Abstracts tagged "Systemic sclerosis"

Abstract Number: 1621 • ACR Convergence 2022
Genome-wide Association Study in African American Systemic Sclerosis Patients Identifies a Novel Target – Transforming Growth Factor-β3 (TGFβ3)
Urvashi Kaundal¹, Julia Hartman², Emilee Stenson¹, Sarah Safran¹, Chloe Borden¹, Mousumi Sahu¹, Janet Wang¹, Andrea Conte¹, Ami Shah³, Maureen Mayes⁴, Ayo Doumatey⁵, Amy Bentley⁵, Daniel Shriner⁵, Robyn Domsic⁶, Thomas Medsger⁷, Paula Ramos⁸, Richard Silver⁸, Virginia Steen⁹, John Varga¹⁰, Vivien Hsu¹¹, Lesley Ann Saketkoo¹², Elena Schiopu¹³, Dinesh Khanna¹⁴, Jessica Gordon¹⁵, Lindsey Criswell¹⁶, Heather Gladue¹⁷, Chris Derk¹⁸, Elana Bernstein¹⁹, S. Louis Bridges, Jr.¹⁵, Victoria Shanmugam²⁰, Lorinda Chung²¹, Suzanne Kafaja²², Reem Jan²³, Marcin Trojanowski²⁴, Avram Goldberg²⁵, Benjamin Korman²⁶, Settara Chandrasekharappa⁵, Yongbing Zhao²⁷, Stephen Brooks²⁷, Stefania Dell'Orso¹, Adebowale Adeyemo⁵, Charles Rotimi⁵, Elaine Remmers⁵, Daniel Kastner⁵, Francesco Boin²⁸, Rafael Casellas²⁷, Fredrick Wigley²⁹ and Pravitt Gourh³⁰, ¹National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Washington, DC, ³Johns Hopkins Rheumatology, Baltimore, MD, ⁴Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁵National Human Genome Research Institute, Bethesda, MD, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁸Medical University of South Carolina, Charleston, SC, ⁹Georgetown University School of Medicine, Washington, DC, ¹⁰University of Michigan, Ann Arbor, MI, ¹¹Rutgers-RWJ Medical School, South Plainfield, NJ, ¹²University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, ¹³Michigan Medicine, Ann Arbor, MI, ¹⁴Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁵Hospital for Special Surgery, New York, NY, ¹⁶National Human Genome Research Institute, NIH, Bethesda, MD, ¹⁷Arthritis & Osteoporosis Consultants of the Carolinas, Charlotte, NC, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Columbia University, New York, NY, ²⁰George Washington University, Great Falls, VA, ²¹Stanford University, Stanford, CA, ²²UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ²³University of Chicago, Chicago, IL, ²⁴Boston University School of Medicine, Boston, MA, ²⁵NYU Langone Medical Center - NYU Hospital for Joint Diseases, Lake Success, NY, ²⁶University of Rochester, Rochester, NY, ²⁷National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ²⁸Cedars-Sinai Medical Center, Los Angeles, CA, ²⁹Johns Hopkins University, Baltimore, MD, ³⁰National Institutes of Health, Bethesda, MD
Background/Purpose: Transforming Growth Factor-β (TGFβ) isoforms play an important role in extracellular matrix biology. Increased TGFβ-regulated gene signature has been observed in lesional skin and…
Abstract Number: 2172 • ACR Convergence 2022
Pulmonary Function in Patients Diagnosed of Early Systemic Sclerosis: 10 Years-Experience
Samuel Leal Rodriguez¹, Francisco Miguel Ortiz Sanjuan², José Ivorra Cortés³, Laura Mas Sanchez³, Pablo Muñoz Martinez⁴, Carmen Riesco Bárcena⁵, Anderson Huaylla Quispe⁶, Cristobal Pavez Perales³, Inés Cánovas Olmos³, Luis Gonzalez Puig⁷, Elena Grau García³, Isabel Martínez-Cordellat³, Carmen Nájera Herranz³, Rosa Negueroles Albuixech³, JOSE ELOY OLLER RODRIGUEZ⁸, Elvira Vicens Bernabeu², Alba Torrat Novés⁵, Daniel Ramos Castro³ and José andrés Román ivorra¹, ¹Hospital Universitari i Politécnic La Fe, València, Spain, ²Hospital Universitario de La Fe, València, ³Rheumatology Department. Hospital Universitario y Politécnico La Fe, València, Spain, ⁴Rheumatology Department. Hospital Universitario y Politécnico La Fe, Sagunto, Spain, ⁵Rheumatology Department. Hospital Universitario y Politécnico La Fe, València, ⁶Medicina, València, Spain, ⁷Hospital La Fe, Torrente, Valencia, Spain, ⁸Hospital Universitario de La Fe, València, Spain
Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc), often progressive and has a poor prognosis. A restrictive ventilatory defect could…
Abstract Number: 0522 • ACR Convergence 2022
Cohort Enrichment Strategies for Progressive Interstitial Lung Disease in Systemic Sclerosis from EUSTAR
Anna-Maria Hoffmann-Vold¹, Cathrine Brunborg¹, Paolo Airò², Lidia P. Ananyeva³, László Czirják⁴, Serena Guiducci⁵, Eric Hachulla⁶, MENGTAO LI⁷, Carina Mihai⁸, Gabriela Riemekasten⁹, Petros P. Sfikakis¹⁰, Gabriele Valentini¹¹, Otylia Kowal-Bielecka¹², Yannick Allanore¹³ and Oliver Distler⁸, ¹Oslo University Hospital, Oslo, Norway, ²Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, Italy, ³V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia, ⁴Medical school of Pécs, Pecs, Hungary, ⁵University of Firenze, Firenze, Italy, ⁶University of Lille, Lille, France, ⁷Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, State Key Laboratory of Complex Severe and Rare Diseases, Ministry of Science & Technology, Beijing, China, ⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁹University Clinic Schleswit-Holstein (UKSH), Luebeck, Germany, ¹⁰Joint Academic Rheumatology Program, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece, ¹¹Università della Campania “Luigi Vanvitelli”, Napoli, Italy, ¹²Medical University of Bialystok, Bialystok, Poland, ¹³Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: Enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) have been partly successful but have not been tested in a…
Abstract Number: 1168 • ACR Convergence 2022
Dermal Fibroblast-derived Exosomes Drive Profibrotic Macrophage Activation in Systemic Sclerosis
Heetaek Yang¹, Rajan Bhandari¹, Noelle Kosarek², Jonathan Garlick³, Michael Whitfield⁴ and Patricia Pioli¹, ¹Geisel School of Medicine at Dartmouth, Lebanon, NH, ²Dartmouth Geisel School of Medicine, Lebanon, NH, ³Tufts University School of Dental Medicine, Boston, MA, ⁴Geisel School of Medicine, Lebanon, NH
Background/Purpose: Macrophage (MØ) activation derives from coordination of signals received in local tissue microenvironments. In prior studies, we demonstrated that cocultured MØs and fibroblasts from…
Abstract Number: 1367 • ACR Convergence 2022
Patient and Physician Reported Outcomes of Juvenile Systemic Sclerosis Patients Significantly Improve over 12 Months Observation Period in the Juvenile Systemic Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Maria Teresa Terreri⁶, Brian Feldman⁷, Jordi Anton⁸, Maria Katsicas⁹, Valda Stanevica¹⁰, FLAVIO SZTAJNBOK¹¹, Simone Appenzeller¹², Tadey Avcin¹³, Mikhail Kostik¹⁴, Edoardo Marrani¹⁵, Walter Alberto Sifuentes-Giraldo¹⁶, Sindu Johnson¹⁷, Raju Khubchandani¹⁸, Dana Nemcova¹⁹, Maria José Santos²⁰, Cristina Battagliotti²¹, Lillemor Berntson²², Blanca Bica²³, Jürgen Brunner²³, Rolando Cimaz²⁴, Despina Eleftheriou²⁵, Liora Harel²⁶, Gerd Horneff²⁷, Mahesh Janarthanan²⁸, Tilmann Kallinich²⁹, Kirsten Minden³⁰, Anjali Patwardhan³¹, Dieneke Schonenberg-Meinema³², Vanessa Smith³³ and Nicola Helmus³⁴, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Istanbul, Turkey, ⁴Cerrahpaşa Medical School, Istanbul University, Istanbul, Turkey, ⁵Pediatric Rheumatology, Children's Hospital of UPMC, Pittsburgh, PA, ⁶Universidad Federal São Paulo, São Paulo, Brazil, ⁷Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada, ⁸Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, ⁹Hospital Garrahan, Buenos Aires, Argentina, ¹⁰Children's Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹¹UFRJ/UERJ, São Paulo, Brazil, ¹²Unicamp, Campinas, São Paulo, Brazil, ¹³University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ¹⁴Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁵University of Florence, Firenze, Italy, ¹⁶Hospital Universitario Ramon y Cajal, Madrid, Spain, ¹⁷University of Toronto, Toronto, ON, Canada, ¹⁸SRCC Children's Hospital, Mumbai, India, ¹⁹Charles University, Prague, Czech Republic, ²⁰Hospital Garcia de Orta, Almada, Charneca da Caparica, Portugal, ²¹Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ²²Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ²³UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ²⁴University of Milano, Milano, Italy, ²⁵Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ²⁶Schneider Children's Medical center, Tel Aviv University, Nettnja, Israel, ²⁷Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ²⁸SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, India, ²⁹Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ³⁰Charité Universitätsmedizin Berlin, Berlin, Germany, ³¹University of Missouri, Columbia, MO, ³²Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ³³Department of Rheumatology, Ghent University Hospital – Department of Internal Medicine, Ghent University, Belgium – Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Gent, Belgium, ³⁴Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. The Juvenile Systemic Scleroderma Inception cohort…
Abstract Number: 1624 • ACR Convergence 2022
Blocking IL-1, IL-33 and IL-36 Signaling with the Anti-IL1RAP Antibody mCAN10 Ameliorates Inflammation and Fibrosis in Preclinical Models of Systemic Sclerosis
Caitriona Grönberg¹, Sara Rattik¹, Meik Kunz², Thoung Trinh-Minh³, Cuong Tran-Manh³, Xiang Zhou³, Petter Skoog¹, David Liberg¹ and Jörg Distler³, ¹Cantargia AB, LUND, Sweden, ²Friedrich-Alexander University (FAU) Erlangen-Nuremberg, Erlangen, Germany, ³Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: The IL-1 receptor accessory protein (IL1RAP) is a co-receptor required for signaling through the IL-1, IL-33, and IL-36 receptors. IL1RAP-dependent signaling has been implicated…
Abstract Number: 2174 • ACR Convergence 2022
Influence of Immunosuppressive Therapy on Gastrointestinal Symptoms in Patients with Systemic Sclerosis
Lea Stamm¹, Alexandru Garaiman¹, Norina Zampatti¹, Mike Becker¹, Cosimo Bruni¹, Rucsandra Dobrota¹, Muriel Elhai¹, Sherif Ismail², Suzana Jordan¹, Aurora Tatu³, Oliver Distler¹ and Carina Mihai¹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²Department of Internal Medicine, National Research Center, Cairo, Egypt, ³Department of Gastroenterology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: The gastrointestinal (GI) tract is frequently affected in systemic sclerosis (SSc), leading to considerable morbidity and even mortality. While important progress has been made…
Abstract Number: 0712 • ACR Convergence 2022
Disease Prevalence and Patient Characteristics in Patients with and Without Scleroderma Renal Crisis – A National US-based Inpatient Comparison Study
Anila Hussain¹, Pushti Khandwala² and Thais Moldovan³, ¹Crozer Chester Medical Center, Glen Mills, PA, ²Einstein Medical Center Philadelphia, Glen Mills, PA, ³Rheumatology Care Center, Media, PA
Background/Purpose: Scleroderma renal crisis is a rare complication of Systemic Sclerosis (SSc) with high mortality and morbidity. Most patients present with acute symptomatic increase in…
Abstract Number: 1173 • ACR Convergence 2022
Single-cell Multi-omic Analysis of a 3D Skin-Like Tissue Model Provides Insights into Molecular and Cellular Drivers of Systemic Sclerosis
Tamar Abel¹, Noelle Kosarek², Rezvan Parvizi³, Helen Jarnagin¹, Mengqi Huang⁴, Avi Smith⁵, Michael Mariani¹, Dillon Popovich⁶, Heetaek Yang⁷, Tammara Wood⁸, Jonathan Garlick⁹, Patricia Pioli⁷ and Michael Whitfield¹⁰, ¹Dartmouth College, Lebanon, NH, ²Dartmouth Geisel School of Medicine, Lebanon, NH, ³Dartmouth, Lebanon, NH, ⁴University of Pittsburgh, Pittsburgh, PN, ⁵Tufts University, Boston, MA, ⁶Dartmouth College, West Lebanon, NH, ⁷Geisel School of Medicine at Dartmouth, Lebanon, NH, ⁸Dartmouth College, Hanover, NH, ⁹Tufts University School of Dental Medicine, Boston, MA, ¹⁰Geisel School of Medicine, Lebanon, NH
Background/Purpose: Systemic Sclerosis (SSc) currently lacks reliable in vitro models of skin fibrosis constructed from all human cells. We have developed a skin-like tissue model of systemic…
Abstract Number: 1385 • ACR Convergence 2022
Clinical and Serologic Characterization of Juvenile Systemic Sclerosis Overlap Disease
Amanda Robinson and Kathryn Torok, Pediatric Rheumatology, Children's Hospital of UPMC, Pittsburgh, PA
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a systemic fibroinflammatory autoimmune disease characterized by skin fibrosis and multiple extracutaneous manifestations. A proportion of children with jSSc…
Abstract Number: 1659 • ACR Convergence 2022
FAM13A (Family with Sequence Similarity 13 Member A) Polymorphism Is Associated with Presence of a Radiographic Usual Interstitial Pneumonia Pattern in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease
Elana Bernstein¹, Francesco Boin², Brett Elicker³, Yawen Ren⁴, John Varga⁵ and Shervin Assassi⁶, ¹Columbia University, New York, NY, ²Cedars-Sinai Medical Center, Los Angeles, CA, ³University of California San Francisco, San Francisco, CA, ⁴University of Texas Health Science Center at Houston, Houston, TX, ⁵University of Michigan, Ann Arbor, MI, ⁶McGovern Medical School, University of Texas, Houston, TX
Background/Purpose: The pathogenesis of SSc-associated interstitial lung disease (ILD) is incompletely understood. The MUC5B promoter gain-of-function single nucleotide polymorphism (SNP) rs35705950 has been associated with…
Abstract Number: 2175 • ACR Convergence 2022
Evaluation of the Distribution of Haemodynamic Parameters and Prognostic Impact in a Cohort of Patients with Systemic Sclerosis
Zoe Brown¹, Dylan Hansen², Wendy Stevens², Laura Ross¹, Nava Ferdowsi², Susanna Proudman³, Jenny Walker⁴, Jo Sahhar⁵, Gene-Siew Ngian⁶, Lauren Host⁷, Gabor Major⁸, Mandana Nikpour⁹ and Kathleen Morrisroe², ¹St Vincent's Hospital, Melbourne, Australia, ²St Vincent's Hospital Melbourne, Melbourne, Australia, ³University of Adelaide, Medindie, Australia, ⁴Flinders Medical Centre, Flinders University, Daw Park, Australia, ⁵Monash Health, Melbourne, Australia, ⁶Melbourne Health, Northcote, Australia, ⁷Fiona Stanley Hospital, London, United Kingdom, ⁸Hunter New England Health Service, Rankin Park - Newcastle, New South Wales, Australia, ⁹The University of Melbourne at St. Vincent's Hospital Melbourne, Melbourne, Australia
Background/Purpose: Scleroderma (SSc) is a complex multisystem autoimmune disease, characterised by vasculopathy and fibrosis of skin and organs. Involvement of the cardiovascular system occurs frequently…
Abstract Number: 0832 • ACR Convergence 2022
Anti-NOR 90 Antibodies: What Is the Clinical Significance in Rheumatic Inflammatory Conditions?
Paulo Vilas-Boas¹, Gisela Eugenio² and Anabela Barcelos², ¹Rheumatology Department, Centro Hospitalar Baixo Vouga, Aveiro, Portugal, ²Centro Hospitalar Baixo Vouga, Aveiro, Portugal
Background/Purpose: Anti-NOR 90 antibodies have been detected in inflammatory conditions, neoplastic diseases and even in healthy individuals. Despite numerous epidemiologic studies, large scale studies are…
Abstract Number: 1174 • ACR Convergence 2022
Cellular Immunotherapy for Systemic Sclerosis
Chanhyuk Park¹, Rajan Bhandari², Jason Gunn³, Timothy Sullivan², Gretel Torres², Joana Murad³, Michael Whitfield⁴ and Patricia Pioli², ¹Geisel School of Medicine at Dartmouth, West Lebanon, NH, ²Geisel School of Medicine at Dartmouth, Lebanon, NH, ³Celdara Medical, Lebanon, ⁴Geisel School of Medicine, Lebanon, NH
Background/Purpose: Pro-fibrotic macrophages (MØs) are implicated in the pathogenesis of systemic sclerosis (SSc). In prior work, we and others have shown that MØs derived from…
Abstract Number: 1521 • ACR Convergence 2022
Long-term Follow-Up of Participants of the Phase II Study That Evaluated Subcutaneous Abatacept vs. Placebo in Diffuse Cutaneous Systemic Sclerosis (ASSET Trial)
Nadine Sarsam¹, Sindhu R. Johnson², Lorinda Chung³, Suzanne Kafaja⁴, Janet Pope⁵, Robyn Domsic⁶, Maureen Mayes⁷, Nora Sandorfi⁸, Virginia Steen⁹, Flavia Castelino¹⁰, Faye Hant¹¹, Suiyuan Huang¹ and Dinesh Khanna¹², ¹University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, Department of Medicine, Schroeder Arthritis Institute, Krembil Research Institute, Toronto Western and Mount Sinai Hospitals; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada, ³Stanford University, Stanford, CA, ⁴UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ⁵University of Western Ontario, London, ON, Canada, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁸University of Pennsylvania, Philadelphia, PA, ⁹Georgetown University School of Medicine, Washington, DC, ¹⁰Massachusetts General Hospital, Boston, MA, ¹¹Medical University of South Carolina, Charleston, SC, ¹²Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: Abatacept (ABA) is a cytotoxic T-lymphocyte-associated molecule-4 immunoglobulin fusion protein which has been shown to induce regression of dermal fibrosis in patients with systemic…

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