Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 1590 • ACR Convergence 2025
Association of levels of soluble CD13 with clinical features and fibrosis in systemic sclerosis patients
Yuzo Ikari¹, Poulami Dey², James St. Clair³, Anna Webber⁴, Caroline Foster⁵, Yi-Chen Chen⁵, Ramadan Ali⁶, Dinesh Khanna⁷, David Fox⁸ and Pei-Suen Tsou⁷, ¹Division of Rheumatology, Department of Medicine, Showa Medical University School of Medicine, Tokyo, Japan, ²Michigan Medicine, Ann Arbor, MI, ³University of Michigan, Ann Arbor, ⁴Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, ⁵Division of Rheumatology, Department of Internal Medicine, University of Michigan, ANN ARBOR, MI, ⁶University of Michigan, Ann Arobr, MI, ⁷University of Michigan, Ann Arbor, MI, ⁸University of Michigan, Dexter, MI
Background/Purpose: Soluble CD13 (sCD13), released by the cleavage of cell surface CD13 by matrix metalloproteinase-14 (MMP14), has potent proinflammatory, angiogenic, and arthritogenic activities. The receptors…
Abstract Number: 0977 • ACR Convergence 2025
The nuclear receptor DAX1 regulates WNT/β-catenin signaling to promote fibroblast activation and skin fibrosis in systemic sclerosis
Lichong Shen¹, Xianyi Meng², Yun Zhang³ and Joerg Distler⁴, ¹³Department of Rheumatology, Ren Ji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China (People's Republic), ²Clinic for Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Duesseldorf, Germany, ³University Hospital Duesseldorf, Duesseldorf, Germany, ⁴Clinic for Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University; Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Duesseldorf, Germany
Background/Purpose: Nuclear receptors are a family of transcription factors that is commonly targeted for therapeutic intervention. Dosage-sensitive sex reversal adrenal hypoplasia congenital critical region on…
Abstract Number: 0812 • ACR Convergence 2025
Anti-CD206 CAR T Cell Immunotherapy Mitigates Dermal Pathology in Systemic Sclerosis
Chanhyuk Park¹, Helen Jarnagin², Asmaa Mohamed³, Noelle Kosarek⁴, Owen Wilkins¹, Fred Kolling¹, Yina Huang¹, Michael Whitfield⁵ and Patricia Pioli¹, ¹Geisel School of Medicine at Dartmouth, Lebanon, NH, ²Dartmouth College, Lebanon, NH, ³Geisel School of Medicine at Dartmouth, Charlottesville, VA, ⁴Dartmouth Geisel School of Medicine, Lebanon, NH, ⁵Geisel School of Medicine, Lebanon, NH
Background/Purpose: Systemic sclerosis (SSc) is a progressive, chronic multi-system disorder of unknown etiology that is characterized by immune dysfunction, fibrosis, and loss of dermal white…
Abstract Number: 2655 • ACR Convergence 2025
Fetal and maternal outcomes in systemic sclerosis and very early diagnosis of systemic sclerosis pregnancies, a national prospective study
Anne Murarasu¹, lauren beaudeau², Véronique Le Guern³, gaelle guettrot-Imbert³, claire cazalets⁴, cecile durant⁵, Cecile Yelnik⁶, Céline Roussin⁷, Marie-Charlotte Besse⁸, Emilie Berthoux⁹, Emmanuel Chatelus¹⁰, Eric Hachulla¹¹, Estibaliz LAZARO¹², Francois Maurier¹³, Gaelle leroux¹⁴, Grégory Pugnet¹⁵, Isabelle durieu¹⁶, Loic RAFFRAY¹⁷, Maelle Le Besnerais¹⁸, Mélanie roriz¹⁹, Odile SOUCHAUD-DEBOUVERIE²⁰, Patrick JEGO²¹, Pauline ORQUEVAUX²², Claire de Moreuil²³, Helene MAILLARD²⁴, Nathalie Morel²⁵, Anna Molto²⁶, Camille Le Ray²⁷, emmanuelle pannier²⁷, loic sentilhes²⁵, Luc Mouthon²⁸, Nathalie Costedoat-Chalumeau²⁹ and Benjamin Chaigne³⁰, ¹Tours University Hospital, Tours, France, ²Centre Hospitalier Ales Les Cevennes, Ales, France, ³Cochin hospital, Paris, France, ⁴Rennes Hospital, Rennes, France, ⁵Nantes University Hospital, Nantes, France, ⁶lille university, Lille, France, ⁷Centre Hospitalier Ouest Réunion, Saint Paul, France, ⁸Tours University Hospital, ³⁷⁰⁰⁰ tours, Ile-de-France, France, ⁹Internal Medicine, Saint Luc Saint Joseph Hospital, Lyon, France, ¹⁰Department of Rheumatology, University Hospital Strasbourg, France, Strasbourg, France, ¹¹University of Lille, LILLE, France, ¹²Bordeaux University Hospital, Pessac, France, ¹³HOPITAUX PRIVES DE METZ, Vaux / Frankreich, France, ¹⁴Pitié-Salpêtrière University Hospital, Paris, France, ¹⁵CHU Toulouse Rangueil Service de Medecine Interne et Immunologie Clinique, Toulouse, France, ¹⁶Lyon University Hospital, Lyon, France, ¹⁷Félix-Guyon University Hospital of La Réunion, Saint Denis, France, ¹⁸Rouen University Hospital, Rouen, France, ¹⁹Agen hospital, Agen, France, ²⁰Poitiers University Hospital, Poitiers, France, ²¹Rennes University Hospital, Rennes, France, ²²Reims University Hospital, Reims, France, ²³CHU DE BREST, BREST, France, ²⁴Lille University Hospital, Lille, France, ²⁵Bordeaux University Hospital, Bordeaux, France, ²⁶Assistance Publique Hôpitauxde Paris, Paris, France, ²⁷Cochin Port Royal University Hospital, Paris, France, ²⁸Hopital Cochin - Paris University, Paris, France, ²⁹Inserm DR Paris ⁵, Paris, France, ³⁰Cochin University Hospital, Paris, France
Background/Purpose: Pregnancy in women with systemic sclerosis (SSc) is considered high-risk due to complications like scleroderma renal crisis and preeclampsia, related to vasculopathy. Prospective data…
Abstract Number: 1589 • ACR Convergence 2025
Nintedanib in Systemic Sclerosis-Associated Interstitial Lung disease: Real-World Cohort Study on Tolerability and Discontinuation
Punchalee Kaenmuang¹, Nina Goldman², Arthihai Srirangan¹, Fiammetta Danzo¹, Wing-Ho Yip¹, Ali Taki³, Shereen Boreland¹, Benedetta Vitri¹, Carmel JW. Stock¹, Christopher Denton⁴, Athol U. Wells¹, Voon H. Ong⁵, Piersante Sestini⁶ and Elisabetta A. Renzoni¹, ¹Interstitial Lung Disease Unit, Royal Brompton Hospital, Guy’s and St Thomas’ National Health Service Foundation Trust, London, England, United Kingdom, ²Division of Medicine, Centre for Rheumatology, University College London, London, England, United Kingdom, ³Royal Free Hospital, London, England, United Kingdom, ⁴University College London, UK, London, United Kingdom, ⁵Division of Medicine, Centre for Rheumatology, University College London, London, United Kingdom, ⁶Department of Medical and Surgical Sciences and Neurosciences, University of Siena, Siena, Toscana, Italy
Background/Purpose: Nintedanib slows the progression of systemic sclerosis-associated interstitial lung disease (SSc-ILD). However, potential high discontinuation rates due to adverse side-effects raise concerns about its…
Abstract Number: 0976 • ACR Convergence 2025
LMPTP Drives Fibrosis in Systemic Sclerosis via TGF-beta Signaling Activation
Yuan Zhan¹, Colton Sanders², Michael Diaz², Jinmin Miao³, Arminja Kettenbach⁴, Zhong-Yin Zhang⁵, Paul Wolters⁶, Francesco Boin⁷, Stephanie Stanford² and Nunzio Bottini⁸, ¹Cedars-Sinai Medical Center, Los Angeles, CA, ²University of California San Diego, San Diego, CA, ³Purdue University, West Lafayette, IN, ⁴Geisel School of Medicine at Dartmouth, Lebanon, NH, ⁵Purdue University, Department of Medicinal Chemistry and Molecular Pharmacology, West Lafayette, IN, ⁶University of California San Francisco, San Francisco, CA, ⁷Cedars-Sinai Medical Center, Beverly Hills, CA, ⁸Cedars Sinai Medical Center, Beverly Hills, CA
Background/Purpose: The identification of effective and safe anti-fibrotic agents is a critical unmet need in systemic sclerosis (SSc). Although fibrosis in SSc is driven by…
Abstract Number: 0811 • ACR Convergence 2025
SSc Skin Cell Atlas: a Scalable Web Portal for scRNA-Seq Analysis
Helen Jarnagin¹, Zhiyun Gong¹, Rachael Bogle², Alex Tsoi², Rezvan Parvizi³, Madeline Morrisson⁴, Dinesh Khanna⁵, Johann Gudjonsson⁵ and Michael Whitfield⁶, ¹Dartmouth College, Lebanon, NH, ²University of Michigan, Holland, OH, ³Dartmouth, lebanon, NH, ⁴Geisel School of Medicine at Dartmouth College, Hanover, NH, ⁵University of Michigan, Ann Arbor, MI, ⁶Geisel School of Medicine, Lebanon, NH
Background/Purpose: Despite the recent popularity and utility of modern high-resolution sequencing technologies, leveraging publicly available single-cell studies remains hampered by the need for substantial computational…
Abstract Number: 2653 • ACR Convergence 2025
First Prospective Evaluation of Recombinant Herpes Zoster Vaccine in Systemic Sclerosis: Immunogenicity, Safety, and Disease Activity Outcomes
Ana Paula Luppino-Assad¹, Renata P Mello², Ana C Medeiros-Ribeiro³, Nadia E Aikawa⁴, Sandra G Pasoto³, Leonard V Kupa⁵, Renata Miossi⁶, Percival D Sampaio-Barros⁶, Eduardo Borba⁷, Eloisa Bonfa⁷ and Clovis Artur Silva⁸, ¹Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, SAO PAULO, Brazil, ²Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, Sao Paulo, Brazil, ³Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, Sao Paulo, Brazil, ⁴Pediatric Rheumatology Unit, Instituto da Criança e do Adolescente, Hospital das Clinicas HCFMUSP, Sao Paulo, Brazil., Sao Paulo, Sao Paulo, Brazil, ⁵Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, SP, Brazil, São Paulo, Sao Paulo, Brazil, ⁶Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, ⁷Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, São Paulo, Brazil, ⁸University of São Paulo, São Paulo, São Paulo, Brazil
Background/Purpose: Systemic sclerosis (SSc) patients are at higher risk of herpes zoster (HZ) due to underlying immune dysregulation and immunosuppressive therapies. Vaccination is a key…
Abstract Number: 1587 • ACR Convergence 2025
Longitudinal Clinical Response to JAK Inhibitors in Systemic Sclerosis: A Real-Life Multicentric Study Across Clinical Domains
Stefano Di Donato¹, JUAN JOSE ALEGRE SANCHO², Anastas Batalov³, Zguro Batalov⁴, Silvia Bellando-Randone⁵, carmela coccia⁶, Marco de Pinto⁷, Dilia Giuggioli⁸ and Michael Hughes⁹, ¹University of Leeds, Canosa Sannita, Chieti, Italy, ²Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ³Medical University of Plovdiv, Plovdiv, Bulgaria, ⁴UMHAT Kaspela, Ploudiv, Bulgaria, ⁵University of Florence, Florence, Florence, Italy, ⁶Rheumatology Unit, Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy, ⁷Azienda Ospedaliero Universitaria Policlinico di Modena, Parma, Parma, Italy, ⁸Scleroderma Unit, Rheumatology Unit, University Hospital of Modena and Reggio Emilia, Modena, Italy, Modena, Italy, ⁹Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK, Manchester, England, United Kingdom
Background/Purpose: JAK inhibitors (JAKi) have shown promising early results in systemic sclerosis (SSc) patients, yet real-world data on their longitudinal effects across different disease domains…
Abstract Number: 0968 • ACR Convergence 2025
Mesenchymal Stromal Cells in Systemic Sclerosis are Dysfunctional and Have a Profibrotic and Senescent Phenotype
Marianela Brizio¹, Benoit Brilland¹, Maximilien Lora², Mathieu Mancini¹, David Langlais¹, Marie Hudson³ and Ines Colmegna⁴, ¹Research Institute of the McGill University Health Centre, Montreal, QC, Canada, ²Research Institute of the McGill University Health Centre, Montreal, ³McGill University, Montréal, QC, Canada, ⁴The Research Institute of the McGill University Health Centre, Montréal, QC, Canada
Background/Purpose: Mesenchymal stromal cells (MSCs) are non-hematopoietic multipotent cells with immunomodulatory, proangiogenic, and antifibrotic properties. MSC functions are mediated by paracrine soluble factors and small…
Abstract Number: 0259 • ACR Convergence 2025
Inhaled pirfenidone as an innovative therapeutic approach to treat autoimmune ILD and other forms of Progressive Pulmonary Fibrosis: Phase 2b Study Design
Kristin Highland¹, Colin Reisner², Allison Trucillo³, Deepthi Nair⁴, Felix Woodhead⁴, Howard Lazarus⁴, Craig Conoscenti⁴ and Martin Kolb⁵, ¹Cleveland Clinic, Shaker Heights, OH, ²DevPro Biopharma, Basking Ridge, NJ, ³Avalyn Pharma, Austin, TX, ⁴Avalyn Pharma, Cambridge, MA, ⁵McMaster University, Hamilton, ON, Canada
Background/Purpose: Progressive Pulmonary Fibrosis (PPF) is an increasingly recognized condition, defined in 2022 to address the progression of pulmonary fibrosis in patients with interstitial lung…
Abstract Number: 2493 • ACR Convergence 2025
Investigating scoring systems to measure clinically relevant changes in systemic sclerosis-related finger acro-osteolysis over time, as assessed in serial hand radiographs
Michael Hughes¹, Graham Dinsdale², Aayushi Agnihotri³, Joanne Manning², Muditha Samaranayaka⁴, Jonathan Harris² and Ariane Herrick⁵, ¹The University of Manchester, Manchester, England, United Kingdom, ²Northern Care Alliance NHS Foundation Trust, Salford, United Kingdom, ³The University of Manchester, Manchester, United Kingdom, ⁴Salford Royal Hospital, Salford, United Kingdom, ⁵The University of Manchester, UK, Aberdeen, United Kingdom
Background/Purpose: Acro-osteolysis (A-O, terminal tuft digit resorption) is characteristic of systemic sclerosis (SSc), occurring in 20-25% of patients (1). Outcome measures are much needed. Following…
Abstract Number: 1580 • ACR Convergence 2025
Lung vasculature quantification on computed tomography predicts new onset of interstitial lung disease in systemic sclerosis
Maria Iacovantuono¹, Nicholas landini², lisa Jungblut³, Gesa Sauer⁴, Rucsandra Dobrota⁵, Sinziana Muraru⁶, Muriel Elhai⁷, Carina Mihai⁸, Mike Becker⁹, Maria Sole Chimenti¹⁰, Thomas Frauenfelder³, Anna-Maria Hoffmann-Vold¹¹, Oliver Distler¹² and Cosimo Bruni¹³, ¹Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, Tor Vergata University, Rome, Italy. Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Spinete, Italy, ²Department of Radiological Sciences, Oncology and Pathology, "Sapienza" University, Policlinico Umberto I, Rome, Italy. Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Rueil Malmaison, France, ³Institute for Diagnostic and Interventional Radiology, University Hospital Zurich, University Zurich, Zurich, Switzerland, zurich, Switzerland, ⁴Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, zurich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, the LOOP Zurich, Zurich, Switzerland, ⁶University of Zurich, University Hospital Zurich, Zürich, Switzerland, ⁷University Hospital zurich, Zürich, Switzerland, ⁸University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁹Dept. of Rheumatology, University Hospital Zurich, Zürich, Switzerland, ¹⁰Rheumatology, Allergology and Clinical Immunology, Department of Systems Medicine, Tor Vergata University, Rome, Italy, Roma, Italy, ¹¹Oslo University Hospital, Oslo, Norway, ¹²Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland, ¹³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
Background/Purpose: Interstitial lung disease (ILD) is highly prevalent in systemic sclerosis (SSc) and a leading cause of mortality. Recent studies have identified clinical risk factors…
Abstract Number: 0965 • ACR Convergence 2025
Inhibition of Nicotinamide N-Methyltransferase Reverses Fibroblast Activation via Epigenetic and Metabolic Remodeling in Systemic Sclerosis
Kamal Saba¹, Rong Huang², Priyanka Verma¹, M Asif Amin¹ and John Varga¹, ¹University of Michigan, Ann Arbor, MI, ²Purdue University, West Lafayette, IN
Background/Purpose: Systemic sclerosis (SSc) is characterized by progressive fibrosis driven by sustained fibroblast activation and senescence. Nicotinamide N-methyltransferase (NNMT), a SAM-dependent enzyme, is upregulated in…
Abstract Number: 2492 • ACR Convergence 2025
Autoantibody Profiles and Disease Trajectories in Early Systemic Sclerosis: Insights from a Prospective Cohort
Marzieh Jamali¹, Amisha Kaur¹, Suiyuan Huang¹, James St. Clair², John Varga¹, Carleigh Zahn², Erica Mulcaire-Jones¹, Alain Lescoat¹ and Dinesh Khanna¹, ¹University of Michigan, Ann arbor, MI, ²University of Michigan, Ann Arbor
Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease with high mortality. Early stratification by autoantibody (SSc-Ab) profile may inform prognosis and management, but data…

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