Abstracts tagged "Scleroderma"

Abstract Number: 0497 • ACR Convergence 2021
Efficacy in Patient Subgroups in the INCREASE Trial, a Phase III Trial to Evaluate Inhaled Treprostinil in Patients with Pulmonary Hypertension Due to Parenchymal Lung Disease
Victor Tapson¹, Steven Nathan², Reda Girgis³, James Runo⁴, Remzi Bag⁵, Arunabh Talwar⁶, Peter Smith⁷, Lisa Edwards⁷, Christine Park⁷ and Aaron Waxman⁸, ¹Cedars-Sinai, Los Angeles, CA, ²Inova Fairfax, Falls Church, VA, ³Michigan State University, Lansing, MI, ⁴University of Wisconsin, Madison, WI, ⁵University of Chicago, Chicago, IL, ⁶Hofstra Northwell School of Medicine, Hempstead, NY, ⁷United Therapeutics Corporation, Durham, NC, ⁸Brigham and Women's Hospital, Boston, MA
Background/Purpose: INCREASE was a 16-week trial evaluating the safety and efficacy of inhaled treprostinil (iTRE) in patients with pulmonary hypertension associated with interstitial lung disease…
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: 0501 • ACR Convergence 2021
Immunogenicity of a Single Dose of Covid-19 Vaccination in Patients with Systemic Sclerosis with or Without Immunosupression
Vishal Kakkar¹, Rebecca Ross², Ranjitha Karanth³, Sumit Lahiri⁴, Panji Mulipa¹, Pamela Hughes⁵, Brendan Clarke⁵, Clive Carter⁵, Mark Lobb⁵, Sinisa Savic⁶ and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²LIRMM University of Leeds, Leeds, United Kingdom, ³Leeds Institute of Rheumatic and Musculoskeletal Medicine, LTHT, Leeds, United Kingdom, ⁴Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁵Transplant and Cellular Immunology, Leeds, United Kingdom, ⁶Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) is a rare connective tissue disease with multi-systemic involvement, which at times requires the use of immunosuppressive medication. None of the…
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: 0542 • ACR Convergence 2021
Functional Characterization of Glycoprotein Nonmetastatic Melanoma Protein B in Scleroderma Fibrosis
Pamela Palisoc¹, Leah Vaikutis¹, Ellen Model¹, Morgan Omara¹, Dinesh Khanna¹, Eliza Pei-Suen Tsou¹ and Amr Sawalha², ¹University of Michigan, Ann Arbor, MI, ²University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Glycoprotein nonmetastatic melanoma protein B (GPNMB) is widely expressed on stromal cells and immune cells. It is involved in various cell functions such as…
Abstract Number: 1643 • ACR Convergence 2021
Increased Incidence of Pediatric SLE and Other Interferon Activated Diseases During COVID-19 Pandemic
Xinyu Dou¹, David Kaelber² and Hulya Bukulmez¹, ¹MetroHealth Medical Center, Case Western Reserve, Cleveland, OH, ²The MetroHealth System Campus of Case Western Reserve University, Cleveland, OH
Background/Purpose: COVID-19, as a member of the Coronavirus family, has been described to trigger host immune response via type I interferon (IFN) signaling pathways with…
Abstract Number: 0557 • ACR Convergence 2021
The Beta Secretase BACE1 Induces Systemic Sclerosis (SSc) Fibroblast Activation Through the Regulation of the Pro-Fibrotic Notch Signalling Pathway
Christopher Wasson¹, Eva Clavane², Rebecca Ross¹, Paul Meakin² and Francesco Del Galdo³, ¹LIRMM University of Leeds, Leeds, United Kingdom, ²LICAMM University of Leeds, Leeds, United Kingdom, ³University of Leeds, Leeds, United Kingdom
Background/Purpose: Extensive work in the Alzheimer’s field has shown BACE1 plays an important role in amyloid beta processing. Additionally, there is evidence showing BACE1 regulating…
Abstract Number: 1838 • ACR Convergence 2021
Further Construct Validation of the
Julia Spierings¹, Voon Ong² and Christopher Denton³, ¹University Medical Center Utrecht, Utrecht, Netherlands, ²University College London Medical School Royal Free Campus, London, United Kingdom, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom
Background/Purpose: Evaluation of skin is central in clinical management of systemic sclerosis (SSc). Due to the COVID-19 pandemic remote consultations were widely implemented, which inevitably…
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: 1090 • ACR Convergence 2021
Contribution of Scleroderma/Myositis-Related Antibodies Detected by Immunoblot to the Diagnosis of Systemic Autoimmune Rheumatic Diseases in 134 Patients from a Single Referral Center
Diana Prieto-Peña¹, Belén Atienza-Mateo², Miguel Ángel gonzalez-Gay³, Ricardo Blanco⁴ and Marcos Lopez-Hoyos⁵, ¹Hospital Universitario Marqués de Valdecilla, santander, Spain, ²Group "Research in genetic epidemiology and atherosclerosis of systemic diseases and in bone metabolic diseases of the locomotor system", IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Division of Rheumatology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, ⁴Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ⁵Marques de Valdecilla University Hospital-IDIVAL, Santander, Spain
Background/Purpose: Immunoblot assays are increasingly used in clinical practice as part of the diagnostic armamentarium of systemic autoimmune rheumatic diseases (SARDs). Our aim was to…
Abstract Number: 1852 • ACR Convergence 2021
Healthcare Utilization and Economic Burden in Systemic Sclerosis
Leonardo Martin Calderon¹, Mitali Chaudhary¹ and Janet Pope², ¹Western University, London, ON, Canada, ²University of Western Ontario, London, ON, Canada
Background/Purpose: Systemic Sclerosis (SSc) is a multi-system autoimmune disease, characterized by vasculopathy, fibrosis of the skin and internal organs, and autoimmunity with distinct antibodies. SSc…
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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