Abstracts tagged "Scleroderma, Systemic"

Abstract Number: L01 • ACR Convergence 2024
Targeted Exosite Inhibition of STING Activation of TBK1 Selectively Blocks Type I Interferon and NFκB Responses for Treatment of Autoimmune Diseases
Matthew Martin¹, Erik Wilker¹, Diana Gikunju¹, Usha Narayanan¹, Unnati Pandya¹, Vijetha Prakash¹, Ashley Edwards¹, Sameer Kawatkar¹, Tenghui Chen¹, Ragunath Chandran¹, Sai Sunder¹, Sumathi Biradar¹, Joerg Distler², Alexandra Joseph¹, Stephanos Ioannidis¹ and Bhavatarini Vangamudi¹, ¹Exo Therapeutics, Cambridge, MA, ²Exo Therapeutics, Düsseldorf, Germany
Background/Purpose: The cGAS-TBK1-STING pathway senses nucleic acids for innate immunity. Aberrant activation of the pathway is linked to autoimmune diseases including Systemic and Cutaneous Lupus…
Abstract Number: 0961 • ACR Convergence 2024
Multi-omics Study of Systemic Sclerosis Immunoglobulins G Effects on Endothelial Cells: A Distinct Profile in Anti-Topoisomerase I Positive Patients
Aurélien Chepy¹, Solange Vivier², Abderrahmane Elhannani², Fabrice Bray³, Clément Chauvet⁴, Martin Figeac⁵, Lucile Guilbert⁴, Eric HACHULLA⁶, Christian Rolando³, Sylvain Dubucquoi⁴, David Launay⁴ and Vincent sobanski⁴, ¹Univ. Lille, Inserm, CHU Lille, U1286—INFINITE—Institute for Translational Research in Inflammation, Lille, France., Lille, Nord-Pas-de-Calais, France, ²Univ. Lille, Inserm, CHU Lille, U1286—INFINITE—Institute for Translational Research in Inflammation, Lille, France., Lille, France, ³Univ. Lille, CNRS, USR 3290 - MSAP - Miniaturisation pour la Synthèse l’Analyse et la Protéomique, F-59000 Lille, France, Lille, France, ⁴Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, F-59000 Lille, France., Lille, France, ⁵Univ. Lille, CNRS, Inserm, CHU Lille, Institut Pasteur de Lille, US 41 - UAR 2014 - PLBS, F-59000 Lille, France, Lille, France, ⁶CHU 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., LILLE, France
Background/Purpose: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease characterized by autoimmunity, fibrosis and vasculopathy. Antinuclear antibodies (ANA) are strong diagnostic and prognosis biomarkers…
Abstract Number: 1976 • ACR Convergence 2024
Artificial Intelligence Analysis of HRCT Images Reflects Pulmonary Involvement in Systemic Sclerosis Interstitial Lung Disease
Enrico De Lorenzis¹, Lucy Elizabeth Thornton², Stefano Di Donato³, Marco Minerba³, Lesley-Anne Bissell⁴, Riccardo Bixio⁵, Anand Devaraj⁶, Peter George⁷ and Francesco Del Galdo⁸, ¹Catholic University of the Sacred Heart, Roma, Rome, Italy, ²1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, ³1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, United Kingdom, ⁴Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁵University of Verona, Verona, Verona, Italy, ⁶Department of Radiology, Royal Brompton Hospital, London, London, United Kingdom, ⁷Department of Interstitial Lung Disease, Royal Brompton Hospital London, London, United Kingdom, ⁸University of Leeds, Leeds, United Kingdom
Background/Purpose: Interstitial lung disease (ILD) is the leading cause of mortality and morbidity in systemic sclerosis (SSc) patients. Current functional, imaging, and clinical measures of…
Abstract Number: 2645 • ACR Convergence 2024
Human NCF1-p.R90H Variant Promotes Pulmonary Fibrosis in the Bleomycin-induced Mouse Model and Systemic Sclerosis Patients via Expansion of SPP1+Monocytes-derived Macrophages
Xinran Yuan¹, Xiaodong Qin², Kenji Takemoto¹, Jian Zhao¹, Matthew Sanderson¹, Xue Xu¹, Kristi L Helke³, Bethany Wolf⁴, Joel Guthridge⁵, Judith A James⁶, Xiaodong Zhou⁷, Shervin Assassi⁸, Carol Feghali-Bostwick⁴, Dandan Wang⁹, Lingyun Sun¹⁰ and Betty P Tsao¹, ¹Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, Charleston, SC, ²Department of Orthopedic Surgery, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, Jiangsu, China (People's Republic), ³Department of Comparative Medicine, Medical University of South Carolina, Charleston, SC, ⁴Medical University of South Carolina, Charleston, SC, ⁵Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁶Arthritis and Clinical Immunology, Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁷Division of Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, ⁸UTHealth Houston Division of Rheumatology, Houston, TX, ⁹Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical Schoo, Nanjing, Jiangsu, China (People's Republic), ¹⁰Department of Rheumatology and Immunology, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, Jiangsu, China (People's Republic)
Background/Purpose: We previously identified a systemic lupus erythematosus (SLE) causal risk variant, p.Arg90His (p.R90H, rs201802880) substitution encoded in neutrophil cytosolic factor 1 (NCF1), an essential component…
Abstract Number: 0967 • ACR Convergence 2024
Transcriptional Heterogeneity of Immune Cells in the Esophagus of Systemic Sclerosis Patients: A Comparison of Upper and Lower Esophageal Regions
Hadijat Makinde¹, Miranda Gurra¹, Salina Dominguez², Matthew Dapas², Tyler Therron², Kathleen Aren³, Marie-Pier Tetreault², Monique Hinchcliff⁴, Deborah Winter⁵ and Harris Perlman¹, ¹Northwestern University, Chicago, IL, ²Northwestern University, Chicago, ³Northwestern University Division of Rheumatology, Chicago, IL, ⁴Yale School of Medicine, Westport, CT, ⁵Northwestern University, Skokie, IL
Background/Purpose: Systemic sclerosis (SSc) is characterized by an initial inflammatory phase followed by fibrosis. Esophageal dysfunction in SSc is associated with gastroesophageal reflux, leading to…
Abstract Number: 2196 • ACR Convergence 2024
Identifying Specific Criteria for Juvenile Systemic Sclerosis: A Comparison of Adult and Pediatric Ratings
Suzanne Li¹, Natalia Vasquez-Canizares², Clare Pain³, Marinka Twilt⁴, Amra Adrovic⁵, Abdulrahman Alrasheed⁶, Simone Appenzeller⁷, ADELE CIVINO⁸, narendra bagri⁹, Patricia Costa Reis¹⁰, Muserref Cucueoglu¹¹, Fatma Dedeoglu¹², samundeeswari Deepak¹³, Jianghong Deng¹⁴, Dalia El-Ghoneimy¹⁵, Ivan Foeldvari¹⁶, Fernando Garcia-Rodriguez¹⁷, Marija Jelusic¹⁸, Ankur Jindal¹⁹, Ozgur Kasapcopur²⁰, Maria Katsicas²¹, Archana Khan²², Raju Khubchandani²³, Meiping Lu²⁴, Hanna Lythgoe²⁵, edoardo marrani²⁶, Giorgia Martini²⁷, Takako Miyamae²⁸, Tomo Nozawa²⁹, Seza Ozen³⁰, Lidia Rutkowska-Sak³¹, Sunil Sampath¹³, Dieneke Schonenberg-Meinema³², Hongmei Song³³, Betul Sozeri³⁴, Hayakazu Sumida³⁵, Maria Teresa Terreri³⁶, Kathryn Torok³⁷, Seyma Turkmen³⁸, Dilara Unal³⁸, Gong Yinv³⁹, Emily Willis²⁵, Rongjun Zheng²⁴ and Brian Feldman⁴⁰, and Pediatric International Consortium for Scleroderma, ¹Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ, ²Children's Hospital at Montefiore, Bronx, NY, ³Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, ⁴Alberta Children's Hospital, Calgary, AB, Canada, ⁵Koc University Hospital, Istanbul, Turkey, ⁶King Abdullah Specialized Children Hospital, Riyadh, Saudi Arabia, ⁷Unicamp, Campinas, SP, Brazil, ⁸Pediatric Rheumatology "Vito Fazzi" Hospital LECCE, LECCE, Italy, ⁹All India Institute of Medical Sciences, New Delhi, Delhi, India, ¹⁰Hospital de Santa Maria, Lisbon, Portugal, ¹¹Hacettepe University, Ankara, Turkey, ¹²Boston Children's Hospital, Boston, MA, ¹³THE NEWCASTLE UPON TYNE HOSPITALS NHS FOUNDATION TRUST, Newcastle upon Tyne, United Kingdom, ¹⁴Beijing Children's Hospital, Beijing, China, ¹⁵Children's Hospital, Ain Shams University, Cairo, Egypt, ¹⁶Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹⁷Universidad Autonoma de Nuevo Leon, Monterrey, NL, Mexico, ¹⁸University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ¹⁹Postgraduate Institute of Medical Education and Research, Chandigarh, India, ²⁰Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ²¹Hospital Garrahan, Buenos Aires, Argentina, ²²SRCC Children's Hospital, Mumbai, India, Mumbai, India, ²³The SRCC Children's Hospital, Mumbai, India, ²⁴Department of Rheumatology Immunology and Allergy, Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China (People's Republic), ²⁵Royal Manchester Children's Hospital, Manchester, United Kingdom, ²⁶Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, ²⁷University of Udine University Hospital Santa Maria della Misericordia, Udine, Italy, ²⁸Tokyo Women's Medical University, Tokyo, Japan, ²⁹Yokohama City University, Yokohama, Japan, ³⁰Department of Pediatrics, Hacettepe University, Ankara, Turkey, ³¹National Institute of Geriatrics, Rheumatology and Rehabilitation, Warszawa, Poland, ³²Amsterdam University Medical Center, Amsterdam, Netherlands, ³³Peking Union Medical College Hospital, Beijing, China, ³⁴University of Health Sciences, Istanbul, Turkey, ³⁵The University of Tokyo, Tokyo, Japan, ³⁶UNIFESP, São Paulo, SP, Brazil, ³⁷Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ³⁸University of Health Sciences, Umraniye Training and Research Hospital, Ankara, Turkey, ³⁹Children's Hospital Affiliated to Fudan University, Shanghai, China (People's Republic), ⁴⁰Division of Rheumatology, The Hospital for Sick Children; Departments of Pediatrics and Medicine, Faculty of Medicine; The Institute for Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada
Background/Purpose: Despite the high morbidity and mortality risk associated with juvenile systemic sclerosis (jSSc), evidence to guide management is limited. No jSSc clinical trials have…
Abstract Number: 2654 • ACR Convergence 2024
Adapting and Testing a Cognitive Rehabilitation Intervention Tailored for People with Systemic Sclerosis: Acceptability, Feasibility, & Impact on Health-related Outcomes
Yen Chen¹, Mary Alore², Dinesh Khanna¹ and Susan Murphy³, ¹University of Michigan, Ann Arbor, MI, ²Self-employed, TROY, MI, ³University of Michigan, Plymouth, MI
Background/Purpose: Cognitive dysfunction affects up to 87% of individuals with systemic sclerosis (SSc), often significantly impairing their work and daily activities and exacerbating other symptoms…
Abstract Number: 1070 • ACR Convergence 2024
Evaluating & Improving Adherence to Pulmonary Hypertension Screening in Systemic Sclerosis Patients
John Hickernell¹ and Zineb Aouhab², ¹Loyola Univeristy Medical Center, Chicago, IL, ²Loyola University Medical Center, Oak Brook, IL
Background/Purpose: Annual screening for pulmonary hypertension (PH) in patients with Systemic Sclerosis (SSc) has been shown to decrease mortality. The American College of Radiology, European…
Abstract Number: 2200 • ACR Convergence 2024
Whole Blood Transcriptome Profiling in Juvenile Systemic Sclerosis Patients Reveals Active Immune Upregulation and Enhanced Fibrotic Signature
Rania Elbakri¹, Amanda Robinson², Anwesha Sanyal¹ and Kathryn Torok³, ¹University of Pittsburgh, pittsburgh, PA, ²University of Utah Health, Salt Lake City, UT, ³Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA
Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare chronic autoimmune disorder characterized by skin thickening and multisystem organ involvement, leading to significant morbidity. The pathogenesis…
Abstract Number: 2685 • ACR Convergence 2024
Association Between Gastrointestinal Bacterial Species and Radiological Features of Systemic Sclerosis-Interstitial Lung Disease (SSc-ILD): A Multicenter Study from the SSc Microbiome Consortium Project
Arissa Young¹, Kristofer Andreasson², Swapna Joshi³, Jen Labus³, Andrea Low Hsiu Ling⁴, vanessa smith⁵, Zsuzsanna McMahan⁶, Antonia Valenzuela Vergara⁷, Susanna Proudman⁸, Grace Kim³, Gracijela Bozovic², Jonathan Goldin³, Ezinne Aja³, Jonathan Jacobs³ and Elizabeth Volkmann⁹, ¹UCLA, Los Angeles, CA, ²Lund University, Lund, Sweden, ³UCLA, Los Angeles, ⁴3Singapore General Hospital, Duke-NUS, Singapore, Singapore, ⁵Ghent University Hospital, Gent, Belgium, ⁶UTHealth Houston Division of Rheumatology, Houston, TX, ⁷Pontificia Universidad Catolica de Chile, Santiago, Chile, ⁸Royal Adelaide Hospital, Adelaide, Australia, ⁹University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Variations in gastrointestinal (GI) microbial communities may influence the development of lung diseases. Patients with SSc-ILD have a unique intestinal microbial signature. To further…
Abstract Number: 1316 • ACR Convergence 2024
Dual Therapy with a JAKi and bDMARD in Patients with Standard Treatment Resistant Rheumatic Disease: A Case Series
Bella Garg¹, John Antowan² and daniel Furst³, ¹Centinela Hospital Medical Center, Inglewood, CA, ²UCLA, Los Angeles, ³Pacific Arthritis Center, Inglewood
Background/Purpose: Due to concerns about side effects, combined JAK inhibitors (JAKi) and biologic DMARDs (bDMARDs) have not been recommended for rheumatologic conditions. However, combining them…
Abstract Number: 2442 • ACR Convergence 2024
Clustering Algorithm as an Exploratory Analysis of Different Clinical Phenotypes in Systemic Sclerosis. Data from the PRECISESADS Study
Santiago Dans Caballero¹, Clementina López Medina², Chary Lopez-Pedrera³, Carlos Perez-Sanchez⁴, Alejandro Escudero-Contreras⁵ and Rafaela Ortega-Castro⁶, ¹Reina Sofia University Hospital, Lebrija, Andalucia, Spain, ²Reina Sofia University Hospital, Cordoba, Spain, ³IMIBIC/Reina Sofia Hospital/University of Cordoba, Cordoba, Andalucia, Spain, ⁴IMIBIC/Reina Sofia Hospital/University of Cordoba, Cordoba, Spain, ⁵Reina Sofia University Hospital, Córdoba, Spain, ⁶Hospital Reina Sofía, Cordoba, Andalucia, Spain
Background/Purpose: Systemic sclerosis is a systemic autoimmune disease with multiorgan involvement. Its pathophysiology is based on three pillars: vasculopathy, fibrosis, and autoimmunity. Despite advances in…
Abstract Number: 2687 • ACR Convergence 2024
Therapeutic Choices in Systemic Sclerosis Associated Interstitial Lung Disease, a Survey of International Experts
Michael Macklin¹, Iazsmin Bauer Ventura¹ and Dinesh Khanna², ¹University of Chicago, Chicago, IL, ²University of Michigan, Ann Arbor, MI
Background/Purpose: The therapeutic options for systemic sclerosis-associated interstitial lung disease (SSc-ILD) have evolved rapidly, with recent guidelines released by the ACR and American Thoracic Society…
Abstract Number: 1320 • ACR Convergence 2024
Acceptability and Satisfaction of an Online Resilience-Building Intervention with Peer Health Coaches for Managing Fatigue and Well-Being in Systemic Sclerosis
Susan Murphy¹, Yen Chen², Alexandra Harper², Nirali Shah², Mary Alore², Sheri Hicks², Adam Pape³ and Dinesh Khanna², ¹University of Michigan, Plymouth, MI, ²University of Michigan, Ann Arbor, MI, ³University of Michigan, University of Michigan, MI
Background/Purpose: Non-pharmacological fatigue management interventions in systemic sclerosis (SSc) are limited, despite people with SSc reporting that fatigue is inadequately addressed with clinical care. We…
Abstract Number: 2448 • ACR Convergence 2024
Content Validity and Sensitivity to Change of ScleroID Through the Natural History of Scleroderma. a Validated Tool to Measure the Impact of SSc on How Patients Feel and Function
Seda Colak¹, Stefano Di Donato², Riccardo Bixio³, Lesley-Anne Bissell⁴, Theresa Barnes⁵, Christopher Denton⁶, Muhammad Nisar⁷ and Francesco Del Galdo⁸, ¹Gulhane Training and Research Hospital, Ankara, Turkey, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ³University of Verona, Verona, Verona, Italy, ⁴Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁵Countess of Chester, Liverpool, United Kingdom, ⁶University College London, Northwood, United Kingdom, ⁷Bedfordshire hospitals, BEDFORD, United Kingdom, ⁸University of Leeds, Leeds, United Kingdom
Background/Purpose: Multiple patient-reported outcomes (PROs) have been utilized to capture the burden of Systemic Sclerosis (SSc), including the Health Assessment Questionnaire Disability Index (HAQ-DI), Cochin…

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