Abstracts tagged "Systemic sclerosis"

Abstract Number: 0947 • ACR Convergence 2023
Fcγ Receptors Define Pro-Phagocytic Macrophages and Trigger Pro-Inflammatory Responses in Patients with Systemic Sclerosis
Amela Hukara¹, Gino Andrea Bonazza¹, Tracy Tabib², Raphael Micheroli¹, Suzana Jordan³, Kristina Bürki¹, Sylvie Schlitz Schönbächler¹, Adrian Ciurea⁴, Oliver Distler³, Robert Lafyatis⁵, Przemyslaw Blyszczuk¹ and Gabriela Kania¹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁴University Hospital Zurich, Zürich, Switzerland, ⁵University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Fcy receptors (FcγR) are opsonic phagocytic receptors, requiring tight regulations to prevent uncontrolled activation of pro-inflammatory phagocytosis. SSc macrophages display an alternatively-activated profibrotic phenotype.…
Abstract Number: 1513 • ACR Convergence 2023
Trends in Systemic Sclerosis- related Mortality by Age, Sex and Race in the United States, 1999-2019
Anum Akhlaq¹, Emily He² and Risha Fayyaz³, ¹University of Mississippi Medical Center, Jackson, MS, ²Loma Linda University Health, Loma Linda, CA, ³University of Mississippi Medical Center, Flowood, MS
Background/Purpose: Systemic Sclerosis (SSc) is a rare chronic disease characterized by widespread vascular damage and tissue fibrosis of the skin and various internal organs, which…
Abstract Number: 1667 • ACR Convergence 2023
Characterization of the Vascular Niches in Systemic Sclerosis (SSc) as a Prototypical Immune-mediated Fibrotic Disease with Prominent Vasculopathy Using Imaging Mass Cytometry
Minrui Liang¹, Aleix Rius Rigau², yi-nan Li¹ and Joerg Distler¹, ¹Clinic for Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany; Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany, ²Department of Internal Medicine 3, Rheumatology and Clinical Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany; Deutsches Zentrum Immuntherapie (DZI), FAU Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: Systemic sclerosis (SSc) is a prototypical collagen-vascular disease with prominent vasculopathy. Although microvascular changes are the earliest histopathologic manifestation of SSc, the vascular dysregulation…
Abstract Number: 2360 • ACR Convergence 2023
Systemic Sclerosis Macrophages Stimulate Calcinosis in Adipose Derived Mesenchymal Stem Cells via the Activin a Pathway
Tamara Searle¹, Ujvalla Kalluri¹, Sonia Ahmad², Bahja Ahmed Abdi¹, Sandra Lopez¹, Shiwen Xu¹, Teresa Collins¹, Jennifer Cross³, George Martin⁴, Henry Lopez⁵, Clayton Yates⁶ and Richard Stratton¹, ¹University College London, London, United Kingdom, ²Holy Family Red Crescent Medical College, Dhaka, Bangladesh, ³Aurinia Pharmaceuticals Inc., Rockford, MD, ⁴Riptide Bioscience Inc., Bethesda, MD, ⁵Riptide Bioscience Inc., Valejo, CA, ⁶Tuskegee University, Tuskegee, AL
Background/Purpose: Calcinosis may result from localised trans-differentiation of tissue resident stem cells in the subcutaneous layer of affected skin in systemic sclerosis (SSc), as a…
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: 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: 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…
Abstract Number: 1661 • ACR Convergence 2022
Osteoclastogenesis and Vascular Endothelial Growth Factor Blood Levels in Patients with Systemic Sclerosis with and Without Calcinosis Cutis
Antonia Valenzuela¹, Guillermo Perez², Carolina Iturriaga² and Rebeca Montalva², ¹Pontificia Universidad Catolica de Chile, Santiago, Chile, ²Pontificia Universidad Catolica de Chile, Santiago de Chile, Chile
Background/Purpose: Calcinosis cutis is a debilitating complication of systemic sclerosis (SSc) with no effective treatments. Although the pathogenesis of SSc-related calcinosis remains unknown, there is…
Abstract Number: 2178 • ACR Convergence 2022
BNT162b2 mRNA Vaccine Against Covid 19, Humoral Response, Safety and Efficacy in Scleroderma Patients
Yolanda Braun-Moscovici¹, Marielle Kaplan¹, Maya Braun², Doron Markovits³, Sami Giryes³, Kohava Toledano³, Yonit Tavor³, Katya Dolnikov³, Rula Daood³ and Alexandra Balbir-Gurman⁴, ¹Rambam Health Care Campus and Rappaport Faculty of Medicine, Haifa, Israel, ²Hebrew University of Jerusalem, Bioinformatics, Jerusalem, Israel, ³Rambam Health Care Campus, Haifa, Israel, ⁴Rheumatology Institute, Rambam Health Care Campus, Haifa, Israel
Background/Purpose: In the begining of pandemic and till spring 2021 there were no clinical and laboratory data regarding sefety and efficacy of mRNA vaccines against…
Abstract Number: 1048 • ACR Convergence 2022
Nailfold Capillaroscopy and Candidate Biomarker Levels in Systemic Sclerosis-associated Pulmonary Hypertension, Profiling of Non-invasive Markers: A Cross Sectional Study
Jacqueline Lemmers¹, Arjan van Caam², Brigit Kersten², Els Ende, van den², Hanneke KNAAPEN², Arie Dijk, van², Wanda Hagmolen of ten Have², Frank van den Hoogen², Hans Koenen², Sander van Leuven³, wynand Alkema⁴, Ruben Smeets² and Madelon Vonk⁵, ¹Radboud University Medical Centre Nijmegen, Nijmegen, Netherlands, ²Radboudumc, Nijmegen, Netherlands, ³Radboud University, Nijmegen, Netherlands, ⁴Hanze University, Groningen the Netherlands, Dept. Data Science for Life Sciences & Health, Groningen, Netherlands, ⁵Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands
Background/Purpose: Pulmonary hypertension (PH) is one of the leading causes of death in Systemic Sclerosis (SSc). Early detection and treatment of PH in SSc is…
Abstract Number: 1177 • ACR Convergence 2022
Identification of a New Type of Pro-phagocytic Macrophages in Patients with Systemic Sclerosis
Amela Hukara¹, Tracy Tabib², Michal Rudnik¹, Oliver Distler³, Przemyslaw Blyszczuk¹, Robert Lafyatis² and Gabriela Kania¹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: Phagocytosis is a crucial cellular process, which under certain immuno-pathological conditions can be activated in macrophages in an uncontrolled manner. Biomarker studies have implicated…
Abstract Number: 1523 • ACR Convergence 2022
Anti-RNPC3 Autoantibodies in Mixed Connective Tissue Disease Are Associated with Systemic Sclerosis Skin Involvement
Darya S. Jalaledin¹, Hajar El Kamouni², Alexandra Albert³, Sabrina Hoa⁴, Josiane Bourre-Tessier⁵, Eric Rich⁵, Jean-Richard Goulet⁶, Martial Koenig⁷, Minoru Satoh⁸, Marvin Fritzler⁹, May Choi¹⁰, Yves Troyanov¹¹, Jean-Luc Senécal, MD¹² and Océane Landon-Cardinal⁵, ¹Division of Internal Medicine, Centre Hospitalier de l'Université de Sherbrooke, Saint-Lambert, Canada, ²Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Laval, Canada, ³Division of Rheumatology, Centre Hospitalier de l'Université Laval, Québec, QC, Canada, ⁴University of Montreal, Montréal, QC, 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, ¹²Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Montréal, Canada
Background/Purpose: RNA-binding region containing 3 (RNPC3) protein acts as a molecular bridge, promoting U11/U12 RNP complex formation. In previous studies, systemic sclerosis (SSc) patients who…

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