Abstracts tagged "Systemic sclerosis"

Abstract Number: 0785 • ACR Convergence 2024
Exploring the Link Between Atgl-Dependent Lipolysis and Dermal Fibrosis in Systemic Sclerosis
Elizabeth Caves¹, Agrani Dixit¹, Anna Jussila², Vivian Lei³, Hailey Edelman⁴, Muhammad Hamdan⁵, Ian Odell⁵, Monique Hinchcliff⁶, Radhika Atit⁷ and Valerie Horsley¹, ¹Yale School of Medicine, New Haven, CT, ²Stanford Medicine, Stanford, CA, ³University of North Carolina School of Medicine, Chapel Hill, ⁴Vanderbilt School of Engineering, New Haven, ⁵Yale School of Medicine, New Haven, ⁶Yale School of Medicine, Westport, CT, ⁷Case Western Reserve University College of Arts and Sciences, Cleveland
Background/Purpose: Resident lipid-filled dermal adipocytes are depleted in both systemic sclerosis (SSc) and scleroderma mouse models, but mechanisms are poorly understood. We undertook studies in mouse…
Abstract Number: 0969 • ACR Convergence 2024
Role and Mechanism of the Wnt3a-FZD5 Pathway in Regulating the Transformed Intermediate State of Alveolar Epithelial Cells Involved in Pulmonary Fibrosis in Systemic Sclerosis
Hang Yu¹ and Jing Xue², ¹zhejiang university, Hangzhou, China (People's Republic), ²The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China (People's Republic)
Background/Purpose: Systemic sclerosis (SSc) is a diffuse connective tissue disease associated with multi-system and multi-organ tissue damage. The transformation of different precursor cells to myofibroblasts…
Abstract Number: 1573 • ACR Convergence 2024
Early Scleroderma with Non-Raynaud’s Symptoms Prior to Raynaud’s Onset Is Associated with Rapid Progression to Diffuse Skin Disease and Joint Contractures
Iqtidar Hanif¹, Shervin Assassi¹, Maureen Mayes¹, Meng Zhang¹, Julio Charles¹, John VanBuren², Jessica Alvey², Kimia Ghaffari², Elana Bernstein³, Flavia Castelino⁴, Lorinda Chung⁵, Luke Evnin⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Laura Hummers¹⁰, Dinesh Khanna¹¹, Kimberly Lakin¹², Dorota Lebiedz-Odrobina¹³, Yiming Luo¹⁴, Ashima Makol¹⁵, Jerry Molitor¹⁶, Duncan Moore¹⁷, Carrie Richardson¹⁸, Nora Sandorfi¹⁹, Ami Shah²⁰, Ankoor Shah²¹, Victoria Shanmugam²², Virginia Steen²³, Elizabeth Volkmann²⁴, Carleigh Zahn¹¹ and Brian Skaug¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²University of Utah, Salt Lake City, UT, ³Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁴Massachusetts General Hospital, Boston, MA, ⁵Stanford University, Woodside, CA, ⁶Scleroderma Research Foundation, Brisbane, CA, ⁷Vanderbilt University Medical Center, Nashville, TN, ⁸Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹¹University of Michigan, Ann Arbor, MI, ¹²Hospital for Special Surgery, New York, NY, ¹³University of Utah, Cottonwood Heights, UT, ¹⁴Columbia University, New York, NY, ¹⁵Mayo Clinic, Rochester, MN, Rochester, MN, ¹⁶University of Minnesota, Minneapolis, MN, ¹⁷Northwestern Memorial Hospital, Chicago, IL, ¹⁸Northwestern University, Chicago, IL, ¹⁹University of Pennsylvania, Philadelphia, PA, ²⁰Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ²¹Duke University, Durham, NC, ²²Office of Autoimmune Disease Research, Office of Research on Women's Health, National Institutes of Health, Great Falls, VA, ²³Georgetown University School of Medicine, Washington, DC, ²⁴University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Raynaud’s phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc). RP alerts clinicians to the possibility of an autoimmune rheumatic disease,…
Abstract Number: 1750 • ACR Convergence 2024
Safety and Preliminary Efficacy of CD19 CAR-T Cell Treatment in Rheumatic Disease – Data from the First Part of the Phase I/II CASTLE Basket Study
Georg Schett¹, Fabian Müller², Melanie Hagen³, Andreas Wirsching³, Daniela Bohr⁴, Christina Bergmann⁵, Carlo Tur³, Simon Völkl⁶, Michael Aigner⁷, Sascha Kretschmann⁷, Silvia Spoerl⁷, Soraya Kharboutli⁷, Ingrid Vasova⁷, Daniel Aletaha⁸, Hans Kiener⁹, Gerlando Natalello¹⁰, Franco Locatelli¹¹, Maria Antonietta D´Agostino¹², Aline Bozec³, Ricardo Grieshaber-Bouyer¹³ and Andreas MAckensen¹⁴, ¹Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ²Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Germany, ³Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ⁴Department of Internal Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Deutsches Zentrum für Immuntherapie (DZI), Friedrich Alexander University Erlangen-Nuremberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ⁵Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁶Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Bayern, Germany, ⁷Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁸Department of Medicine III, Division of Rheumatology, Medical University of Vienna, Wien, Austria, ⁹Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria, Vienna, Austria, ¹⁰Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ¹¹IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ¹²Catholic University of Sacred Heart, Rome, Italy, Rome, Italy, ¹³Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, ¹⁴Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Bayern, Germany
Background/Purpose: Systemic autoimmune diseases are based on an aberrant activation of B cells. Autologous CD19 chimeric antigen receptor (CAR) T cells allow deep depletion of…
Abstract Number: 1834 • ACR Convergence 2024
Altered Mechanotransduction via Myosin II Contributes to Collagen and IL-6 Production in Systemic Sclerosis Skin
Barbara Russo¹, Shutova Maria², Fanny Noulet², Geroge-Radu Romanescu³, Nicolo brembilla² and Wolf-Henning Boehncke², ¹Hopitaux Universitaire de Geneva, Geneva, Geneve, Switzerland, ²Hôpitaux Universitaires des Genève, Geneva, Geneve, Switzerland, ³University of Geneva, Geneva, Geneve, Switzerland
Background/Purpose: Scleroderma (SSc), an autoimmune disease, features progressive fibrosis, leading to significant morbidity and mortality. Current therapies manage symptoms but lack efficacy in directly targeting…
Abstract Number: 2455 • ACR Convergence 2024
Improvement Across Multi-organ Domains and Patient Reported Outcomes in Refractory Juvenile-Onset Systemic Sclerosis (jSSc) up to 4 Years After Autologous Stem Cell Transplantation (ASCT)
Jonathan Li¹, Paulina Horvei², Franziksa Rosser³, Kirsten Rose-Felker⁴, Vibha Sood⁵, Adam Olson⁶, Vickie Vandergrift⁷, Nicole Hogue², Lauren Farver⁸, Devin Mcguire⁹, Haley Havrilla⁷, Jessie Alexander¹⁰, Shawna McIntyre², Paul Szabolcs² and Kathryn Torok¹¹, ¹Division of Rheumatology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ²Division of Blood and Marrow Transplantation and Cellular Therapies, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ³Division of Pulmonology Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Division of Cardiology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁵Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁶Department of Radiation Oncology, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁷Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁸Department of Physical Therapy, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁹Pediatric Behavioral Health, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ¹⁰Pediatric Bone Marrow Transplant and Cellular Therapies, Stanford Children's Hospital, Palo Alto, CA, USA, Pittsburgh, ¹¹Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is a systemic autoimmune disease characterized by vasculopathy and multiorgan fibrosis leading to significant morbidity and early mortality. Autologous stem…
Abstract Number: 2643 • ACR Convergence 2024
Integrated Bulk and Single Cell RNA Sequencing Defines Key Pathways Regulating Myofibroblast Differentiation Across ANA Subgroups in Diffuse Systemic Sclerosis
Kristina Clark¹, Corrado Campochiaro², Emma Derrett-Smith³, Voon Ong⁴, Christopher Buckley⁵ and Christopher Denton⁶, ¹University of Oxford, Oxford, United Kingdom, ²IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milan, Milan, Italy, ³University College London Division of Medicine, Birmingham, United Kingdom, ⁴University College London, London, England, United Kingdom, ⁵Kennedy Institute of Rheumatology, University of Oxford, Oxford, United Kingdom, ⁶University College London, Northwood, United Kingdom
Background/Purpose: Myofibroblasts are key cells in the pathogenesis of systemic sclerosis (SSc). TGFβ is a key growth factor driving myofibroblast formation in SSc. The main…
Abstract Number: 0687 • ACR Convergence 2024
Patterns of Disease Progression in Early Systemic Sclerosis Patients with Interstitial Lung Disease
Lorenzo Beretta¹, Alessandro Santaniello², Silvia Bosello³, Enrico De Lorenzis⁴, Gerlando Natalello⁵, Nicoletta Del Papa⁶, Silvia Cavalli⁷, Devis Benfaremo⁸, Giacomo De Luca⁹, Corrado Campochiaro¹⁰, Lorenzo Cavagna¹¹, Veronica Codullo¹², Francesco Bonomi¹³, gaia Montanelli¹⁴, Adriana Severino¹⁵, Monica Caronni², Barbara Vigone², Silvia Bellando-Randone¹⁶, Carlomaurizio Montecucco¹⁷, Marco Matucci-Cerinic¹⁸, Lorenzo Dagna¹⁹, Gianluca Moroncini⁸, Roberto Caporali²⁰ and Chiara Bellocchi²¹, ¹Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico di MIlano, Milan, Milan, Italy, ²Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, MILANO, Italy, ³FONDAZIONE POLICLINICO UNIVERSITARIO A GEMELLI- IRCCS, Rome, Rome, Italy, ⁴Catholic University of the Sacred Heart, Roma, Rome, Italy, ⁵Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ⁶ASST Centro Specialistico Ortopedico Traumatologico Gaetano Pini-CTO, Milano, Milan, Italy, ⁷University of Milan, Milan, Milan, Italy, ⁸Marche Polytechnic University, Ancona, Italy, ⁹Vita-Salute San Raffaele University & IRCCS San Raffaele Hospital, Milan, Milan, Italy, ¹⁰IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milan, Milan, Italy, ¹¹University of Pavia and Fondazione IRCCS Policlinico San Matteo Hospital of Pavia, Pavia, Pavia, Italy, ¹²Division of Rheumatology - Policlinico San Matteo, Pavia, Italy, Pavia, Lombardia, Italy, ¹³Azienda Ospedaliera Universitaria Careggi, Florence, Florence, Italy, ¹⁴Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, Milan, Italy, ¹⁵Fondazione IRCCS Ca' Granda Ospedale Policlinico di Milano, Milan, Milan, Italy, ¹⁶University of Florence, Florence, Florence, Italy, ¹⁷IRCCS policlinico S. Matteo foundation, University of Pavia, Pavia, Italy, ¹⁸University San Raffaele Milano, Milano, Milan, Italy, ¹⁹Ospedale San Raffaele, Milano, Italy, ²⁰Department of Clinical Sciences and Community Health, University of Milan, and Department of Rheumatology and Medical Sciences, ASST Gaetano Pini-CTO, Milan, Italy, ²¹Università degli Studi di Milano, IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy
Background/Purpose: Understanding the trajectory of disease progression in SSc-ILD is crucial for effective patient management and prognostication. Trajectory modeling offers a novel approach to deciphering…
Abstract Number: 0786 • ACR Convergence 2024
First Analyses of Transcriptomic Changes in the Skin of SSc Patients upon CD19-targeting CAR T Cell Therapy
Janina Auth¹, Manoj Kumar Selvaraju², Fabian Müller³, Pooja Gupta², Arif Ekici⁴, Carlo Tur¹, Maria Gabriella Raimondo¹, Jörg Distler⁵, Clara Dees⁶, Sara Chenguiti⁶, Melanie Hagen¹, Andreas Wirsching¹, Jule Taubmann⁷, Soraya Kharboutli⁸, Silvia Spoerl⁸, Michael Aigner⁸, Sascha Kretschmann⁸, Ingrid Vasova⁸, Andreas MAckensen⁹, Georg Schett¹⁰ and Christina Bergmann¹¹, ¹Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ²CUBiDA Department (Core Unit Bioinformatics and Data Analysis), UKER and FAU, Erlangen, Germany, ³Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Germany, ⁴Core Unit Next Generation Sequencing, Erlangen, Germany, ⁵Universitätsklinikum Düsseldorf and Heinrich-Heine-Universität Düsseldorf, Duesseldorf, Germany, ⁶Department Internal Medicine III, Erlangen, Germany, ⁷Friedrich-Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ⁸Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁹Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ¹⁰Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ¹¹Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany
Background/Purpose: CD19-targeting CAR T cells showed remarkable improvements of modified Rodnan skin score in SSc patients within 6 months after treatment and stable reduction afterwards…
Abstract Number: 0973 • ACR Convergence 2024
Mechano-transduction via MRTF-A Pathway Is Required for Cytokine Release by Scleroderma Macrophages
Sandra Lopez Garces¹, Guan Hui Tricia Lim², Jorlin Liu³, Maahia Choudhary³, Juno Erin Vimalenthiran,³, Angela Tam¹, David Abraham³, Christopher Denton⁴, Bahja Ahmed Abdi¹ and Richard Stratton⁵, ¹University College London, London, United Kingdom, ²Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Campus, London, United Kingdom, ³UCL, London, United Kingdom, ⁴University College London, Northwood, United Kingdom, ⁵UCL, London, England, United Kingdom
Background/Purpose: In keeping with hallmark clinical evidence of stiffening and fibrotic thickening of the skin, we and others have previously demonstrated a role for the…
Abstract Number: 1575 • ACR Convergence 2024
Anti-U1RNP Antibodies Are Associated with a Distinct Clinical Phenotype and a Worse Survival in Patients with Systemic Sclerosis
Kevin Chevalier¹, Guillaume Chassagnon², Sarah LOUIS-LEONARD³, Pascal cohen⁴, bertrand Dunogue⁵, Alexis Régent⁶, Benjamin Thoreau⁵, Luc Mouthon⁵ and Benjamin Chaigne⁷, ¹Paris Cardiovascular Research Center, INSERM U970, Paris, France, ²Department of Radiology, Hôpital Cochin, AP-HP. Centre Université Paris Cité, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France, Paris, France, ³Sorbonne Université, INSERM, Department of Neurormyologie and Neuropathology, AP-HP, Hôpital Pitié-Salpêtrière, Paris, France, Paris, France, ⁴National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ⁵Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ⁶National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ⁷Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, Ile-de-France, France
Background/Purpose: Systemic sclerosis (SSc) is a rare systemic disease within the connective tissue disease (CTD) spectrum. It is characterized by microcirculatory abnormalities, skin and internal…
Abstract Number: 1814 • ACR Convergence 2024
Understanding Monocyte Derived Macrophages in the Skin of SSc Patients Through Single Cell Analysis of Blister Fluid Immune Cell Populations
Sandra Lopez Garces¹, Tamara Searle², Siyu Zhang¹, Henry Lopez³, Isra Elhussin⁴, Clayton Yates⁴, David Abraham⁵, Christopher Denton⁶, Bahja Ahmed Abdi¹ and Richard Stratton⁷, ¹University College London, London, United Kingdom, ²Imperial College London, London, United Kingdom, ³Riptide Bioscience Inc, Bethesda, MD, ⁴Johns Hopkins University, Baltimore, MD, ⁵UCL, London, United Kingdom, ⁶University College London, Northwood, United Kingdom, ⁷UCL, London, England, United Kingdom
Background/Purpose: Cells of the monocyte-macrophages (Mφ) are key players in the pathogenesis of systemic sclerosis (SSc), contributing to inflammation and fibrosis. To advance beyond the…
Abstract Number: 1867 • ACR Convergence 2024
RNA Polymerase III Specific CD8+ T Cells at the Interface Between Scleroderma and Cancer
Eleni Tiniakou¹, Mekha Thomas², Ami Shah³, Fredrick Wigley⁴, Livia Casciola-Rosen², Kellie Smith², Antony Rosen² and Erika Darrah², ¹Johns Hopkins University, Lutherville Timonium, MD, ²Johns Hopkins University, Baltimore, MD, ³Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ⁴Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Baltimore, MD
Background/Purpose: Increasing evidence suggests an immunologic link between cancer and autoimmunity. Systemic sclerosis (SSc), offers a unique opportunity to study the evolution of naturally occurring…
Abstract Number: 2456 • ACR Convergence 2024
High Levels of Circulating IFNα Are Associated with Increased Mortality in Patients with Diffuse Cutaneous Systemic Sclerosis
François Maillet¹, Carine Schmidt¹, Vincent Bondet², Alexandre Bense¹, Darragh Duffy³, Luc Mouthon¹, Mathieu Paul Rodero⁴ and Benjamin Chaigne⁵, ¹AP HP, Paris, France, ²INSERM PAsteur Institute, Paris, France, ³Inserm Pasteur Institue, Paris, France, ⁴Université Paris CIté, Laboratoire de Chimie et de Biochimie Pharmacologiques et Toxicologiques, Faculté des Saint-Pères, Paris, France, ⁵Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, Ile-de-France, France
Background/Purpose: Systemic sclerosis (SSc) is a connective tissue disease characterized by skin and organ fibrosis, autoimmunity, and vasculopathy. Although its pathophysiology remains unclear, recent data…
Abstract Number: 2646 • ACR Convergence 2024
Rare Variants in the IL1RAP Gene Implicate the IL-1 Signaling Pathway in the Pathogenesis of Systemic Sclerosis in African and European Ancestries
Yosuke Kunishita¹, Urvashi Kaundal², Martin Kerick³, Ryan Routsong⁴, Justin Lack⁴, Ami Shah⁵, Maureen Mayes⁶, Daniel Shriner⁷, Ayo P. Doumatey⁷, Amy Bentley⁷, Robyn Domsic⁸, Thomas Medsger, Jr⁹, Paula Ramos¹⁰, Richard Silver¹¹, Virginia Steen¹², John Varga¹³, Vivien Hsu¹⁴, Lesley Ann Saketkoo¹⁵, Dinesh Khanna¹³, Elena Schiopu¹⁶, Jessica Gordon¹⁷, Lindsey Criswell¹⁸, Heather Gladue¹⁹, Chris Derk²⁰, Elana Bernstein²¹, S. Louis Bridges¹⁷, Victoria Shanmugam²², Lorinda Chung²³, Suzanne Kafaja²⁴, Reem Jan²⁵, Marcin Trojanowski²⁶, Avram Goldberg²⁷, Benjamin Korman²⁸, James W. Thomas²⁹, Elaine Remmers³⁰, Adebowale Adeyemo⁷, Charles Rotimi⁷, Fredrick Wigley³¹, Francesco Boin³², Javier Martin³, Daniel Kastner³³ and Pravitt Gourh³⁴, ¹Scleroderma Genomics and Health Disparities Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²Scleroderma Genomics and Health Disparities Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Chevy Chase, MD, ³Instituto de Parasitología y Biomedicina ‘López-Neyra’, CSIC, PTS Granada, Spain, Granada, Spain, ⁴Integrated Data Sciences Section, Research Technologies Branch, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁵Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ⁶UTHealth Houston Division of Rheumatology, Houston, TX, ⁷Center for Research on Genomics and Global Health, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁸Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁹Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Verona, PA, ¹⁰Medical University of South Carolina, Charleston, SC, ¹¹Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, ¹²Georgetown University School of Medicine, Washington, DC, ¹³University of Michigan, Ann Arbor, MI, ¹⁴Department of Medicine, Rheumatology Division, Rutgers-RWJ Medical School, South Plainfield, NJ, ¹⁵New Orleans Scleroderma and Sarcoidosis Patient Care and Research Center, Louisiana State University and Tulane University Medical Schools, New Orleans, LA, ¹⁶Division of Rheumatology, Medical College of Georgia at Augusta University, Martinez, GA, ¹⁷Division of Rheumatology, Weill Cornell Medical College, New York, NY, ¹⁸Genomics of Autoimmune Rheumatic Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, Bethesda, MD, ¹⁹Arthritis & Osteoporosis Consultants of the Carolinas, Charlotte, NC, ²⁰Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ²¹Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ²²Office of Autoimmune Disease Research, Office of Research on Women's Health, National Institutes of Health, Great Falls, VA, ²³Stanford University, Woodside, CA, ²⁴Division of Rheumatology, University of California, Los Angeles, Los Angeles, CA, ²⁵Section of Rheumatology, Department of Medicine, University of Chicago, Chicago, IL, ²⁶Department of Rheumatology, Boston University School of Medicine, Boston, MA, ²⁷NYU Langone Health - NYU Hospital for Joint Diseases, Lake Success, NY, ²⁸University of Rochester, Rochester, NY, ²⁹NIH Intramural Sequencing Center, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ³⁰Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ³¹Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Baltimore, MD, ³²Cedars-Sinai Medical Center, Los Angeles, CA, ³³National Human Genome Research Institute, Bethesda, MD, ³⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by systemic inflammation and fibrosis. Interleukin-1 receptor accessory protein (IL1RAP) is a co-receptor for the Interleukin-1…

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