Abstracts tagged "Scleroderma"

Abstract Number: 0942 • ACR Convergence 2023
G Protein-coupled Receptor Kinase 5 in Fibrotic Tissue Remodeling
Cuong Tran-Manh¹, Thuong Trinh-Minh¹, Christoph Liebel¹, Chih-Wei Chen² and Joerg Distler¹, ¹Clinic 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, ²3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany
Background/Purpose: G-protein coupled receptor kinase 5, GRK5, is a central regulator of G protein-coupled receptors (GPCRs), a vast family of cell surface receptors involved in…
Abstract Number: 1524 • ACR Convergence 2023
The Effectiveness of Nintedanib in Treating Fibrosing Interstitial Lung Disease in Both Scleroderma and Non-scleroderma Patients: A Systematic Review and Meta-analysis
Yasmin Khader, Fatima Rawish, Aya Abughrbyeh, Samantha Davis, Sabeen Sidiki, Pejma Safavi and Nezam Altorok, University of Toledo, Toledo, OH
Background/Purpose: Nintedanib, an intracellular inhibitor of tyrosine kinases, has shown promise in clinical trials by inhibiting key processes associated with the advancement of lung fibrosis.…
Abstract Number: 2366 • ACR Convergence 2023
Spatial Frequency Domain Imaging as a Novel Method to Quantify Longitudinal Skin Changes in Scleroderma
Hung Vo¹, Aarohi Mehendale², Anahita Pilvar³, Eugene Kissin⁴, Marcin Trojanowski⁵, Michael York⁶, Darren Roblyer² and Andreea Bujor⁶, ¹Boston Medical Center, Peabody, MA, ²Department of Biomedical Engineering, Boston University, Boston, MA, ³Department of Electrical and Computer Engineering, Boston University, Boston, MA, ⁴Boston University, Newton, MA, ⁵Boston University School of Medicine, Boston, MA, ⁶Boston University, Boston, MA
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by excessive collagen deposition in the skin and internal organs, along with vascular dysfunction. The modified…
Abstract Number: 0093 • ACR Convergence 2023
Single Cell RNA-seq and Mass Cytometry Reveal a Cytotoxic CD8 Effector T Cell Population Associated with Interstitial Lung Disease in Systemic Sclerosis Patients
Ye Cao¹, Takanori Sasaki², Richard Ainsworth³, Kim Taylor⁴, Nunzio Bottini⁵, Mehreen Elahee⁶, Edy Kim⁷, Francesco Boin³ and Deepak Rao⁷, ¹Brigham and Women's Hospital, Boston, MA, ²Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ³Cedars-Sinai Medical Center, Los Angeles, CA, ⁴University of California San Francisco, San Francisco, CA, ⁵University of California, San Francisco, CA, ⁶University of Pittsburgh Medical Center, Pittsburgh, PA, ⁷Brigham and Women's Hospital, Boston, MA
Background/Purpose: Interstitial lung disease (ILD) is a major cause of morbidity and mortality in systemic sclerosis (SSc). We aimed to identify features of circulating immune…
Abstract Number: 0639 • ACR Convergence 2023
Evaluating Esophageal Dysmotility by Scintigraphy in Systemic Sclerosis: Subsets and Phenotypes
Antonio Salas¹, Lisa Yanek² and Zsuzsanna McMahan³, ¹Johns Hopkins University School of Medicine, Baltimore, MD, ²Johns Hopkins University School of Medicine Department of Medicine, Baltimore, MD, ³Johns Hopkins Rheumatology, Lutherville, MD
Background/Purpose: Gastrointestinal (GI) dysmotility affects most patients with systemic sclerosis (SSc), and the esophagus is the most commonly affected region. While most SSc patients are…
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: 1527 • ACR Convergence 2023
Acute Effects of Intravenous Iloprost on Finger Power Doppler Ultrasound in Scleroderma Patients
Sofia Testoni¹, Luca Magnani², Caterina Tomassini³, Alessia Laneri³, Carlo Salvarani⁴ and PIERLUIGI MACCHIONI², ¹Università degli Studi di Modena e Reggio Emilia, San Giovanni in Persiceto, Italy, ²Azienda USL -IRCCS di Reggio Emilia, Reggio Emilia, Italy, ³Università degli Studi di Modena e Reggio Emilia, Modena, Italy, ⁴Azienda USL -IRCCS di Reggio Emilia and Università di Modena e Reggio Emilia, Reggio Emilia, Italy
Background/Purpose: So far few studies explored ultrasound (US) as a tool to assess vascular subcutaneous involvement in patients affected by systemic sclerosis (SSc). We aim…
Abstract Number: 2368 • ACR Convergence 2023
Is Skin Disease a Local Manifestation of Systemic Tissue Turnover? Serological Collagen Biomarkers Provide Important Information on Skin Diseases Arising from Mutations in Collagen Genes
Signe Holm Nielsen¹, Matej Anđelić², Dovile Sinkeviciute¹, Anne-Christine Bay-Jensen¹ and Morten Karsdal¹, ¹Nordic Bioscience, Herlev, Denmark, ²Nordic Bioscience, Copenhagen, Denmark
Background/Purpose: Collagens are the main constituents of the skin. Genetic mutations in type VI, VII, and XVII collagen cause skin diseases, such as atopic dermatitis,…
Abstract Number: 029 • 2023 Pediatric Rheumatology Symposium
Cardiac Magnetic Resonance Imaging in Children with Systemic Lupus Erythematosus and Scleroderma Spectrum Disorders: A Single Center Experience
Meredith Rae, Tam Doan and Eyal Muscal, Baylor College of Medicine, Houston, TX
Background/Purpose: Cardiac disease is a major cause of morbidity and mortality in children with SLE and SSc. Various studies have demonstrated an association between duration…
Abstract Number: 086 • 2023 Pediatric Rheumatology Symposium
Systemic Sclerosis Overlap Syndrome: A Case Series from a Single Large Pediatric Center
Jessica Nguyen¹, Miriah Gillispie-Taylor¹, Eyal Muscal¹ and Marietta Deguzman², ¹Baylor College of Medicine, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare but potentially life-threatening autoimmune condition with features including immune, fibrotic, and vascular manifestations affecting the skin and…
Abstract Number: 098 • 2023 Pediatric Rheumatology Symposium
Characterization of Pulmonary Nodules in Juvenile-onset Systemic Sclerosis: A Single Center Case-Series
Jonathan Li¹, Franziska Rosser¹, Sameh Tadros¹ and Kathryn Torok², ¹UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, ²University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Given the high prevalence of intestinal lung disease (ILD) in systemic sclerosis (SSc) and the low sensitivity of pulmonary function testing for diagnosing ILD,…
Abstract Number: 116 • 2023 Pediatric Rheumatology Symposium
Clinical Significance of Anti-Scl-70 Antibodies in Pediatric Lupus Patients: A Single Center Cohort
Dawn Janysek¹, Yiressy Pina², Danielle Guffey³ and Marietta De Guzman¹, ¹Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ³Baylor College of Medicine, Houston, TX
Background/Purpose: Production of autoantibodies is a hallmark of SLE, with ANAs as a required diagnostic feature and anti-dsDNA and anti-Smith antibodies being disease-specific. Anti-Scl-70 antibodies…
Abstract Number: 123 • 2023 Pediatric Rheumatology Symposium
Development of Specific Classification Criteria for Juvenile System Sclerosis Patients: A Scoping Review
Ioana Dobre¹, Suzanne Li², Natalia Vasquez Canizares³, Barbara Reich⁴, Xurong Zhao⁵, Quinn McCormick⁶ and Marinka Twilt⁵, ¹Alberta Children's Hospital/University of Calgary, Calgary, AB, Canada, ²Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ, ³Children's Hospital at Montefiore, New York, NY, ⁴Hackensack University Medical Center, Hackensack, NJ, ⁵Alberta Children's Hospital, Calgary, AB, Canada, ⁶Hackensack Medical Hospital Network, Hackensack, NJ
Background/Purpose: Juvenile systemic sclerosis (jSSc) is associated with one of the highest morbidity and mortality rate in pediatric rheumatology, yet care recommendations are based upon…
Abstract Number: 0712 • ACR Convergence 2022
Disease Prevalence and Patient Characteristics in Patients with and Without Scleroderma Renal Crisis – A National US-based Inpatient Comparison Study
Anila Hussain¹, Pushti Khandwala² and Thais Moldovan³, ¹Crozer Chester Medical Center, Glen Mills, PA, ²Einstein Medical Center Philadelphia, Glen Mills, PA, ³Rheumatology Care Center, Media, PA
Background/Purpose: Scleroderma renal crisis is a rare complication of Systemic Sclerosis (SSc) with high mortality and morbidity. Most patients present with acute symptomatic increase in…
Abstract Number: 1170 • ACR Convergence 2022
Systemic Sclerosis Dermal Fibroblast-derived Exosomes Trigger a Type 1 Interferon Rresponse in Keratinocytes Through TBK1
Jessica Bryon¹, Christopher Wasson¹, Rebecca Ross¹, Elton Zeqiraj¹ and Francesco Del Galdo², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: Elevated Type I IFN response is present in blood and affected tissues in systemic sclerosis (SSc) and correlates with disease activity and response to…

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