Abstracts tagged "Systemic sclerosis"

Abstract Number: 0953 • ACR Convergence 2023
Flavin-containing Monooxygenase (FMO3) Links the Gut and Fibrosis in Systemic Sclerosis
Priyanka Verma¹, Bharath Yalavarthi¹, Karen Ho², Lutfiyya Muhammad³, Seokjo Kim⁴, Johann Gudjonsson¹, Rebecca C Schugar⁵, Mark Brown⁶, Xinmin Li⁶, Stanley L Hazen⁷, Swati Bhattacharyya¹ and John Varga¹, ¹University of Michigan, Ann Arbor, MI, ²Northwestern University, Chicago, IL, ³Northwestern University Feinberg School of Medicine, Chicago, IL, ⁴4SCM Lifescience Co. Ltd, South Korea, Japan, ⁵Stanford University, Stanford, CA, ⁶Department of Cardiovascular & Metabolic Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, ⁷Division of Endocrinology, Department of Medicine, Stanford University, Stanford, CA
Background/Purpose: Trimethylamine (TMA) generated by the gut microbiome is converted into trimethylamine N-oxide (TMAO) via the host enzyme FMO3. The TMA-FMO3-TMAO metaorganismal axis has been…
Abstract Number: 1520 • ACR Convergence 2023
Identifying Core Domains for Clinical Trials in Systemic Sclerosis-Associated Raynaud’s Phenomenon and Digital Ulcers Using the Delphi Consensus Method
Susanna Proudman¹, Michael Hughes², Nancy Maltez³, Edith Brown⁴, Virginia Hickey⁵, shawna grosskleg⁶, B Shea⁶, Ariane Herrick⁷, John Pauling⁸ and Peter Merkel⁹, ¹Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia, ²Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, United Kingdom, ³Department of Medicine, Division of Rheumatology, The Ottawa Hospital, Ottawa, ON, Canada, ⁴OMERACT, Manchester, United Kingdom, ⁵OMERACT, Adelaide, Australia, ⁶OMERACT, Ottawa, ON, Canada, ⁷University of Manchester, Salford, United Kingdom, ⁸North Bristol NHS Trust, Bristol, United Kingdom, ⁹University of Pennsylvania, Philadelphia, PA
Background/Purpose: The OMERACT Scleroderma Vascular Disease Working Group sought to identify essential core outcome domains for inclusion in clinical trials focusing on Raynaud's phenomenon (RP)…
Abstract Number: 1701 • ACR Convergence 2023
Treatment Regimens and Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in Light of the 2022 ESC/ERS Guidelines
Hilde Jenssen Bjørkekjær¹, Cosimo Bruni², Cathrine Brunborg³, Patricia Carreira⁴, Paolo Airò⁵, Carmen Pilar Simeon-Aznar⁶, Marie-Elise Truchetet⁷, Alessandro Giollo⁸, Alexandra Balbir-Gurman⁹, Mickael Martin¹⁰, Christopher Denton¹¹, Armando Gabrielli¹², Havard Fretheim¹³, Imon Barua¹³, Helle Bitter¹⁴, Oyvind Midtvedt¹³, Torhild Garen¹⁵, Kaspar Broch¹⁶, Arne Andreassen¹⁷, Yoshiya Tanaka¹⁸, Gabriela Riemekasten¹⁹, Ulf Müller-Ladner²⁰, marco Matucci Cerinic²¹, Iván Castellvi²², Elise Siegert²³, Eric Hachulla²⁴, Oliver Distler² and Anna-Maria Hoffmann-Vold¹³, ¹Department of Rheumatology, Hospital of Southern Norway, Kristiansand & University of Oslo, Institute of Clinical Medicine, Oslo, Norway, ²Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital - Rikshospitalet, Oslo, Norway, ⁴Hospital Universitario 12 de Octubre, Madrid, Spain, ⁵Spedali Civili di Brescia, Scleroderma UNIT, UOC Reumatologia ed Immunologia Clinica, Piazzale Spedali Civili 1, 25123, Brescia, Italy, ⁶Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario Vall d'Hebronh, Barcelona, Spain, ⁷Bordeaux University Hospital, Bordeaux, France, ⁸University of Verona, Rheumatology Section, Department of Medicine, Verona, Italy, Verona, Italy, ⁹Rheumatology Institute, Rambam Health Care Campus and Rappaport Faculty of |Medicine, Technion, Haifa, Israel, ¹⁰Department of Internal Medicine, INSERM U1313, Poitiers University, Poitiers University Hospital, Poitiers, France, ¹¹University College London, London, United Kingdom, ¹²Ospedali Riuniti Marche, Ancona, Italy, ¹³Oslo University Hospital, Oslo, Norway, ¹⁴Sorlandet sykehus, Kristiansand, Norway, ¹⁵Dept of Rheumatology, University Hospital Oslo, Oslo, Norway, ¹⁶Oslo University Hospital, Rikshospitalet, Department of Cardiology, Oslo, Norway, KG Jebsen center, Institute for Experimental Medical Research, University of Oslo, Oslo, Norway, ¹⁷Oslo University Hospital, Rikshospitalet, Department of Cardiology, Oslo, Norway, ¹⁸University of Occupational and Environmental Health, Kitakyushu, Japan, ¹⁹University Clinic Schleswit-Holstein (UKSH), Lübeck, Germany, ²⁰Justus Liebig University Gießen, Campus Kerckhoff, Bad Nauheim, Germany, ²¹Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy, ²²Hospital de Santa Creu i Sant Pau, Barcelona, Spain, ²³Department of Rheumatology, Charité University Hospital, Charité Platz 1, D-10117, Berlin, Germany, ²⁴University of Lille, Lille, France
Background/Purpose: The 2022 ESC/ERS Guidelines recommend upfront combination therapy for low- and intermediate-risk, and triple therapy for high-risk patients with systemic sclerosis (SSc)-associated pulmonary arterial…
Abstract Number: 2365 • ACR Convergence 2023
Decoding the Peripheral Immune Landscape of Systemic Sclerosis to Investigate Disease Stages and Interstitial Lung Disease Progression
Vasuki Ranjani Chellamuthu¹, Maria Noviani², Ahmad bin Mohamed Lajam¹, Andrea Hsiu Ling Low² and Salvatore Albani¹, ¹Translational Immunology Institute, SingHealth, Singapore, Singapore, ²Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore
Background/Purpose: Systemic sclerosis (SSc) is a heterogenous autoimmune disease characterized by fibrosis, vascular abnormalities and immune dysregulation. Characterization of peripheral blood immune signatures in relation…
Abstract Number: 074 • 2023 Pediatric Rheumatology Symposium
Clinical Characteristics of Juvenile Systemic Sclerosis in Korea: A 30-year Single Center Study
Jieun Jeong¹, Minji Kim¹, Jiwon Jung², Seon Hee Lim³ and Seong Heon Kim¹, ¹Department of Pediatrics, Seoul National University Children’s Hospital, Seoul, Republic of Korea, ²Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Republic of Korea, ³Department of Pediatrics, Pusan National University Children’s Hospital, Yangsan, Republic of Korea
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare autoimmune, chronic, multisystem, connective tissue disease characterized by progressive tissue fibrosis of the skin and internal organs.…
Abstract Number: 086 • 2023 Pediatric Rheumatology Symposium
Systemic Sclerosis Overlap Syndrome: A Case Series from a Single Large Pediatric Center
Jessica Nguyen¹, Miriah Gillispie-Taylor¹, Eyal Muscal¹ and Marietta Deguzman², ¹Baylor College of Medicine, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare but potentially life-threatening autoimmune condition with features including immune, fibrotic, and vascular manifestations affecting the skin and…
Abstract Number: 026 • 2023 Pediatric Rheumatology Symposium
Performance of the UCLA Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 Instrument in a Juvenile Systemic Sclerosis Cohort
Sophie Stefancic, Amanda Robinson, Haley Havrilla, Samantha Branton, Vibha Sood and Kathryn Torok, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Gastrointestinal (GI) manifestations in juvenile onset systemic sclerosis (jSSc) reflect adult disease with a range of involvement along the GI tract, including oropharyngeal dysphagia…
Abstract Number: 1051 • ACR Convergence 2022
A Deep Neural Network Classifier to Identify Intrinsic Molecular Subsets of Systemic Sclerosis Using Histological Images
Yiwei Yuan¹, Qingyuan Song¹, Paul Harms², Johann Gudjonsson², Robert Lafyatis³, Dinesh Khanna⁴ and Michael Whitfield⁵, ¹Dartmouth College, Lebanon, NH, ²University of Michigan, Ann Arbor, MI, ³Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ⁴Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ⁵Geisel School of Medicine, Lebanon, NH
Background/Purpose: Intrinsic gene analyses based on the gene expression profiles in skin have identified four intrinsic molecular subsets among patients with systemic sclerosis (SSc); these…
Abstract Number: 1180 • ACR Convergence 2022
Deficiency and Altered Phenotype of Mucosal-associated Invariant T Cells in Systemic Sclerosis
Manon Lesturgie-Talarek¹, Virginie Gonzalez¹, Camelia Frantz², Noémie Sénot¹, Zouriatou Gouda¹, Camille Rousseau¹, Lucie Beaudoin¹, Agnès Lehuen¹, Jérôme Avouac³ and Yannick Allanore⁴, ¹INSERM U1016, Institut Cochin, Paris, France, ²Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ³University of Paris, Paris, France, ⁴Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs such as the lung. Mucosal-associated invariant T (MAIT)…
Abstract Number: 1527 • ACR Convergence 2022
Maintained Complete B Cell Depletion Is Associated with Rituximab Efficacy in Connective Tissue Disorder Interstitial Lung Disease
Jérôme Avouac¹, Roba Ghossan², Omar Al Tabaa³, Alice Combier², Alexia Steelandt², Marion Thomas⁴ and Yannick Allanore⁵, ¹University of Paris, Paris, France, ²Hôpital Cochin, AP-HP.Centre - Université Paris Cité, Paris, France, ³Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ⁴Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ⁵Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: The benefit of RTX has been suggested for interstitial lung disease (ILD) associated with connective tissue disorders (CTD). Preliminary data have suggested that B…
Abstract Number: 1885 • ACR Convergence 2022
Histopathological Patterns of Muscle Involvement and Clinical Correlations in a Retrospective Cohort of Scleroderma Patients
Noemie Le Gouellec¹, Louai Zaidan², Eric Hachulla³, Nicolas Dognon³, Robin Dhote⁴, Sebastien Riviere⁵, Yurdagul Uzunhan⁴, Luc Mouthon⁶ and Jerome Authier⁷, ¹CH Valenciennes, Valenciennes, France, ²CHU Henri Mondor, Creteil, France, ³University of Lille, LILLE, France, ⁴Hopital Avicenne - Paris University, Bobigny, France, ⁵Hopital Saint Antoine - Paris University, Paris, France, ⁶Hopital Cochin - Paris University, Paris, France, ⁷Paris Est - Creteil University, Creteil, France
Background/Purpose: Muscle involvement in systemic sclerosis (SSc) has been shown to be heterogeneous. Histopathological pattern seems to be associated with prognosis, but studies evaluating the…
Abstract Number: 0154 • ACR Convergence 2022
Anti-SMN Autoantibodies in Mixed Connective Tissue Disease Are Associated with a Scleromyositis Phenotype
Hajar El Kamouni¹, Alexandra Albert², Sabrina Hoa³, Darya S. Jalaledin⁴, Josiane Bourre-Tessier⁵, Eric Rich⁵, Jean-Richard Goulet⁶, Martial Koenig⁷, Minoru Satoh⁸, Marvin Fritzler⁹, May Choi¹⁰, Yves Troyanov¹¹, Jean-Luc Senécal⁵ and Océane Landon-Cardinal⁵, ¹Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Laval, Canada, ²Division of Rheumatology, Centre Hospitalier de l'Université Laval, Québec, QC, Canada, ³University of Montreal, Montréal, QC, Canada, ⁴Division of Internal Medicine, Centre Hospitalier de l'Université de Sherbrooke, Saint-Lambert, Canada, ⁵Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada, ⁶Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Saint-Lambert, Canada, ⁷Division of Internal Medicine, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada, ⁸Department of Human, Information and Science, University of Occupational Environmental Health, Kitakyusyu Fukuoka, Japan, ⁹University of Calgary, Calgary, AB, Canada, ¹⁰Brigham and Women's Hospital | University of Calgary, Calgary, AB, Canada, ¹¹Division of Rheumatology, Hôpital du Sacré-Coeur de Montréal, Montréal, QC, Canada
Background/Purpose: The survival of motor neuron (SMN) is a ubiquitous macromolecular complex, located in the Cajal body of mammalian nuclei, with an essential role in…
Abstract Number: 1053 • ACR Convergence 2022
The Collaborative National Quality and Efficacy Registry for Scleroderma: Association of Resource Utilization and Gastrointestinal Tract Symptoms in Early Disease
Sarah Luebker¹, Tracy Frech¹, Shervin Assassi², Jessica Gordon³, Elana Bernstein⁴, Virginia Steen⁵, Laura Hummers⁶, Ami Shah⁷, Carrie Richardson⁸, Dinesh Khanna⁹, Flavia Castelino¹⁰, Lorinda Chung¹¹, Faye Hant¹², Vicki Shanmugam¹³, John VanBuren¹⁴, Jessica Alvey¹⁴, Monica Harding¹⁴, Luke Evnin¹⁵ and Nora Sandorfi¹⁶, ¹Vanderbilt University Medical Center, Nashville, TN, ²McGovern Medical School, University of Texas, Houston, TX, ³Hospital for Special Surgery, New York, NY, ⁴Columbia University, New York, NY, ⁵Georgetown University School of Medicine, Washington, DC, ⁶Johns Hopkins Univerisity, Baltimore, MD, ⁷Johns Hopkins Rheumatology, Baltimore, MD, ⁸Northwestern University, Chicago, IL, ⁹Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁰Massachusetts General Hospital, Boston, MA, ¹¹Stanford University, Stanford, CA, ¹²Medical University of South Carolina, Charleston, SC, ¹³George Washington University, Washington, DC, ¹⁴University of Utah, Salt Lake City, UT, ¹⁵Scleroderma Research Foundation, Brisbane, CA, ¹⁶University of Pennsylvania, Philadelphia, PA
Background/Purpose: Health costs and resource utilization are important in chronic disease management. Systemic sclerosis (SSc) is a multi-organ system disease with outcomes that are often…
Abstract Number: 1181 • ACR Convergence 2022
Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis
Feiyang Ma¹, Pei-Suen Tsou¹, Grace Hile¹, John Varga¹, J. Michelle Kahlenberg¹, Allison Billi¹, Johann Gudjonsson¹ and Dinesh Khanna², ¹University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…
Abstract Number: 1528 • ACR Convergence 2022
Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) and Organ Damage: Data from the SENSCIS Trial
Janet Pope¹, Susanna Proudman², Wendy Stevens³, Joerg Henes⁴, Rozeta Simonovska⁵, Margarida Alves⁶ and Yannick Allanore⁷, ¹University of Western Ontario, London, ON, Canada, ²Rheumatology Unit, Royal Adelaide Hospital, and Discipline of Medicine, University of Adelaide, Adelaide, Australia, ³Department of Rheumatology, St Vincent’s Hospital, Melbourne, Australia, Melbourne, Australia, ⁴Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ⁵mainanalytics GmbH, Sulzbach (Taunus), Germany, Sulzbach (Taunus), Germany, ⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁷Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: The Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI) was developed to quantify organ damage in patients with SSc. We assessed outcomes in the SENSCIS…

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