Abstracts tagged "Scleroderma, Systemic"

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: 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: 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: 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: 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: 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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