum: Is Serum Proteome a Reflection of Disease Process at the End-Organ Level in Systemic Sclerosis?
Victor Farutin¹, Elma Kurtagic², Joel Pradines³, Ishan Capila³, Maureen Mayes⁴, Minghua Wu⁵, Anthony Manning³ and Shervin Assassi⁶, ¹Janssen R&D, LLC, Watertown, MA, ²Janssen R&D, LLC, Cambridge, ³Momenta Pharmaceuticals, Inc., Cambridge, MA, ⁴Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁵University of Texas Health Science Center at Houston, Houston, TX, ⁶University of Texas McGovern Medical School at Houston, Houston, TX
Background/Purpose: Discovery of biomarkers in systemic sclerosis (SSc) represents an unmet clinical need. Samples from prominently affected fibrotic end-organs such as lung and skin are…
Abstract Number: 1846 • ACR Convergence 2021
Effect of Nintedanib in Patients with Systemic Sclerosis-associated Interstitial Lung Disease and Risk Factors for Rapid Decline in Forced Vital Capacity: Further Analyses of the SENSCIS Trial
Dinesh Khanna¹, Toby M Maher², Elizabeth Volkmann³, Yannick Allanore⁴, Vanessa Smith⁵, Shervin Assassi⁶, Michael Kreuter⁷, Anna-Maria Hoffmann-Vold⁸, Masataka Kuwana⁹, Christian Stock¹⁰, Margarida Alves¹¹, Steven Sambevski¹¹ and Christopher Denton¹², ¹University of Michigan, Ann Arbor, MI, ²National Heart and Lung Institute, Imperial College London, UK, National Institute for Health Research Clinical Research Facility, Royal Brompton Hospital, London, UK, and Keck School of Medicine, University of Southern California, Los Angeles, CA, ³Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, ⁴Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, ⁵Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, ⁶University of Texas McGovern Medical School at Houston, Houston, TX, ⁷Center for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Care Medicine, Thoraxklinik, University of Heidelberg, Member of the German Center for Lung Research, Heidelberg, Germany, ⁸Department of Rheumatology, Oslo University Hospital, Oslo, Norway, ⁹Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ¹⁰Boehringer Ingelheim Pharma GmbH & Co. KG, Biberach, Germany, ¹¹Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany, ¹²University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom
Background/Purpose: In the SENSCIS trial conducted in a population of subjects with systemic sclerosis-associated interstitial lung disease (SSc-ILD), with a mean time since onset of…
Abstract Number: 0391 • ACR Convergence 2021
High Frequency Ultrasound Imaging of Skin Thickness in Patients with Early Diffuse Cutaneous Systemic Sclerosis: An Objective Outcome Measure to Track Change over Time
Elizabeth Marjanovic¹, Calvin Heal¹, Tonia Moore¹, Joanne Manning², Sarah Wilkinson¹, Graham Dinsdale², Mark Dickinson¹, Jack Wilkinson¹, Ariane Herrick³ and Andrea Murray¹, ¹University of Manchester, Manchester, United Kingdom, ²Salford Royal NHS Foundation Trust, Manchester, United Kingdom, ³University of Manchester, Salford, United Kingdom
Background/Purpose: There are currently no validated objective measures of systemic sclerosis (SSc)-related skin thickening and this hampers clinical trials of potential new treatments. The modified…
Abstract Number: 0496 • ACR Convergence 2021
Safety and Efficacy of Rituximab for Systemic Sclerosis: A Double-Blind, Parallel-Group Comparison, Investigators Initiated Confirmatory Randomized Clinical Trial (DESIRES Study)
Satoshi Ebata¹, Ayumi Yoshizaki¹, Koji Oba², Kosuke Kashiwabara³, Keiko Ueda³, Yukari Umemura⁴, Takeyuki Watadani⁵, Takemichi Fukasawa¹, Shunsuke Miura¹, Asako Yoshizaki-Ogawa¹, Yoshihide Asano¹, Naoko Okiyama⁶, Masanari Kodera⁷, Minoru Hasegawa⁸ and Shinichi Sato¹, ¹Department of Dermatology, The University of Tokyo, Graduate School of Medicine, Tokyo, Japan, ²Department of Biostatistics, School of Public Health, Graduate School of Medicine, and Interfaculty Initiative in Information Studies, The University of Tokyo, Tokyo, Japan, ³Clinical Research Support Center, The Tokyo University Hospital, Tokyo, Japan, ⁴Biostatistics Section, Department of Data Science, Center for Clinical Sciences, National Center for Global Health and Medicine, Tokyo, Japan, ⁵Department of Diagnostic Radiology, The University of Tokyo, Graduate School of Medicine, Tokyo, Japan., Tokyo, Japan, ⁶Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan., Ibaraki, Japan, ⁷Department of Dermatology, Japan Community Health Care Organization Chukyo Hospital, Aichi, Japan., Aichi, Japan, ⁸Department of Dermatology, Division of Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan., Fukui, Japan
Background/Purpose: Systemic sclerosis (SSc) is a systemic autoimmune disease belonging to collagen diseases, characterized by fibrosis of various organs including the skin and lungs, and…
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: 1366 • ACR Convergence 2021
The Use of Lipid-Lowering Agents in Systemic Sclerosis: Is There a Relationship with Prevalence of Digital Ulcers and Overall Survival?
Olivia Sallis¹, Dylan Hansen², Kathleen Morrisroe³, Wendy Stevens³, Mandana Nikpour⁴ and Susanna Proudman⁵, ¹Royal Adelaide Hospital, Adelaide, Australia, ²St. Vincent’s Hospital Melbourne, Victoria, Australia, Adelaide, Australia, ³St Vincent's Hospital, Melbourne, Australia, ⁴University of Melbourne at St Vincent's Hospital, Melbourne, Australia, ⁵University of Adelaide, Medindie, Australia
Background/Purpose: Digital ulcers (DU) are common in systemic sclerosis (SSc). Vasodilator therapies have limited benefit in prevention and promoting healing. Lipid-lowering agents (LLAs) such as…
Abstract Number: 1435 • ACR Convergence 2021
Identification of Distinct Fibroblast Populations in Systemic Sclerosis 3D Skin Tissues with Single Cell Omics
Noelle Kosarek¹, Heetaek Yang², Fred W. Kolling³, Tamar Abel⁴, Mengqi Huang⁵, Avi Smith⁶, Jonathan Garlick⁶, Patricia A. Pioli² and Michael L. Whitfield⁷, ¹Dartmouth Geisel School of Medicine, Hartford, VT, ²Geisel School of Medicine, Hanover, NH, ³Centers for Quantitative Biology, Hanover, ⁴Geisel School of Medicine, West Lebanon, NH, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶Tufts University School of Dental Medicine, Boston, MA, ⁷Geisel School of Medicine, Lebanon, NH
Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by skin and internal organ fibrosis, vascular abnormalities, and autoantibody formation. Single cell genomics studies…
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: 0393 • ACR Convergence 2021
Severity and Impact of Gastrointestinal Symptoms in Patients with SSc-ILD Treated with Nintedanib: Data from SENSCIS-ON
Dinesh Khanna¹, Elizabeth Volkmann², Kristin B Highland³, Yannick Allanore⁴, Stéphane Jouneau⁵, James R Seibold⁶, Alexandra James⁷, Margarida Alves⁸ and Oliver Distler⁹, ¹University of Michigan, Ann Arbor, MI, ²Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, ³Cleveland Clinic, Cleveland, OH, ⁴Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, ⁵Department of Respiratory Medicine, Competences Centre for Rare Pulmonary Diseases, Univ Rennes, CHU Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) - UMR_S 1085, F-35000 Rennes, France, Rennes, France, ⁶Scleroderma Research Consultants LLC, Aiken, SC, ⁷Elderbrook solutions GmbH, Bietigheim-Bissingen, Germany, Bietigheim-Bissingen, Germany, ⁸Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany, ⁹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland
Background/Purpose: Gastrointestinal (GI) involvement is a common manifestation of systemic sclerosis (SSc) and a frequent side-effect of drugs used to treat SSc. In the SENSCIS…
Abstract Number: 0498 • ACR Convergence 2021
Background Mycophenolate (MMF) Treatment Is Associated with Improved Outcomes in a Phase 3 Trial of Lenabasum in Diffuse Cutaneous Systemic Sclerosis (dcSSc)
Robert Spiera¹, Dan Furst², Tracy Frech³, Masataka Kuwana⁴, Laura Hummers⁵, Wendy Stevens⁶, Suzanne Kafaja⁷, Eun bong Lee⁸, Scott Constantine⁹, Nancy Dgetluck⁹, Barbara White¹⁰ and Christopher Denton¹¹, ¹Hospital for Special Surgery, New York, NY, ²University of California Los Angeles, Los Angeles, CA, ³University of Utah, Salt Lake City, UT, ⁴Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ⁵Johns Hopkins Univerisity, Baltimore, MD, ⁶St Vincent's Hospital, Melbourne, Australia, ⁷University of California Los Angeles Division of Rheumatology, Los Angeles, CA, ⁸Seoul National University Hospital, Seoul, Republic of Korea, ⁹Corbus Pharmaceuticals, Inc., Norwood, MA, ¹⁰Corbus Pharmaceuticals, Norwood, MA, ¹¹University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom
Background/Purpose: Treatment of dcSSc is evolving, with limited information on relative efficacies of different immunosuppressive therapies (IST). Our hypothesis was that patients on MMF have…
Abstract Number: 0764 • ACR Convergence 2021
Patients with Juvenile Systemic Sclerosis Have a Distinct Pattern of Organ Involvement: Results from the Juvenile Systemic Sclerosis Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Maria Terreri⁶, Ana Paula Sakamoto⁷, Flavio Sztajnbok⁸, Brian Feldman⁹, Valda Stanevicha¹⁰, Jordi Anton¹¹, Raju Khubchandani¹², Ekaterina Alexeeva¹³, Sindhu Johnson¹⁴, Maria Martha Katsicas¹⁵, Sujata Sawhney¹⁶, Vanessa Smith¹⁷, Simone Appenzeller¹⁸, Tadej Avcin¹⁹, Mikhail Kostik²⁰, Thomas Lehman²¹, Edoardo Marrani²², Dieneke Schonenberg-Meinema²³, Walter Alberto Sifuentes-Giraldo²⁴, Natalia Vasquez-Canizares²⁵, Mahesh Janarthanan²⁶, Hana Malcova²⁷, Monika Moll²⁸, Dana Nemcova²⁹, Anjali Patwardhan³⁰, Maria José Santos³¹, cristina battagliotti³², Lillemor Berntson³³, Blanca Elena Rios Gomes Bica³⁴, Jürgen Brunner³⁵, Rolando Cimaz³⁶, Patricia Costa Reis³⁷, Despina Eleftheriou³⁸, Liora Harel³⁹, Gerd Horneff⁴⁰, Daniela Kaiser⁴¹, Tilmann Kallinich⁴², Dragana Lazarevic⁴³, Kirsten Minden², Susan Nielsen⁴⁴, Farzana Nuruzzaman⁴⁵, Siri Opsahl Hetlevik⁴⁶, Yosef Uziel⁴⁷ and Nicola Helmus⁴⁸, ¹Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey, ⁴Cerrahpasa Medical School, Istanbul, Turkey, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶UNIFESP, São Paulo, Brazil, ⁷Federal University of So Paulo (UNIFESP), São Paulo, Brazil, ⁸UFRJ/UERJ, Rio de Janeiro, Brazil, ⁹The Hospital for Sick Children, Toronto, ON, Canada, ¹⁰Paediatric Rheumatology International Trials Organisation (PRINTO), Riga, Latvia, ¹¹Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain, ¹²Jaslok Hospital and Research Center, Mumbai, India, ¹³Scientific Center of Children Health of RAMS, Moscow, Russia, ¹⁴University of Toronto, Toronto, ON, Canada, ¹⁵Hospital de Pediatria J.P Garrahan, Buenos Aires, Argentina, ¹⁶Pediatric Rheumatology Department, Institute of Child Health, Sir Gangarm Hospital, New Delhi, India, ¹⁷Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, ¹⁸Hospital das Clínicas da Universidade Estadual de Campinas, Campinas, Brazil, ¹⁹University Medical Center Ljubljana, Ljubljana, Slovenia, ²⁰Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²¹Hospital for Special Surgery, New York, NY, ²²University of Florence, Florence, Italy, ²³Emma Children’s Hospital, Amsterdam, Netherlands, ²⁴Hospital Universitario Ramón y Cajal, Madrid, Spain, ²⁵Children’s Hospital at Montefiore, Bronx, NY, ²⁶Sri Ramachandra University, Chennai, India, ²⁷Motol University Hospital, Prague, Czech Republic, ²⁸University Tuebingen, Tübingen, Germany, ²⁹Charles University, Prague, Czech Republic, ³⁰University of Missouri-Columbia, Columbia, MO, ³¹Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal, ³²Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ³³Uppsala University, Uppsala, Sweden, ³⁴Faculdade de Medicina, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ³⁵Medical University Innsbruck, Innsbruck, Austria, ³⁶ASST Gaetano Pini-CTO, Università degli Studi di Milano, Milan, Italy, ³⁷Hospital de Santa Maria, Lisbon, Portugal, ³⁸Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³⁹Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel, ⁴⁰Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁴¹Luzerner Kantonsspital, Kinderspital, Luzern, Switzerland, ⁴²Charité University Medicine, Nuremberg, Germany, ⁴³Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, ⁴⁴Rigshospitalet, Copenhagen, Denmark, ⁴⁵Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁶Oslo University Hospital, Oslo, Norway, ⁴⁷Meir Medical Center, Kfar Saba, Israel, ⁴⁸Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of around 3 in 1,000,000 children. To better capture the clinical manifestations of…
Abstract Number: 1367 • ACR Convergence 2021
Associations of Esophageal Dysmotility Patterns with Extra-intestinal Features in Patients with Systemic Sclerosis
Ana Tucker¹, Jamie Perin², Elizabeth Volkmann³, Ami Shah⁴, John Pandolfino⁵ and Zsuzsanna McMahan⁴, ¹Medical University of South Carolina, Charleston, SC, ²Johns Hopkins University, Baltimore, MD, ³Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, ⁴Johns Hopkins Rheumatology, Baltimore, MD, ⁵Northwestern University, Chicago, IL
Background/Purpose: The gastrointestinal (GI) tract is the most commonly affected internal organ in systemic sclerosis (SSc). SSc GI disease is heterogeneous, with some patients experiencing…

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