Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 2473 • ACR Convergence 2024
Criterion Validity of Modified Rodnan Skin Score: Does It Capture Skin Thickness or Hardening? Results of a Large Skin Histology Study
Shervin Assassi¹, Ruhani Desai², Jeffrey Browning³, Samuel Theodore¹, Meng Zhang¹, Brian Skaug¹, Jerry Alonso¹, Zsuzsanna McMahan¹, Maureen Mayes¹ and Minghua Wu¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²UTHealth Houston Division of Rheumatology, DeLand, FL, ³Boston University, Cambridge, MA
Background/Purpose: Modified Rodnan Skin Score is a widely used outcome measure for skin involvement in systemic sclerosis (SSc). Its criterion validity (comparison to the gold…
Abstract Number: 0702 • ACR Convergence 2024
AISA 021, a Novel Calcium Channel Antagonist in Development for Raynaud’s & Systemic Sclerosis, Has Antagonistic Activity at Sodium Channel Targets for Pain Relief and Treats Scleroderma Pain Better Than Current Calcium Channel Blockers in a Phase 2A Study
Andrew Sternlicht¹ and Meredith Todd², ¹Tufts University Medical Center and Aisa Pharma, Inc., Boston, MA, ²Aisa Pharma Pty Ltd., East Lindfield, New South Wales, Australia
Background/Purpose: Improved treatments for Raynaud's phenomenon in Systemic Sclerosis (SSc) are needed. A novel oral calcium channel blocker (cilnidipine), with N-type channel antagonism is being…
Abstract Number: 1591 • ACR Convergence 2024
Domains and Outcome Measures for the Assessment of Digital Vasculopathy and Raynaud Phenomenon in Juvenile Systemic Sclerosis: A Systematic Review
Valerio maniscalco¹, Simone Appenzeller², Jennifer lemon³, Lucy stead⁴, Natalia Vasquez Canizares⁵, Gabriele Simonini⁶, Suzanne Li⁷ and Clare Pain⁸, and IJOG study, ¹Santo Stefano Hospital; Meyer Children Hospital IRCCS, Firenze, Toscana, Italy, ²Unicamp, Campinas, SP, Brazil, ³Alder Hey Children's NHS Foundation Trust, Liverpool, ⁴Liverpool University, Liverpool, ⁵Children's Hospital at Montefiore; Albert Einstein College of Medicine;, New York, NY, ⁶Meyer Children Hospital IRCCS; NEUROFARBA Department, University of Florence, Florence, Italy, ⁷Hackensack University Medical Center, Hackensack, ⁸Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom
Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease that primary affect the vascular system determining multiple organ damage and reducing quality of life…
Abstract Number: 2477 • ACR Convergence 2024
Quantifying Tissue Microstructural Changes in Scleroderma Skin Using a Novel, Point-of-care, High-frequency-ultrasound Instrument
Kimberly Lakin¹, Cameron Hoerig², Jessica Gordon³, Elmira Ghahramani², Mia Diaz¹, Robert Spiera⁴, dana Orange⁵ and Jonathan Mamou², ¹Hospital for Special Surgery, New York, NY, ²Weill Cornell Medical College, New York, NY, ³Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁴Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, ⁵Rockefeller University, New York, NY
Background/Purpose: A highly reproducible, quantitative method of measuring skin severity in systemic sclerosis (SSc) trials and patient care is needed. While conventional skin US, operating…
Abstract Number: 0706 • ACR Convergence 2024
Single Center Prospective Cohort of Systemic Sclerosis Patients Who Are At-Risk for Pulmonary Hypertension
Carleigh Zahn¹, Scott Visovatti², Rosemary Gedert¹, Suiyuan Huang¹, Victor Moles¹, Amber Young¹, Vallerie McLaughlin¹ and Dinesh Khanna¹, ¹University of Michigan, Ann Arbor, MI, ²The Ohio State University, Columbus
Background/Purpose: Pulmonary hypertension (PH) is a leading cause of mortality in systemic sclerosis (SSc). We launched an IRB approved prospective study (NOVEL) in 2013 at…
Abstract Number: 1691 • ACR Convergence 2024
Treatment Response Biomarkers for Systemic Sclerosis-Associated Interstitial Lung Disease
Elizabeth Volkmann¹, Holly Wilhalme², Donald Tashkin², Grace Kim², Jonathan Goldin², Alana Haussmann², Masataka Kuwana³, Michael Roth² and Shervin Assassi⁴, ¹University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles, ²UCLA, Los Angeles, ³Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan, Tokyo, Japan, ⁴UTHealth Houston Division of Rheumatology, Houston, TX
Background/Purpose: Despite the expanding treatment landscape for systemic sclerosis-associated interstitial lung disease (SSc-ILD), the lack of early and valid treatment response biomarkers (measurable in the…
Abstract Number: 2641 • ACR Convergence 2024
Identification of Systemic Sclerosis Intrinsic Subtypes in the ASSET Clinical Trial Using PBC Gene Expression
Zhiyun Gong¹, Timothy Sullivan², Tammara Wood³, David Fox⁴, Dinesh Khanna⁵ and Michael Whitfield⁶, ¹Dartmouth College, Lebanon, NH, ²Geisel School of Medicine at Dartmouth, Lebanon, NH, ³Dartmouth, Hanover, NH, ⁴University of Michigan, Dexter, MI, ⁵University of Michigan, Ann Arbor, MI, ⁶Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Systemic sclerosis (SSc) is molecularly heterogeneous and distinct subtypes of patients have been identified based on gene expression in skin. Although treatments have improved,…
Abstract Number: 0708 • ACR Convergence 2024
Outcomes of Intermittent Intravenous Steroid Use During Infusion Therapy in Systemic Sclerosis
Vangala Adarsh¹, dihowm Fatma¹, Eyerusalem Akpan², Meredith Keller², Maheswari Muruganandam², N. Suzanne Emil², Frank O'Sullivan³, Roderick Fields² and wilmer sibbitt¹, ¹UNM, Albuquerque, NM, ²University of New Mexico, Albuquerque, NM, ³University of New Mexico School of Medicine, Albuquerque, NM
Background/Purpose: Use of high dose corticosteroids in systemic sclerosis (SSc) is considered a significant risk factor for development of scleroderma renal crisis (SRC). Intravenous (IV)…
Abstract Number: 1829 • ACR Convergence 2024
Genomic Analysis of Skin Biopsies Differentiates Major Anti-Nuclear Autoantibody Subsets in Limited Cutaneous Systemic Sclerosis
Philip Yee¹, Medha Kanitkar², Kristina Clark³, Voon Ong¹ and Christopher Denton⁴, ¹University College London, London, England, United Kingdom, ²University College London, London, United Kingdom, ³University of Oxford, Oxford, United Kingdom, ⁴University College London, Northwood, United Kingdom
Background/Purpose: Systemic sclerosis (SSc) displays significant heterogeneity. Those with limited cutaneous systemic sclerosis (lcSSc) experience significant systemic organ involvement similar to those with diffuse cutaneous…
Abstract Number: 2644 • ACR Convergence 2024
Baseline Fibroblast Immunophenotype Predicts Clinical Improvement Among Individuals with Early Diffuse Cutaneous Systemic Sclerosis
Kimberly Lakin¹, Robert Spiera², John Spivack¹, Yaxia Zhang¹, Jessica Gordon³ and dana Orange⁴, ¹Hospital for Special Surgery, New York, NY, ²Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, ³Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁴Rockefeller University, New York, NY
Background/Purpose: We have previously shown that fibroblast CD34 is low and alpha-smooth muscle actin (aSMA) is high in severe diffuse cutaneous systemic sclerosis (dcSSc) skin,…
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…

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