Abstracts tagged "Systemic sclerosis"

Abstract Number: 1571 • ACR Convergence 2024
Growth Differentiation Factor 15 Is a Serum Biomarker and Pathogenic Factor of Progressive Fibrosis in Systemic Sclerosis
Vishal Kakkar¹, Sunhwa Kim², Yingtao Bi³, Christopher Wasson¹, Stefano Di Donato⁴, Rebecca Ross⁵, Marco Di Battista⁶, Enrico De Lorenzis⁷, Thierry Sornasse² and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²AbbVie, South San Francisco, CA, ³Abbvie, Worcester, MA, ⁴Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ⁵Medicine and Health, University of Leeds, Leeds, United Kingdom, ⁶Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Pisa, United Kingdom, ⁷Catholic University of the Sacred Heart, Roma, Rome, Italy
Background/Purpose: In systemic sclerosis (SSc), interstitial lung disease (ILD) remains the leading cause of mortality. A decline in forced vital capacity (FVC) is considered a…
Abstract Number: 1690 • ACR Convergence 2024
FAPI-PET/CT as a Predictor of Accelerated Lung Function Deterioration in Systemic Sclerosis Related Interstitial Lung Disease (ILD): Preliminary-interim Analysis from a Confirmatory Risk Factor Study
Armin Atzinger¹, Yixing Huang², Koray Tascilar³, Nadine Bayerl⁴, Jörg Distler⁵, Katrin Staudt³, Havanur Kartalczik⁶, Cosimo Bruni⁷, Oliver Distler⁸, Anna-Maria Hoffmann-Vold⁹, Tobias Bäuerle⁴, Andrea Prenner¹⁰, Florian Putz², Katharina Breininger¹¹, Torsten Kuwert¹², Bernhard Kainz¹¹, Georg Schett¹³ and Christina Bergmann¹⁴, ¹Department of Nuclear Medicine, Friedrich-Alexander-University (FAU) Erlangen-Nurnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ²Department Radiation Therapy, Friedrich-Alexander-University (FAU) Erlangen-Nurnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ³Department of Internal Medicine 3, Rheumatology & Immunology, Friedrich-Alexander-University (FAU) Erlangen-Nurnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ⁴Department Radiology, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Erlangen, Germany, ⁵Universitätsklinikum Düsseldorf and Heinrich-Heine-Universität Düsseldorf, Duesseldorf, Germany, ⁶Department of Internal Medicine 3, Rheumatology & Immunology, Friedrich-Alexander-University (FAU) Erlangen-Nurnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Frankfurt, Germany, ⁷University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland, ⁹Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ¹⁰Department Artificial Intelligence in Biomedical Engineering; FAU, Erlangen, Germany, ¹¹Department Artificial Intelligence in Biomedical Engineering, Erlangen, Germany, ¹²Department Nuclear Medicine, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Erlangen, Germany, ¹³Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ¹⁴Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany
Background/Purpose: 68Ga-FAPI-PET/CT is a novel imaging method monitoring in-vivo fibroblast activation. We recently described an association of 68Ga-FAPI-uptake with deterioration of Forced Vital capacity (FVC)…
Abstract Number: 1830 • ACR Convergence 2024
Single-cell RNA Sequencing Profiling of Very Early-Stage Systemic Sclerosis Skin Reveals a Fibroblast Pro-inflammatory Gene Signature and Keratinocyte Dysregulation
Lumeng Li¹, Elena Pachera², Rucsandra Dobrota³, Kristina Bürki¹, Carina Mihai³, Muriel Elhai⁴, Laura Much¹, Astrid Hofman¹, Pietro Bearzi⁵, Sophie Wagner⁶, Sarah Lötscher⁷, Anna-Maria Hoffmann-Vold⁸ and Oliver Distler⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁴University Hospital zurich, Zürich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁶University of Zurich, Schlieren, Switzerland, ⁷Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Zurich, Switzerland, ⁸Oslo University Hospital, Oslo, Norway, ⁹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: The poor prognosis of patients with systemic sclerosis (SSc) underscores the need to prevent disease onset and irreversible organ damage. This study aims to…
Abstract Number: 2453 • ACR Convergence 2024
Gastrointestinal Symptom Severity and Intestinal Permeability in Systemic Sclerosis: A Single Center Prospective Study
Elvira Lesmana¹, Ashley Keehn², Anukul Karn³, Andrea Pauly⁴, Margaret Breen-Lyles², Adam L Edwinson², Madhusudan Grover⁵ and Ashima Makol⁵, ¹Mayo Clinic, Rochester, MN, New York, NY, ²Mayo Clinic, Rochester, MN, Rochester, ³Mayo Clinic, Rochester, MN, Farmington Hills, MI, ⁴Mayo Clinic Rochester, Rochester, MN, ⁵Mayo Clinic, Rochester, MN, Rochester, MN
Background/Purpose: Systemic Sclerosis (SSc) is a complex, heterogeneous, multisystem autoimmune disease with high morbidity and mortality. Gastrointestinal symptoms impact more than 90% of SSc patients,…
Abstract Number: 2642 • ACR Convergence 2024
Identifying Systemic Sclerosis Molecular Subtypes by Their Cellular Signature – Deconvoluting the Puzzle
Erik Feldman¹, Neta Milman¹, Shai S. Shen-Orr¹ and Doron Rimar², ¹1. Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel., Haifa, Israel, ²Rheumatology unit, Bnai-Zion medical center and the faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel., Haifa, Israel
Background/Purpose: RNA gene expression allows to identify differentially expressed genes (DEG) in Systemic sclerosis (SSc) patients compared to healthy controls. Using unsupervised machine learning Milano…
Abstract Number: 0681 • ACR Convergence 2024
Influence of Occupational Exposure to Pollutants on Clinical and Serological Phenotypes in Systemic Sclerosis
Martina Sanahuja i Sanz¹, Irene Carrión-Barberà², Laura Tío³, Laura Triginer³, María Lee³, Alfredo Guillén-del Castillo⁴, Anna Ribes³, Lidia Valencia Muntalà⁵, Jordi Monfort², Tarek Carlos Salman Monte:⁶, Javier Narvaez-García⁷, Carmen PIlar Simeón-Aznar⁴ and Anna Pros², ¹Universitat Pompeu Fabra, Barcelona, Spain, ²Hospital del Mar, Barcelona, Spain, ³Hospital del Mar Research Institute, Barcelona, Spain, ⁴Hospital Vall d'Hebron, Barcelona, Spain, ⁵Hospital de Bellvitge, Barcelona, Spain, ⁶Hospital del Mar/Parc de Salut Mar-IMIM, Barcelona, Spain, ⁷Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: Environmental factors have been proposed as a trigger for systemic sclerosis (SSc) in genetically predisposed individuals. This study aimed to investigate the association between…
Abstract Number: 0784 • ACR Convergence 2024
RUNX1 Is Expressed in a Subpopulation of Dermal Fibroblasts and Is Increased with Systemic Sclerosis Disease Severity
Rezvan Parvizi¹, Zhiyun Gong², Helen Jarnagin², Tamar Abel², Dillon Popovich³, Madeline Morrisson⁴, Tammara Wood⁵, Sasha Shenk⁶, Monique Hinchcliff⁷, jonathan Garlick⁶, Patricia Pioli⁸ and Michael Whitfield¹, ¹Geisel School of Medicine at Dartmouth, Hanover, NH, ²Dartmouth College, Lebanon, NH, ³Dartmouth College, West Lebanon, NH, ⁴Geisel School of Medicine at Dartmouth College, Hanover, NH, ⁵Dartmouth, Hanover, NH, ⁶Tufts University, Boston, MA, ⁷Yale School of Medicine, Westport, CT, ⁸Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: Systemic Sclerosis (SSc) skin fibrosis results in complex changes in transcriptional and signaling pathways in the skin. Through transcription factor activity network analyses, the…
Abstract Number: 0968 • ACR Convergence 2024
Proteomic, Transcriptomic, and Functional Characterization of Circulating Extracellular Vesicles in Progressive Scleroderma Interstitial Lung Disease
Enrico De Lorenzis¹, Katja Koeppen², Stefano Di Donato³, Sophie Dibb⁴, Elliott Klein², Christopher Wasson⁵ and Francesco Del Galdo⁵, ¹Catholic University of the Sacred Heart, Roma, Rome, Italy, ²Boehringer-Ingelheim, Ridgefield, ³Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ⁴LIRMM University of Leeds, Leeds, ⁵University of Leeds, Leeds, United Kingdom
Background/Purpose: Progressive pulmonary fibrosis (ILD) is a leading cause of mortality in systemic sclerosis (SSc), particularly in the 30% of ILD patients who exhibit a…
Abstract Number: 1572 • ACR Convergence 2024
Associations Between the Gut Microbiota, Ultra-Processed Food Intake, and Gastrointestinal Tract Symptoms in Patients with Systemic Sclerosis
Ju Young Lee¹, Swapna Joshi², Arissa Young³, Jen Labus², Zsuzsanna McMahan⁴, Ezinne Aja², Jonathan Jacobs² and Elizabeth Volkmann⁵, ¹David Geffen School of Medicine, UCLA, Los Angeles, CA, ²UCLA, Los Angeles, ³UCLA, Los Angeles, CA, ⁴UTHealth Houston Division of Rheumatology, Houston, TX, ⁵University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Alterations in the gastrointestinal (GI) microbiome (i.e., dysbiosis) are a feature of systemic sclerosis (SSc) [1]. Diet is a known modifier of the GI…
Abstract Number: 1749 • ACR Convergence 2024
Safety and Long-term Efficacy of CD19-CAR T-cell Therapy in 30 Patients with Autoimmune Disease
Melanie Hagen¹, Fabian Müller², Andreas Wirsching¹, Carlo Tur¹, Tobias Krickau³, Markus Metzler⁴, Laura Bucci¹, Christina Bergmann⁵, Janina Auth¹, Jule Taubmann⁶, Panagiotis Garantziotis⁷, Sebastian Boeltz⁸, Soraya Kharboutli⁹, Silvia Spoerl⁹, Simon Völkl¹⁰, Michael Aigner⁹, Sascha Kretschmann⁹, Ingrid Vasova⁹, Andreas MAckensen¹¹, Ricardo Grieshaber-Bouyer⁸ and Georg Schett¹², ¹Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ²Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Germany, ³Department of Pediatrics and Adolscent Medicine, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Bayern, Germany, ⁴Department of Pediatrics and Adolscent Medicine, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁵Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁶Friedrich-Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ⁷Department of Internal Medicine 3-Rheumatology and Immunology, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, Erlangen, Germany, ⁸Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, ⁹Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ¹⁰Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Bayern, Germany, ¹¹Department of Medicine 5 - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ¹²Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany
Background/Purpose: Treating autoimmune diseases like Systemic Lupus erythematosus (SLE), Idiopathic Inflammatory Myositis (IIM) or Systemic Sclerosis (SSc) is challenging and often requires life-long immunosuppressive therapy.…
Abstract Number: 1833 • ACR Convergence 2024
Immune Complexes of Antibodies Directed to Angiotensin Receptor type-1 and Extracellular Vesicles Are Unique Modulators Contributing to Systemic Sclerosis
Alexander Maximilian Hackel¹, Marieke Leiber¹, Maike Winziers¹, Gabriela Riemekasten² and Reza Akbarzadeh¹, ¹Department of Rheumatology and Clinical Immunology, University of Lübeck, Lübeck, Germany, ²University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany
Background/Purpose: Antibodies (Abs) directed against the angiotensin II type-1 receptor (AT1R) and extracellular vesicles (EVs) derived from systemic sclerosis (SSc) patients have the potential to…
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: 0686 • ACR Convergence 2024
The Role of Inflammation in Systemic Sclerosis: CRP-Associated Phenotypes and Prognostic Implications
cristiana sieiro santos¹, Miriam Retuerto², Paula Pérez García², Jose Ordas Martínez³, Pedro Baenas², Clara Moriano Morales² and Elvira Díez Álvarez⁴, ¹Rheumatology Department, Complejo Asistencial Universitario de León, León, Spain, Leon, Spain, ²Complejo Asistencial Universitario de León, León, Spain, ³Complejo Asistencial Universitario de Leon, Leon, ⁴Complejo Asistencial Universitario de León, Leon, Spain
Background/Purpose: Systemic sclerosis (SSc) manifests through a diverse interplay of inflammatory, fibrotic, and vascular alterations, intricately entwined in its pathogenesis, which encompasses immune system dysregulation,…
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…

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