Abstracts tagged "Systemic sclerosis"

Abstract Number: 0626 • ACR Convergence 2023
Clinical Features of Anti-hUBF Antibodies-positive Patients: A Single-center Retrospective Study
Natsumi Fushida, Motoki Horii, Kie Mizumaki, Natsuki Numata, Yasuhito Hamaguchi and Takashi Matsushita, Department of Dermatology, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa Ishikawa, Japan
Background/Purpose: Anti-human upstream-binding factor (hUBF) antibodies (Abs) have been reported predominantly in patients with connective tissue diseases (CTDs) but have also been reported in non-CTDs…
Abstract Number: 0648 • ACR Convergence 2023
Incidence and Prevalence of Malnutrition and Its Impact on Mortality in the Multicentre Singapore Systemic Sclerosis Cohort
Bochao Jiang¹, Ennaliza Salazar², Seyed Ehsan Saffari³, Gim Gee Teng⁴, Xin Rong Lim⁵, Sue-Ann Ng², Amelia Santosa⁶, Cassandra Hong² and Andrea Hsiu Ling Low⁷, ¹Ministry of Health Holdings, Singapura, Singapore, ²Singapore General Hospital, Singapore, Singapore, ³Center for Quantitative Medicine, Duke-NUS Medical School, National University of Singapore, Singapore, Singapore, ⁴Alexandra Hospital, Department of Medicine, Division of Rheumatology, National University Health System, Singapore, Singapore, ⁵Tan Tock Seng Hospital, Singapore, Singapore, ⁶Division of Rheumatology, Department of Medicine, National University Hospital, National University Health System, Singapore, Singapore, ⁷Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore
Background/Purpose: Gastrointestinal (GI) involvement is common in patients with systemic sclerosis (SSc), and may lead to malnutrition that adversely affects quality of life, morbidity and…
Abstract Number: 0669 • ACR Convergence 2023
Optical Coherence Tomography Angiography in Systemic Sclerosis: Correlations with Morphological and Functional Peripheral Microvascular Status
Carlo Alberto Cutolo¹, Elvis Hysa², Andrea Cere³, Paola Toma¹, Tommaso Cannavacciuolo¹, Chiara Toma¹, Serena Balito⁴, Veronica Gerli⁴, Alberto Sulli³, Vanessa Smith⁵, Carlo Enrico Traverso¹, Massimo Nicolò¹ and Maurizio Cutolo⁶, ¹Ophthalmology Clinic DiNOGMI, IRCCS Ophthalmology Unit San Martino Polyclinic, University of Genoa, Genoa, Italy, ²Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy, ³Laboratory of Experimental Rheumatology and Academic Division of Rheumatology, Department of Internal Medicine, University of Genoa, IRCCS San Martino Polyclinic Hospital, Genoa, Italy, ⁴Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genoa, Italy - IRCCS San Martino Polyclinic, Genoa, Italy, ⁵Ghent University Hospital, Gent, Belgium, ⁶Laboratory of Experimental Rheumatology, Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic, Genova, Italy
Background/Purpose: Systemic sclerosis (SSc) is a rare and complex autoimmune disease featured by a progressive microvascular damage. The involvement of the ocular microvasculature has been…
Abstract Number: 0943 • ACR Convergence 2023
Attenuation of Fibroblast Activation and Fibrosis by Adropin in a Hedgehog-Dependent Manner
Minrui Liang and Joerg Distler, Clinic 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
Background/Purpose: Adropin is a secretory protein encoded by the energy homeostasis - associated (ENHO) gene. Emerging evidence indicate its role in metabolism and energy homeostasis,…
Abstract Number: 1216 • ACR Convergence 2023
“I Call It Zombie Hands and Feet, That’s Actually How I Found out I Had This Disease”: Disease Symptoms and Treatment Side Effects in a Diverse Sample of Patients with Early Limited Cutaneous and Diffuse Cutaneous Systemic Sclerosis
Chelsea Perschon¹, Emilie Jaeger¹, George Greene¹, Alain Lescoat², Yen Chen³, Susan Murphy³, Sara Shaunfield¹, David Cella¹ and Dinesh Khanna³, ¹Northwestern University Feinberg School of Medicine, Chicago, IL, ²University Rennes, Rennes, France, ³University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a multisystem autoimmune rheumatic disease with heterogeneous manifestations, including common symptoms such as pain, fatigue, dyspnea, and Raynaud's phenomenon. It…
Abstract Number: 1663 • ACR Convergence 2023
Clinically Severe Systemic Sclerosis Skin Harbors Inflammatory Fibroblasts Associated with Lymphocytes and Plasmacytoid Dendritic Cells
Kimberly Lakin¹, Robert Spiera², Yaxia Zhang¹, David Oliver¹, Aliza Bloostein¹, Hiranmayi Ravichandran³, Niroshana Anandasabapathy³, Franck Barrat¹, Jessica Gordon¹ and Dana Orange⁴, ¹Hospital for Special Surgery, New York, NY, ²Hosptial for Special Surgery, New York, NY, ³Weill Cornell Medical College, New York, NY, ⁴The Rockefeller University, New York, NY
Background/Purpose: We have previously shown that skin fibroblast activation is related to clinical severity and resolves with improvement in diffuse systemic sclerosis (dcSSc). The immune…
Abstract Number: 2356 • ACR Convergence 2023
A Macrophage-Specific Mechanism for Mycophenolate Mofetil in the Treatment of Systemic Sclerosis
Emily Morris, Rezvan Parvizi, Patricia Pioli and Michael Whitfield, Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Systemic Sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by inflammation and fibrosis. Treatment with mycophenolate mofetil (MMF), an inhibitor of lymphocyte…
Abstract Number: 2598 • ACR Convergence 2023
6 Months-follow up Data of Systemic Sclerosis Patients Treated with CD 19 Targeting CAR-T Cells
Christina Bergmann¹, Fabian Müller², Janina Auth³, Jule Taubmann⁴, Michael Aigner⁵, Dagmar Werner⁶, Andreas Wirsching⁷, Johannes Knitza⁸, Hermina Györfi⁹, Jörg Distler¹⁰, Andreas Mackensen⁵ and Georg Schett¹¹, ¹Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, ²Friedrich Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ³University Hospital Erlangen, Medicine 3, Erlangen, Germany, ⁴Department of Internal Medicine 3, Rheumatology and Immunology, Universitätsklinikum Erlangen and Friedrich-Alexander-Universität Erlangen Nürnberg, Erlangen, Germany, ⁵Department of Internal Medicine 5, Hematology and Oncology, Universitätsklinikum Erlangen and Friedrich-Alexander-Universität Erlangen Nürnberg, Erlangen, Germany, ⁶FAU Erlangen-Nürnberg, Erlangen, Germany, ⁷University Hospital Erlangen, Department of Internal Medicine 3, Erlangen, Germany, ⁸Department of Internal Medicine 3 Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg, University Hospital Erlangen, Erlangen, Germany, ⁹Clinic 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, ¹⁰Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany; Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ¹¹Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany
Background/Purpose: CD19-targeting CAR-T-cells showed remarkable improvements in autoimmune diseases including refractory lupus erythematodes (1,2) and inflammatory myopathy (3). Moreover, first data of a patient with…
Abstract Number: 0478 • ACR Convergence 2023
The Long-term Neurodevelopmental Outcome of Children Born to Women with Systemic Sclerosis: Assessment Through a Self-reported Questionnaire by the Mothers and Nneuropsychiatric Evaluations of the Children
Maria Grazia Lazzaroni¹, Eleonora Pedretti¹, Liala Moschetti¹, Marzia Bendoni², Lucrezia Visconti², Anna Molinaro², Cecilia Nalli³, Franco Franceschini³, Paolo Airò¹, Jessica Galli², Elisa Fazzi², Angela Tincani⁴ and Laura Andreoli⁵, ¹Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy, ²Unit of Child and Adolescent Neuropsychiatry, ASST Spedali Civili of Brescia, University of Brescia, Brescia, Italy, ³Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, ⁴Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, University of Brescia, Gussago, Italy, ⁵University of Brescia, Brescia, Italy
Background/Purpose: The long-term neuropsychiatric (NP) outcome of children born to mothers affected by autoimmune diseases (AIDs) represents a controversial topic, with few studies1-3 reporting a…
Abstract Number: 0627 • ACR Convergence 2023
Clinical Significance of Anti-Ro/SSA Antibodies in Patients with Systemic Sclerosis: A Study from the EUSTAR Database
Blaz Burja¹, Marouane Boubaya², Patricia Carreira³, Christina Bergmann⁴, Lidia P Ananieva⁵, Gabriela Riemekasten⁶, Okada Masado⁷, Jeska de Vries-Bouwstra⁸, Edoardo Rosato⁹, Marie-Elise Truchetet¹⁰, Nicoletta Del Papa¹¹, Antonella Marcoccia¹², FABIOLA ATZENI¹³, Tim Schmeiser¹⁴, Madelon Vonk¹⁵, Francesco Del Galdo¹⁶, Oliver Distler¹⁷ and Muriel Elhai¹⁸, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Department of Clinical Research, CHU Avicenne, APHP, Bobigny, France, ³Hospital Universitario 12 de Octubre, Madrid, Spain, ⁴Department Internal Medicine, University Hospital Erlangen, Germany, Erlangen, Germany, ⁵V.A. Nasonova Research Institute Of Rheumatology Russian Federation, Moscow, Russia, ⁶University Clinic Schleswit-Holstein (UKSH), Lübeck, Germany, ⁷St.Luke's International Hospital Immuno-Rheumatology Center, Tokyo, Japan, ⁸Leiden University Medical Center, Leiden, Netherlands, ⁹Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy, ¹⁰Bordeaux University Hospital, Bordeaux, France, ¹¹Scleroderma Clinic, UOC Reumatologia Clinica, ASST G. Pini-CTO, Milano, Italy, ¹²Centro Di Riferimento Interdisciplinare Per La Sclerosi Sistemica, Rome, Italy, ¹³Rheumatology Unit, Univerity of Messina, Messina, Italy, ¹⁴Krankenhaus St. Josef, Wuppertal-Elberfeld, Germany, ¹⁵Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, ¹⁶University of Leeds - Leeds Institute of Rheumatic and Muskuloskeletal Medicine, Leeds, United Kingdom, ¹⁷Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ¹⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland
Background/Purpose: Despite the progress in understanding the patient heterogeneity in systemic sclerosis (SSc) based on SSc-specific antibodies, better risk stratification is needed. SSc non-specific antibodies…
Abstract Number: 0649 • ACR Convergence 2023
Early Systemic Sclerosis Definitions: Time to Rethink ‘Early’ in SSc Disease?
Francesco Del Galdo¹, Matthew Woods², Mario Ehlers³ and Stefano Di Donato¹, ¹University of Leeds - Leeds Institute of Rheumatic and Muskuloskeletal Medicine, Leeds, United Kingdom, ²Lumanity, Manchester, United Kingdom, ³Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT
Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune fibrotic disease with highly variable type and severity of organ involvement. Pathological changes in the skin, lungs,…
Abstract Number: 0671 • ACR Convergence 2023
Decreased Odds of Malignancy with Mycophenolate Use in a Single-Center Scleroderma Cohort
Jin Feng¹, Chrisanna Dobrowolski², Celestine He², Hannah Verma³, Roshan Vasoya³, Daniel Qian³, Ezequiel olumuyide³, Alicia Leong³, Joseph Menand¹, Murilo Roberto Bastos Silva¹, Vincent Courant¹ and Sophia Lutgen¹, ¹Icahn School of Medicine at Mount Sinai Morningside and West, New York, NY, ²Icahn School of Medicine at Mount Sinai Hospital, New York, NY, ³Icahn School of Medicine at Mount Sinai, New York, NY
Background/Purpose: Prior studies have shown an increased risk of malignancy in patients with systemic sclerosis (SSc). The reasons for this increased risk may be multifactorial,…
Abstract Number: 0944 • ACR Convergence 2023
Macrophages Regulate Adipocyte Differentiation and Proliferation in Skin Fibrosis
Chanhyuk Park, Helen Jarnagin, Michael Whitfield and Patricia Pioli, Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Autoimmune systemic sclerosis (SSc) is characterized by inflammation, vasculopathy, and dermal and internal organ fibrosis. A widely-reported but poorly understood aspect of SSc skin…
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…

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