Abstracts tagged "Scleroderma"

Abstract Number: 0696 • ACR Convergence 2024
MRI DAVIX Index Is an Imaging Biomarker for Endothelial Damage of Systemic Sclerosis Digital Ulcers
Stefano Di Donato¹, Rebecca Ross², Yassir El-Sherbiny³, Riccardo Bixio⁴, Marco Minerba⁵, Christopher Wasson⁶, Jun Li⁷, Jochen SCHMITZ⁸, Sudha Visvanathan⁹ and Francesco Del Galdo⁶, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ²Medicine and Health, University of Leeds, Leeds, United Kingdom, ³Nottingham Trent University, Nottingham, United Kingdom, ⁴University of Verona, Verona, Verona, Italy, ⁵1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, United Kingdom, ⁶University of Leeds, Leeds, United Kingdom, ⁷Boehringer Ingelheim, Danbury, CT, ⁸Boehringer-Ingelheim, Ridgefield, CT, ⁹Boehringer-Ingelheim Pharmaceuticals, Ridgefield, CT
Background/Purpose: Circulating endothelial cells (CEC) and their precursors have been shown to correlate to the severity of vascular manifestations in Systemic sclerosis1, such as Digital…
Abstract Number: 0970 • ACR Convergence 2024
Deubiquitylase (DUB) BRCC36 Isopeptidase Complex (BRISC) Inhibitors Prevent IFNAR1 Deubiquitination to Restore Natural Type I IFN Response in Autoimmunity
Rebecca Ross¹, Poli Adi Narayanna Reddy², Joseph Salvino², Elton Zeqiraj³ and Francesco Del Galdo³, ¹Medicine and Health, University of Leeds, Leeds, United Kingdom, ²The Wistar Institute, Philadelphia, PA, ³University of Leeds, Leeds, United Kingdom
Background/Purpose: Increased Type I IFN activation plays a key role in Scleroderma (SSc), Systemic Lupus Erythematosus and Inflammatory Myositis. Deubiquitylase (DUB) BRCC36 isopeptidase complex (BRISC)…
Abstract Number: 1583 • ACR Convergence 2024
The Association of Weakpositive Anti-RNA Polymerase III Antibodies with the Diagnosis and Complications of Systemic Sclerosis and Other Systemic Autoimmune Diseases
Mashood Badshah¹, Warren Balaja², Brannon Donovan³, Paul Schmidt³ and Megan Krause⁴, ¹The University of Kansas Medical Center, Kansas City, KS, ²University of Kansas Medical Center, Kansas City, KS, ³University of Kansas Medical Center, Kansas City, ⁴University of Kansas, Kansas City, KS
Background/Purpose: Anti-RNA polymerase III antibody (ARA) is associated with systemic sclerosis (SSc). The presence of ARA in patients with SSc is associated with the risk…
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: 2461 • ACR Convergence 2024
Prevalence and Impact of Peripheral Neuropathy in SSc: A Frequent and Disabling Manifestation in SSc, and a Potential Surrogate Marker for Autonomic Neuropathy
Begonya Alcacer-Pitarch¹, marco di battista², Anthony C. Redmond³, Anne-Maree Keenan⁴, Stefano Di Donato⁵, Francesco Del Galdo³ and Maya H. Buch⁶, ¹Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom, ²Rheumatology Unit, University of Pisa, Pisa., Pisa., Italy, ³University of Leeds, Leeds, United Kingdom, ⁴School of Healthcare, university of leeds, leeds, United Kingdom, ⁵Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ⁶Division of Musculoskeletal & Dermatological Sciences, University of Manchester, and NIHR Manchester Biomedical Research Centre, Manchester, United Kingdom
Background/Purpose: SSc patients have high disability and low quality of life (QoL), driven partially by poor physical function (PF), Gastro Intestinal (GI) problems, digital ulcers…
Abstract Number: 0700 • ACR Convergence 2024
Prospective Evaluation of Capillaroscopy in Healthy Children
Tessa Dufour¹, Maurizio Cutolo², Dieneke Schonenberg-Meinema³, Kaat Wyckstandt⁴, Adela Sarbu⁵, joke dehoorne⁶, Steven Wallaert⁷ and vanessa smith⁸, ¹University Hospital Ghent, Gent, Belgium, ²Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genova, Italy, ³Amsterdam University Medical Center, Amsterdam, Netherlands, ⁴University Hospital Ghent, Ghent, Belgium, ⁵University hospital Bern, Bern, Switzerland, ⁶University Hospital Ghent, Ghent, ⁷Ghent University, Ghent, Belgium, ⁸Ghent University Hospital, Gent, Belgium
Background/Purpose: Capillaroscopy is a well-established tool in the diagnosis of adult-onset rheumatic diseases and global consensus on standardisation of execution and interpretation performing capillaroscopy have…
Abstract Number: 0971 • ACR Convergence 2024
Ligand-Receptor Analysis Reveals Myeloid-Mediated Pro-Inflammatory Responses and Loss of Mesenchymal Signaling Driven by TNFR1 in Experimental Pulmonary Hypertension
Gaochan Wang¹, Stacey Duemmel² and Benjamin Korman³, ¹University of Rochester School of Medicine and Dentistry, Rochester, NY, ²URMC, UNIVERSITY OF ROCHESTER, Rochester, NY, ³University of Rochester, Rochester, NY
Background/Purpose: Pulmonary hypertension (PH) is a severe, progressive disorder characterized by elevated pulmonary artery pressures, right ventricular hypertrophy, and increased mortality which is a severe…
Abstract Number: 1587 • ACR Convergence 2024
Predictors of Body Mass Index in an Early Systemic Sclerosis Cohort
Ali Ayla¹, Meng Zhang¹, Claudia Pedroza², Bingrui Chen², Brian Skaug¹, Maureen Mayes¹, Shervin Assassi¹ and Zsuzsanna McMahan¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²UTHealth Houston, Houston, TX
Background/Purpose: Malnutrition is a significant problem among patients with systemic sclerosis (SSc). Body mass index (BMI) is often low in patients with malnutrition. Low BMI…
Abstract Number: 1831 • ACR Convergence 2024
Role of Long Non-Coding RNA H19 in Mediating Skin Fibrosis in Systemic Sclerosis
Begoña Caballero-Ruiz¹, Christopher Wasson², Rebecca Ross³ and Francesco Del Galdo², ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, ²University of Leeds, Leeds, United Kingdom, ³Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) is an immune driven condition leading to skin fibrosis, as well as internal organs. Dermal fibroblasts are the key cellular elements…
Abstract Number: 2462 • ACR Convergence 2024
A Potent Inhibitor of PAI-1, MDI-2517, Mitigates Disease Severity in Preclinical Models of Systemic Sclerosis
Enming Su¹, Pei-Suen Tsou¹, Mark Warnock², Natalya Subbotina¹, Kris Mann¹, Sirapa Vichaikul¹, Xianying Xing¹, Enze Xing¹, Olesya Plazyo¹, Rachael Wasikowski¹, Lam C. Tsoi³, Mark Weinberg⁴, Cory D. Emal⁵, Dinesh Khanna¹, John Varga¹, Thomas H. Sisson¹, Johann Gudjonsson¹ and Daniel Lawrence², ¹University of Michigan, Ann Arbor, MI, ²Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI, ³Michigan, Dept. of Dermatology, Ann Arbor, MI, ⁴MDI, Ann Arbor, MI, ⁵EMU, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a complex and heterogeneous condition characterized by progressive fibrosis in multiple organs. Currently, there is no known cure for SSc,…
Abstract Number: 0701 • ACR Convergence 2024
Diagnostic Performances of Vascular Biomarkers Soluble Fms-like Tyrosine Kinase (sFlt-1) and Placental Growth Factor (PlGF) in Scleroderma Renal Crisis: A Case Control Study
Aïcha Kante¹, Paul Legendre², Bérangère Joly³, bertrand Dunogue⁴, Alexandre Hertig⁵, Benjamin Terrier⁶, Elodie Massolin⁷, Felix Ackermann⁸, Luc Mouthon⁴, Jean Guibourdenche⁹ and Benjamin Chaigne¹⁰, ¹Department of internal medicine, Centre de Compétence Maladies Rares autoimmunes et inflammatoires, Université Paris Cité, Paris, Ile-de-France, France, ²Service immunologie clinique, Centre hospitalier du Mans, Le Mans, France, Le Mans, ³Service d'Hématologie Biologique, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris (APHP.Nord), Université Paris Cité, Paris, Ile-de-France, France, ⁴Service 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, ⁵Department of Nephrology, Hôpital Foch, Suresnes, France, Suresnes, Ile-de-France, France, ⁶Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, ⁷UF d'Hormonologie, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Paris, Paris, Ile-de-France, France, ⁸Department of Internal Medicine, Foch Hospital, Suresnes, France., Suresnes, France, ⁹APHP-CUP, Hôpital Cochin, F-75014 Paris, Université Paris Cité, Paris, Ile-de-France, France, ¹⁰Service 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: Scleroderma renal crisis (SRC) is a major vascular complication of systemic sclerosis (SSc), associated with high morbidity and mortality. Few biomarkers are available to predict…
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: 1588 • ACR Convergence 2024
Validation of a Lung Ultrasound Interpretation Criteria for Interstitial Lung Disease Screening in Systemic Sclerosis and Inflammatory Myopathy
Robert Fairchild¹, Diane Mar², Mariani Deluna³, Henry Guo³, David Fiorentino⁴ and Lorinda Chung⁵, ¹Stanford University, San Francisco, CA, ²VA Palo Alto / Stanford, Palo Alto, CA, ³Stanford University, Palo Alto, CA, ⁴Stanford University, Menlo Park, CA, ⁵Stanford University, Woodside, CA
Background/Purpose: Interstitial lung disease (ILD) is a common complication of connective tissue disease (CTD) with a high prevalence in SSc and inflammatory myopathy (IM). Early…
Abstract Number: 1832 • ACR Convergence 2024
Targeting Hippo Pathway Diminishes Disease Signaling in Scleroderma Skin
Olesya Plazyo¹, Rachael Wasikowski², Mehrnaz Gharaee-Kermani¹, Eliza Pei-Suen Tsou¹, Lam Tsoi¹, Allison Billi¹, J. Michelle Kahlenberg¹, John Varga¹, Johann Gudjonsson¹ and Dinesh Khanna¹, ¹University of Michigan, Ann Arbor, MI, ²Michigan, Dept. of Dermatology, Ann Arbor, MI
Background/Purpose: Scleroderma (SSc), a female-biased autoimmune disease that causes inflammation and fibrosis in skin and internal organs, lacks effective treatment options. Our previous work, including…
Abstract Number: 2463 • ACR Convergence 2024
Interstitial Lung Disease in Very Early Systemic Sclerosis – a Clinical and Radiological Characterization
Sinziana Muraru¹, Christian Blüthgen², Cosimo Bruni¹, Carina Mihai¹, Anna-Maria Hoffmann-Vold³, Muriel Elhai⁴, Mike Becker⁵, Thomas Frauenfelder⁶, Oliver Distler⁷ and Rucsandra Dobrota¹, ¹University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²Institute for Diagnostic and Interventional Radiology, University Hospital Zurich, Zurich, Switzerland, ³Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ⁴University Hospital zurich, Zürich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁶University Hospital Zurich, Zurich, Zurich, Switzerland, ⁷Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Interstitial lung disease (ILD) occurs in about 50% of patients with systemic sclerosis (SSc). The aims of this study were to assess the prevalence…

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