Abstracts tagged "Systemic sclerosis"

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…
Abstract Number: 1660 • ACR Convergence 2022
Skeletal Myopathy in Systemic Sclerosis Associates with Higher Disease Burden and Mortality
Julie Paik¹, Caoilfhionn Connolly¹, Fredrick Wigley¹ and Laura Hummers², ¹Johns Hopkins University, Baltimore, MD, ²Johns Hopkins Univerisity, Baltimore, MD
Background/Purpose: Skeletal myopathy in systemic sclerosis (SSc) is poorly understood. The etiology of myopathy in SSc is heterogeneous and can result from an inflammation, disuse,…
Abstract Number: 2177 • ACR Convergence 2022
Familial Clustering of Systemic Sclerosis, Autoimmunity, and Cancer History
Mariana Bombini¹, Nailú Sinicato¹, Jacqueline Paredes², Theresa Wampler Muskardin², Tiago Nardi Amaral¹, Ana Paula Del Rio¹, Joao Marques Neto³, Timothy Niewold² and Simone Appenzeller⁴, ¹State University of Campinas, Campinas, São Paulo, Brazil, ²Hospital for Special Surgery, New York, NY, ³State University of Campinas, São Paulo, Brazil, ⁴Unicamp, Campinas, São Paulo, Brazil
Background/Purpose: Systemic sclerosis (SSc) is a complex disease characterized by chronic inflammation, fibrosis, and vasculopathy. Genetic factors contribute to pathogenesis, but because it is a…
Abstract Number: 0968 • ACR Convergence 2022
Neuropsychological Outcome of Children Born to Women with Systemic Sclerosis: Assessment Through a Self-administered Multidisciplinary Questionnaire in a Monocentric Cohort
Eleonora Pedretti¹, Laura Andreoli¹, Liala Moschetti¹, Cecilia Nalli¹, Anna Molinaro², Jessica Galli², Elisa Fazzi², Franco Franceschini¹, Angela Tincani³, Paolo Airò⁴ and Maria Grazia Lazzaroni¹, ¹Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Brescia, Italy, ²Unit of Child and Adolescent Neuropsychiatry, ASST Spedali Civili and University of Brescia, Brescia, Italy, ³Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Gussago, Italy, ⁴Rheumatology and Clinical Immunology Unit, ASST Spedali Civili, Brescia, Italy
Background/Purpose: In the last years, the rate of successful pregnancies has significantly increased in Systemic Sclerosis (SSc)1 women. However, the long-term outcome of their children…
Abstract Number: 1176 • ACR Convergence 2022
Immunoglobulins G from Systemic Sclerosis Patients May Alter the Secretome of Dermal Fibroblasts
Aurélien Chepy¹, Marie Duhamel², Solange Vivier³, lucile Guilbert³, Eric Hachulla⁴, Sylvain Dubucquoi³, David Launay³, Michel Salzet² and Vincent Sobanski³, ¹CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France, ²Univ. Lille, Inserm, CHU Lille, U1192 - Protéomique Réponse Inflammatoire Spectrométrie de Masse - PRISM, Lille, France, ³Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France, ⁴University of Lille, LILLE, France
Background/Purpose: Autoantibodies (Aab) are frequent in systemic sclerosis (SSc).Recently, it has been shown that immunoglobulins G (IgG) from SSc promoted a proinflammatory and profibrotic phenotype…
Abstract Number: 1522 • ACR Convergence 2022
The Collaborative National Quality and Efficacy Registry for Scleroderma: Association of Medication Use on Gastrointestinal Tract Symptoms in Early Disease
Sarah Luebker¹, Tracy Frech¹, Shervin Assassi², Jessica Gordon³, Elana Bernstein⁴, Virginia Steen⁵, Ami Shah⁶, Laura Hummers⁷, 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 Rheumatology, Baltimore, MD, ⁷Johns Hopkins Univerisity, 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: Gastrointestinal tract (GIT) symptoms are common amongst systemic sclerosis (SSc) patients.1 The Scleroderma Clinical Trials Consortium University of California Los Angeles Gastrointestinal Tract Questionnaire…

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