Abstracts tagged "Systemic sclerosis"

Abstract Number: 1232 • ACR Convergence 2023
Improvement Across Organ System, Physician and Patient Reported Outcome Measures over a 36-time Period in the Juvenile Systemic Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn Torok⁵, Brian Feldman⁶, Maria Teresa TErreri⁷, Ana Sakamoto⁸, Jordi Anton⁹, Simone Appenzeller¹⁰, Edoardo Marrani¹¹, Maria Katsikas¹², Maria Jose Santos¹³, FLAVIO SZTAJNBOK¹⁴, Lillemor Berntson¹⁵, Juergen Brunner¹⁶, Sindhu Johnson¹⁷, Mikhail Kostik¹⁸, Kirsten Minden¹⁹, Farzana Nuruzzaman²⁰ and Nicola Helmus²¹, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, ⁴Istanbul University - Cerrahpasa, Istanbul, Turkey, ⁵University of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, ⁶The Hospital for Sick Children, Toronto, ON, Canada, ⁷UNIFESP, São Paulo, Brazil, ⁸Federal University of São Paulo (UNIFESP), São Paulo, Brazil, ⁹Hospital Sant Joan de Déu, Pediatric Rheumatology Department, Universitat de Barcelona, Barcelona, Spain, ¹⁰UNICAMP, Campinas, Brazil, ¹¹University of Florence, Firenze, Italy, ¹²Hospital de Pediatria Juan P Garrahan, Servicio de Inmunologia/Reumatologia, Buenos Aires, Argentina, ¹³Hospital Garcia de Orta, Almada, Lisboa, Portugal, ¹⁴UFRJ/UERJ, São Paulo, Brazil, ¹⁵Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ¹⁶Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ¹⁷Institute of Health Policy, Management, and Evaluation, University of Toronto, Toronto, ON, Canada, ¹⁸Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁹Charité University Medicine and German Rheumatism Research Center Berlin, Berlin, Germany, ²⁰Stony Brook Children's Hospital, Stony Brook, NY, ²¹Hamburg 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: 1664 • ACR Convergence 2023
Fibroblast Specific Interferon Regulatory Factor 7 (IRF7) Expression Is a Key Link Between Type I Interferon Activation and the Exaggerated Dermal and Pulmonary Fibrosis in Systemic Sclerosis
Minghua Wu¹, Jerry Alonso², Julio charles², Brian Skaug², Tingting Mills³, Maureen Mayes² and Shervin Assassi⁴, ¹Department of Internal Medicine, Division of Rheumatology, The University of Texas Health Science Center at Houston, Houston, TX, ²Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ³Biochemistry Department, University of Texas McGovern Medical School, Houston, TX, ⁴University of Texas McGovern Medical School at Houston, Houston, TX
Background/Purpose: A prominent interferon (IFN) activation signature has been observed at the peripheral blood and end-organ levels in systemic sclerosis (SSc). However, the mechanisms by…
Abstract Number: 2357 • ACR Convergence 2023
The Involvement of CCR3-CCL24 Axis in Endothelial to Mesenchymal Transition Process and Pulmonary Arterial Hypertension in Systemic Sclerosis Patients
Itzchak Amoyal¹, Tzipi Hornik-Lurie², Tali Zitman-Gal¹, Hilit Levy³, Ilan Vaknin³, Liat Drucker⁴, Ishai Heusler⁵, Yair Levy¹ and Shelly Tartakover Matalon¹, ¹Meir Medical Center, Autoimmune Research Laboratory, Kfar-Saba, Israel, ²Meir Medical Center, Data Research Department, Kfar-Saba, Israel, ³Chemomab Ltd, Tel Aviv, Israel, ⁴Meir Medical Center, Oncogenetics Laboratory, Kfar Saba, Israel, ⁵Meir Medical Center, Department of Obstetric and Genecology, Kfar-Saba, Israel
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular injury and extensive tissue fibrosis of the skin and internal organs. Endothelial cells (ECs),…
Abstract Number: 0611 • ACR Convergence 2023
Demographics and Clinical Features Associated with Small Bowel Hypomotility in Systemic Sclerosis
Jenice Cheah¹, Jamie Perin², Michael Hughes³, Julie Paik⁴, Christopher Mecoli², Laura Hummers⁴, Ami Shah⁵, Fred Wigley⁶ and Zsuzsanna McMahan⁷, ¹University of California Los Angeles, Los Angeles, CA, ²Johns Hopkins University School of Medicine, Baltimore, MD, ³Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, ⁴Johns Hopkins University, Baltimore, MD, ⁵Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, ⁶Department of Rheumatology, Johns Hopkins Medicine, Baltimore, MD, ⁷Johns Hopkins Rheumatology, Lutherville, MD
Background/Purpose: The gastrointestinal (GI) tract is the second most commonly affected organ in patients with systemic sclerosis (SSc). It is estimated that the small bowel…
Abstract Number: 0628 • ACR Convergence 2023
Interstitial Lung Disease in Very Early Systemic Sclerosis
Sinziana Muraru¹, Carina Mihai¹, Anna-Maria Hoffmann-Vold², Cosimo Bruni¹, Muriel Elhai³, Mike Becker⁴, Suzana Jordan¹, Alexandru Garaiman¹, Oliver Distler¹ and Rucsandra Dobrota¹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Oslo University Hospital, Oslo, Norway, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: Interstitial lung disease (ILD) affects around 50% of patients with systemic sclerosis (SSc) and is the main cause of mortality. Preliminary data from small…
Abstract Number: 0650 • ACR Convergence 2023
Understanding the Employment Landscape in People with Systemic Sclerosis
Hila Jazayeri¹, Zareen Ahmad¹, Monique Gignac² and Sindhu Johnson³, ¹University of Toronto, Toronto, ON, Canada, ²Institute for Work & Health, Toronto, ON, Canada, ³Division 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
Background/Purpose: Systemic sclerosis (SSc) is a systemic rheumatic disease that restricts participation in various life roles, including in the workplace. Our objectives were to identify…
Abstract Number: 0672 • ACR Convergence 2023
Risk of New-onset Depression in Patients with Systemic Sclerosis : A Nationwide Population-based Study
Hahee Son and Su-Jin Moon, Yeouido St. Mary's Hospital, Catholic University of Korea, Seoul, South Korea
Background/Purpose: Systemic sclerosis (SSc) is a rare chronic inflammatory disease characterized by vasculopathy, autoimmunity and progressive fibrosis in various organs. The patients with SSc are…
Abstract Number: 0946 • ACR Convergence 2023
Immunoglobulins G Purified from Systemic Sclerosis Patients Influence Cytoplasmic and Nuclear Proteins Profile in Dermal Fibroblasts
Aurélien Chepy¹, Marie Duhamel², Solange Vivier¹, Clément Chauvet¹, Lucile Guilbert³, Eric Hachulla⁴, Sylvain Dubucquoi¹, David Launay¹, Michel Salzet² and Vincent Sobanski¹, ¹Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France, ²Univ. Lille, Inserm, CHU Lille, U1192 - Protéomique Réponse Inflammatoire Spectrométrie de Masse - PRISM, Lille, France, ³CHU Lille, Institut d’Immunologie, Lille, France, ⁴CHU 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
Background/Purpose: Autoantibodies (Aab) are frequent in systemic sclerosis (SSc). While recognized as potent biomarkers, their pathogenic role is unclear. Recently, we showed that immunoglobulins G…
Abstract Number: 1233 • ACR Convergence 2023
The Pattern of Medication Use Significantly Changed over 36 Months Observation Period. Result from the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Brian Feldman⁵, Kathryn Torok⁶, Maria Teresa TErreri⁷, Ana Sakamoto⁸, Jordi Anton⁹, Simone Appenzeller¹⁰, Edoardo Marrani¹¹, Maria Jose Santos¹², FLAVIO SZTAJNBOK¹³, Lillemor Berntson¹⁴, Juergen Brunner¹⁵, Mikhail Kostik¹⁶, Farzana Nuruzzaman¹⁷ and Nicola Helmus¹⁸, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, ⁴Istanbul University - Cerrahpasa, Istanbul, Turkey, ⁵The Hospital for Sick Children, Toronto, ON, Canada, ⁶University of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, ⁷UNIFESP, São Paulo, Brazil, ⁸Federal University of São Paulo (UNIFESP), São Paulo, Brazil, ⁹Hospital Sant Joan de Déu, Pediatric Rheumatology Department, Universitat de Barcelona, Barcelona, Spain, ¹⁰UNICAMP, Campinas, Brazil, ¹¹University of Florence, Firenze, Italy, ¹²Hospital Garcia de Orta, Almada, Lisboa, Portugal, ¹³UFRJ/UERJ, São Paulo, Brazil, ¹⁴Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ¹⁵Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ¹⁶Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ¹⁷Stony Brook Children's Hospital, Stony Brook, NY, ¹⁸Hamburg 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. Currently no medications are licensed for the treatment…
Abstract Number: 1665 • ACR Convergence 2023
Endothelial Response to Type I Interferon Contributes to Vasculopathy and Fibrosis and Predicts Disease Progression of Systemic Sclerosis
Hanlin Yin¹, Oliver Distler², Bin Li³, Qingran Yan⁴ and Liangjing Lu¹, ¹Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China, ²Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³Shanghai institute of immunology, Shanghai, China, ⁴Department of Rheumatology, Renji Hospital, Shanghai Jiaotong Univeisty School of medcine, Shanghai, China
Background/Purpose: Type I interferon (IFN-1) signature is a hallmark of patients with systemic sclerosis (SSc). However, its significance in clinical stratification and contribution to deterioration…
Abstract Number: 2359 • ACR Convergence 2023
Reversal of the Activation State of Pro-fibrotic CD206 Positive Macrophages by Therapeutic Peptide AUR300 in Systemic Sclerosis
Sandra Lopez¹, Bahja Ahmed Abdi¹, Linda Lei¹, Anas Al-Oweidi¹, Claire Macfadyen¹, Lydia Nagib¹, Sarah Ive¹, Anshul Kumar¹, Teresa Collins¹, Jennifer Cross², Christopher Denton¹, David Abraham¹, Clayton Yates³, Charles Garvin⁴, Henry Lopez⁴, George Martin⁵ and Richard Stratton¹, ¹University College London, London, United Kingdom, ²Aurinia Pharmaceuticals Inc., Rockford, MD, ³Tuskegee University, Tuskegee, AL, ⁴Riptide Bioscience Inc., Valejo, CA, ⁵Riptide Bioscience Inc., Bethesda, MD
Background/Purpose: A spectrum of macrophage activation is seen in human pathologic conditions; M2-like CD206 positive macrophages are pro-fibrotic and proresolution, while M1-like macrophages are proinflammatory,…
Abstract Number: 0612 • ACR Convergence 2023
COVID Vaccinations and Infections Among Individuals with Systemic Sclerosis: A Scleroderma Patient-centered Intervention Network (SPIN) Cohort Study
Kimberly Lakin¹, Jessica Gordon¹, Yin Wu², Linda Kwakkenbos³, Marie-Eve Carrier², Richard Henry², Christopher Denton⁴, Luc Mouthon⁵, Robert Spiera⁶ and Brett Thombs², ¹Hospital for Special Surgery, New York, NY, ²Lady Davis Institute for Medical Research, Jewish General Hospital, Montreal, QC, Canada, ³Radboud University, Nijmegen, Netherlands, ⁴University College London, London, United Kingdom, ⁵Hopital Cochin - Paris University, Paris, France, ⁶Hosptial for Special Surgery, New York, NY
Background/Purpose: We previously surveyed individuals with systemic sclerosis (SSc) enrolled in Scleroderma Patient-centered Intervention Network (SPIN) Cohort between April-May 2021 to determine COVID-19 vaccination rates,…
Abstract Number: 0630 • ACR Convergence 2023
Prevalence and Risk Factors for Systemic Sclerosis Digital Ischemic Complications in the Collaborative National Quality and Efficacy Registry
Marissa Savoie¹, Monica Harding², Jessica Alvey², John VanBuren², Shervin Assassi³, Elana Bernstein⁴, Lorinda Chung⁵, Luke Evnin⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Laura Hummers¹⁰, Dinesh Khanna¹¹, Kimberly Lakin⁸, Dorota Lebiedz-Odrobina¹², Yiming Luo¹³, Ashima Makol¹⁴, Jerry Molitor¹⁵, Duncan Moore¹⁶, Carrie Richardson¹⁷, Nora Sandorfi¹⁸, Ami Shah¹⁹, Ankoor Shah²⁰, Brian Skaug²¹, Virginia Steen²², Elizabeth Volkmann²³ and Flavia Castelino¹, ¹Massachusetts General Hospital, Boston, MA, ²University of Utah, Salt Lake City, UT, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁵Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, ⁶Scleroderma Research Foundation, Brisbane, CA, ⁷Vanderbilt University Medical Center, Nashville, TN, ⁸Hospital for Special Surgery, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Johns Hopkins University, Baltimore, MD, ¹¹University of Michigan, Ann Arbor, MI, ¹²University of Utah, Cottonwood Heights, UT, ¹³Division of Rheumatology, Department of Medicine, Columbia University Irving Medical Center, New York, NY, ¹⁴Mayo Clinic, Rochester, MN, Rochester, MN, ¹⁵University of Minnesota, Minneapolis, MN, ¹⁶Northwestern University, Department of Rheumatology, Chicago, IL, ¹⁷Northwestern University, Chicago, IL, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, ²⁰Duke University, Durham, NC, ²¹Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, ²²Georgetown University School of Medicine, Washington, DC, ²³University of California Los Angeles, Los Angeles, CA
Background/Purpose: Digital ischemic ulcers (DIU) develop in 36-44% of patients with systemic sclerosis (SSc).1,2Prior international observational studies have evaluated characteristics of digital pitting and ulcers…
Abstract Number: 0651 • ACR Convergence 2023
Abnormal Nailfold Video-capillaroscopy Is Independently Associated with non-UIP Radiographic Patterns in Autoimmune ILD: A Multicenter Study from the NEREA Registry
Jesus Loarce-Martos¹, Hilda Godoy², Laura Cebrian³, M Jesus Rodriguez-Nieto⁴, Juan Rigual⁵, Rosalía Laporta², Belen Lopez-Muñiz³, lydia Abasolo⁶, Olga Sanchez Pernaute⁷ and Fredeswinda Romero⁷, ¹Ramón y Cajal University Hospital, Madrid, Spain, ²Hospital Universitario Puerta de Hierro, Madrid, Spain, ³Hospital Universitario Infanta Leonor, Madrid, Spain, ⁴Pulmonology department, Hospital Fundación Jimenez Díaz, Madrid, Spain, ⁵Pulmonology department, Hospital Ramón y Cajal,, Madrid, Spain, ⁶Hospital Clínico San Carlos, Madrid, Spain, ⁷IIS-HU Fundación Jiménez Díaz, Madrid, Spain
Background/Purpose: The aim of this study is to describe the nailfold video-capillaroscopy (NVC) findings in patients with interstitial lung disease associated to connective tissue diseases…
Abstract Number: 0783 • ACR Convergence 2023
Tolerability of CAR T Cell Therapy in Autoimmune Disease
Jule Taubmann¹, Fabian Müller², Michael Aigner³, Ioanna Minopoulou⁴, Johannes Knitza⁵, Dagmar Werner⁶, Christina Bergmann⁷, Gerhard Kroenke⁸, Andreas Mackensen³ and Georg Schett⁶, ¹Department of Internal Medicine 3, Rheumatology and Immunology, Universitätsklinikum Erlangen and Friedrich-Alexander-Universität Erlangen Nürnberg, Erlangen, Germany, ²Friedrich Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ³Department of Internal Medicine 5, Hematology and Oncology, Universitätsklinikum Erlangen and Friedrich-Alexander-Universität Erlangen Nürnberg, Erlangen, Germany, ⁴Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg & Universitätsklinikum Erlangen, Department of Internal Medicine 3 - Rheumatology and Immunology / Deutsches Zentrum Immuntherapie, Erlangen, Germany, ⁵Department of Internal Medicine 3 Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany, ⁶FAU Erlangen-Nürnberg, Erlangen, Germany, ⁷Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, ⁸Charite Berlin, Berlin, Germany
Background/Purpose: We have previously shown that CD19 CAR T cell therapy can lead to improvement of severe forms of B cell mediated autoimmune disease including…

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