Abstracts tagged "Scleroderma, Systemic"

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: L01 • ACR Convergence 2024
Targeted Exosite Inhibition of STING Activation of TBK1 Selectively Blocks Type I Interferon and NFκB Responses for Treatment of Autoimmune Diseases
Matthew Martin¹, Erik Wilker¹, Diana Gikunju¹, Usha Narayanan¹, Unnati Pandya¹, Vijetha Prakash¹, Ashley Edwards¹, Sameer Kawatkar¹, Tenghui Chen¹, Ragunath Chandran¹, Sai Sunder¹, Sumathi Biradar¹, Joerg Distler², Alexandra Joseph¹, Stephanos Ioannidis¹ and Bhavatarini Vangamudi¹, ¹Exo Therapeutics, Cambridge, MA, ²Exo Therapeutics, Düsseldorf, Germany
Background/Purpose: The cGAS-TBK1-STING pathway senses nucleic acids for innate immunity. Aberrant activation of the pathway is linked to autoimmune diseases including Systemic and Cutaneous Lupus…
Abstract Number: 0026 • ACR Convergence 2024
Conserved Pro-Fibrotic Fibroblast Signatures in Osteoarthritis Synovium and Systemic Sclerosis Skin
Michael Newton¹, Aanya Mohan², Tareq Hanna³, Dana Orange⁴, John Varga² and Tristan Maerz², ¹University of Michigan, South Lyon, MI, ²University of Michigan, Ann Arbor, MI, ³Wayne State University, Detroit, MI, ⁴The Rockefeller University, New York, NY
Background/Purpose: Synovial fibrosis is a hallmark of osteoarthritis (OA) and a clinical correlate to joint pain. Fibrosis of skin, lungs and other organs is the…
Abstract Number: 1566 • ACR Convergence 2024
Comparative Analysis of Clinical Features and Outcomes in Late Onset vs. Early Onset Scleroderma Renal Crisis – A Multicenter Study
Sumbal Wajid¹, Lee Shapiro², Virginia Steen³, Robyn Domsic⁴, Paul J. Feustel⁵ and Swati Mehta⁶, ¹Albany Medical Center, Albany, NY, ²Division of Rheumatology, Department of Medicine, Albany Medical Center, Albany, NY, ³Georgetown University School of Medicine, Washington, DC, ⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁵Department of Neuroscience and Experimental Therapeutics, Albany Medical Center, Albany, NY, ⁶Division of Nephrology, Department of Medicine, Albany Medical Center, Albany, NY
Background/Purpose: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc), historically described to occur within the first 5 years of SSc diagnosis.…
Abstract Number: 2459 • ACR Convergence 2024
Use of Nailfold Capillaroscopy as a Predictor of Cardiovascular Events and Mortality in Systemic Sclerosis
Carlos Valera-Ribera¹, Javier Narvaez-García², Joaquín Lacasa-Molina³, Laura Perea-Galera⁴, Alida Taberner-Cortés³, Paula Andrea Mencio-Viana³, Patricia Leon-Torralba³, Adrián Mayo-Juanatey³, Pablo Andújar-Brazal³, Ignacio Vázquez Gómez⁵, Desamparados Ybáñez-García³, Àngels Martínez-Ferrer⁶, Elia Valls-Pascual⁷, Montserrat Robustillo Villarino⁸ and JUAN JOSE ALEGRE SANCHO³, ¹Hospital Doctor Peset, Valéncia, Comunidad Valenciana, Spain, ²Hospital Universitario de Bellvitge, Barcelona, Spain, ³Hospital Universitario Doctor Peset, Valencia, Spain, ⁴Hospital Universitario Doctor Peset, Valencia, ⁵H. U. Dr. Peset, Valencia, ⁶Hospital Universitario Dr Peset Valéncia, Valéncia, Spain, ⁷Hospital General de Valencia, Valencia, Comunidad Valenciana, Spain, ⁸Hospital Universitario La Plana, Villarreal, Spain
Background/Purpose: Systemic sclerosis (SSc) is a disease with high morbidity and mortality. Nailfold capillaroscopy (NFC) is a widely used method for its diagnosis. Our objective…
Abstract Number: 0680 • ACR Convergence 2024
Clinical Correlates of Physical and Mental Health in Early Systemic Sclerosis: Data from the Collaborative National Quality and Efficacy (CONQUER) Registry
Ashima Makol¹, Isha Lamba², Dinesh Khanna³, John VanBuren⁴, Angela Child⁴, Jessica Alvey⁴, Shervin Assassi⁵, Elana Bernstein⁶, Flavia Castelino⁷, Lorinda Chung⁸, Luke Evnin⁹, Tracy Frech¹⁰, Faye Hant¹¹, Laura Hummers¹², Kimberly Lakin¹³, Dorota Lebiedz-Odrobina¹⁴, Yiming Luo¹⁵, Jerry Molitor¹⁶, Duncan Moore¹⁷, Carrie Richardson¹⁸, Nora Sandorfi¹⁹, Ami Shah²⁰, Ankoor Shah²¹, Brian Skaug⁵, Virginia Steen²², Elizabeth Volkmann²³ and Jessica Gordon²⁴, ¹Mayo Clinic, Rochester, MN, Rochester, MN, ²New York Presbyterian Hospital/Weill Cornell Medical Centre, New York, NY, ³University of Michigan, Ann Arbor, MI, ⁴University of Utah, Salt Lake City, UT, ⁵UTHealth Houston Division of Rheumatology, Houston, TX, ⁶Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁷Massachusetts General Hospital, Boston, MA, ⁸Stanford University, Woodside, CA, ⁹Scleroderma Research Foundation, Brisbane, CA, ¹⁰Vanderbilt University Medical Center, Nashville, TN, ¹¹Medical University of South Carolina, Charleston, SC, ¹²Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹³Hospital for Special Surgery, New York, NY, ¹⁴University of Utah, Cottonwood Heights, UT, ¹⁵Columbia University, New York, NY, 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, ²²Georgetown University School of Medicine, Washington, DC, ²³University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles, ²⁴Division of Rheumatology, Weill Cornell Medical College, New York, NY
Background/Purpose: Early years of Systemic sclerosis (SSc) are typically the period of peak disease activity and associated with greater pain, fatigue, sleep disturbance, physical impairment,…
Abstract Number: 1569 • ACR Convergence 2024
Scleroderma Renal Crisis – Different Phenotypes and Patient Trajectories
Katya Dolnikov¹, Kochava Toledano¹, Marius Braun², Doron Markovits¹, Sami Giryes¹, Yonit Tavor¹, Alexandra Balbir-Gurman³ and Yolanda Braun Moscovici¹, ¹Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technology, HAIFA, Israel, ²Liver Insitute, Beilinson Hospital, Petach Tikva, Petach Tikva, Israel, ³Rheumatology Institute, Rambam Health Care Campus and The Rappaport Faculty of |Medicine, Technion, Haifa, Israel
Background/Purpose: Scleroderma renal crisis (SRC) is a life-threatening complication that occurs in a minority of patients, particularly those with diffuse cutaneous systemic sclerosis ( SSc).…
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: 0682 • ACR Convergence 2024
Results from Outcomes Selection and Development of a Patient-reported Outcome Measure for a Combined Response Index for Limited Cutaneous SSc: The CRISTAL Project
Alain Lescoat¹, Yen Chen², Susan Murphy³, Nadia Vann², Neda Kortam², Rosemary Gedert², Sue Farrington⁴, Yannick Allanore⁵, David Cella⁶, Lorinda Chung⁷, Philip Clements⁸, Christopher Denton⁹, Francesco Del Galdo¹⁰, Oliver Distler¹¹, Monique Hinchcliff¹², Michael Hughes¹³, Laura Hummers¹⁴, John Pauling¹⁵, Janet Pope¹⁶, Virginia Steen¹⁷, John Varga², Peter Merkel¹⁸, Maya H. Buch¹⁹ and Dinesh Khanna², and all CRISTAL collaborators, ¹CHU Rennes - University Rennes 1, Rennes, France, ²University of Michigan, Ann Arbor, MI, ³University of Michigan, Plymouth, MI, ⁴Scleroderma & Raynaud UK, London, United Kingdom, ⁵Université Paris Cité, Paris, France, ⁶Northwestern University, Chicago, IL, ⁷Stanford University, Woodside, CA, ⁸United States, Los Angeles, CA, ⁹University College London, Northwood, United Kingdom, ¹⁰University of Leeds, Leeds, United Kingdom, ¹¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland, ¹²Yale School of Medicine, Westport, CT, ¹³Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, ¹⁴Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹⁵North Bristol NHS Trust, Bristol, United Kingdom, ¹⁶University of Western Ontario, London, ON, Canada, ¹⁷Georgetown University School of Medicine, Washington, DC, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Division of Musculoskeletal & Dermatological Sciences, University of Manchester, and NIHR Manchester Biomedical Research Centre, Manchester, United Kingdom
Background/Purpose: Limited cutaneous systemic sclerosis (lcSSc) is the most frequent subset of scleroderma, yet there is a paucity of outcome measures available to assess symptoms…
Abstract Number: 1580 • ACR Convergence 2024
Overt Polyautoimmunity and Its Risk Factors in a Multicenter Cohort of Systemic Sclerosis Patients
Irene Carrión-Barberà¹, Laura Tío², Laura Triginer², María Lee², Alfredo Guillén-del Castillo³, Anna Ribes², Lidia Valencia Muntalà⁴, Jordi Monfort¹, Tarek Carlos Salman Monte:⁵, Carmen PIlar Simeón-Aznar³, Anna Pros¹ and Javier Narvaez-García⁶, ¹Hospital del Mar, Barcelona, Spain, ²Hospital del Mar Research Institute, Barcelona, Spain, ³Hospital Vall d'Hebron, Barcelona, Spain, ⁴Hospital de Bellvitge, Barcelona, Spain, ⁵Hospital del Mar/Parc de Salut Mar-IMIM, Barcelona, Spain, ⁶Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: Overt PolyA (the clinical coexistence of ≥2 autoimmune diseases (AID) that fulfill classification criteria), is a frequent phenomenon in systemic sclerosis (SSc). However, the…
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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