Abstracts tagged "Systemic sclerosis"

Abstract Number: 2500 • ACR Convergence 2025
Greater Economic Burden and Healthcare Resource Utilization in Patients with Systemic Sclerosis With Versus Without Interstitial Lung Disease: Results from a Systematic Literature Review
Emily Glowienka¹, Sarah Nickolls², Kevin Coady¹, Eesha Kodi³, Ian Steinfield¹, Florence Wilson³, Alicia N. Pepper³, Svetlana I. Nihtyanova⁴, Roger A. Levy⁵ and Daniel Moldaver⁶, ¹Precision AQ, Boston, MA, ²GSK, Research & Development, Stevenage, United Kingdom, ³Precision AQ, Vancouver, BC, Canada, ⁴GSK, Clinical Sciences, Research & Development, London, United Kingdom, ⁵GSK, Specialty Care, Global Medical Affairs, Collegeville, PA, ⁶GSK, Global Real-World Evidence & Health Outcomes Research, Toronto, ON, Canada
Background/Purpose: SSc is a rare autoimmune connective tissue disorder; up to 64% of patients with SSc develop interstitial lung disease (ILD), which can result in…
Abstract Number: 2474 • ACR Convergence 2025
Unveiling the clinical spectrum of ACA-positive SSc-ILD: not as benign as expected
Carlos Valera Ribera¹, JUAN JOSE ALEGRE SANCHO², Ivan Castellvi³, Marta Ibañez⁴ and Javier Narváez⁵, ¹Hospital Universitario Doctor Peset, València, Spain, ²Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ³Hospital Universitari de la Santa Creu i Sant Pau, Sant Just Desvern, Spain, ⁴Hospital Universitario de la Santa Creu i Sant Pau, Barcelona, Catalonia, Spain, ⁵Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: Anticentromere antibody (ACA)-positive interstitial lung disease (ILD) in systemic sclerosis (SSc) is traditionally considered less aggressive than anti-topoisomerase I (ATA)-positive ILD. However, its clinical…
Abstract Number: 1864 • ACR Convergence 2025
Mitochondrial Dysfunction Drives cGAS-STING–Mediated Type I Interferon Production and Fibrosis in Systemic Sclerosis
Giulio Forte¹, Vasiliki Liakouli¹, Alessia Salzillo², Mario Angeli³, Daniele Mauro¹, Antonio Ciancio¹, Barbara De Marino², Iacopo Panarese¹, Mario Faenza¹, roberto giacomelli⁴, Andreas Ramming⁵ and Francesco Ciccia⁶, ¹Università della Campania Luigi Vanvitelli, Naples, Italy, ²University of Campania L. Vanvitelli, Naples, Italy, ³Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, ⁴Università Campus Bio-Medico, Roma, Italy, ⁵Department of Internal Medicine ³, Rheumatology & Immunology, Friedrich-Alexander-University (FAU) & Universitätsklinikum Erlangen, Erlangen, Germany, ⁶Rheumatology Section, University of Campania L. Vanvitelli, Naples, Italy, Naples, Italy
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by type I interferon (IFN-I) production and mitochondrial dysfunction. Emerging evidence suggests that activation of the…
Abstract Number: 1573 • ACR Convergence 2025
Health-related quality of life over 15 years in systemic sclerosis: impact of sex and survival
Katherine van der Wouden¹, Georgy Gomon², Rachel Knevel², Michel Tsang-A-Sjoe³, Alexandre Voskuijl³ and Jeska de Vries-Bouwstra², ¹Leiden University Medical Center and Amsterdam University Medical Center, Leiden, Zuid-Holland, Netherlands, ²Leiden University Medical Center, Leiden, Netherlands, ³Amsterdam University Medical Center, Amsterdam, Netherlands
Background/Purpose: Patients with systemic sclerosis (SSc) face an uncertain long‑term outlook; understanding how their health‑related quality of life (HRQoL) changes over time can help them…
Abstract Number: 1161 • ACR Convergence 2025
Comparative efficacy, safety and tolerability of antifibrotic therapies across systemic autoimmune diseases
Javier Narváez¹, Orfa Barrios², Pol Maymó-Paituvi³, JUAN JOSE ALEGRE SANCHO⁴, Ivan Castellví⁵, Vanesa Vicens Zygmunt⁶, Guadalupe Bermudo⁷, Laia De Daniel Bisbe³, Martí Aguilar-Coll⁸, Montserrat Roig Kim³, Joan Miquel Nolla³ and Maria Molina-Molina⁷, ¹Hospital Universitario de Bellvitge, Barcelona, Spain, ²Department of Rheumatology. Hospital Nacional Arzobispo Loayza, Lima, Peru, ³Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain, ⁴Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ⁵Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, barcelona, Spain, ⁶Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge, Barcelona, Spain, ⁷Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge., Barcelona, Spain, ⁸Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: To evaluate the efficacy, safety, and tolerability of the antifibrotic agents nintedanib and pirfenidone in the treatment of interstitial lung disease (ILD) associated with…
Abstract Number: 0961 • ACR Convergence 2025
Retinoic acid-related orphan receptor-α: A novel upstream regulator of Hippo signaling and potential therapeutic target in fibrosis
Cuong Tran-Manh¹, Thuong Trinh-Minh², Christoph Liebel³ and Jörg Distler⁴, ¹Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University., Düsseldorf, Nordrhein-Westfalen, Germany, ²Clinic 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, Nordrhein-Westfalen, Germany, ³Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University., Düsseldorf, Germany, ⁴University Hospital Duesseldorf and HHU, Duesseldorf, Germany
Background/Purpose: Retinoic acid-related orphan receptor-α (RORα) is a nuclear transcription factor implicated in immune regulation, circadian rhythm, and metabolism. However, its role in fibrotic diseases…
Abstract Number: 0688 • ACR Convergence 2025
Total lung capacity is predictive of disease severity and survival in systemic sclerosis: a longitudinal analysis in 2347 patients from the French National Cohort Study
Benjamin Chaigne¹, Alexandre bense², Frédérique Aubourg³, Christian AGARD⁴, Yannick Allanore⁵, Alice berezne¹, Gregory Pugnet⁶, Eric Hachulla⁷, Vincent Cottin⁸, Arnaud Hot⁸, bertrand Dunogue¹, Anuxcy Kanagaratnam², Sylvain Palat⁹, Alain Lescoat¹⁰, Sabine berthier¹¹, Emmanuel Chatelus¹², Sébastien Rivière¹³, David Launay¹⁴, Marie-Elise Truchetet¹⁵, Anh-Tuan Dinh-Xuan³ and Luc Mouthon¹, ¹Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin University Hospital, Université Paris Cité, AP-HP, Paris, France, ²AP HP, Paris, France, ³Unité exploration fonctionnelle respiratoire, Pneumology department, Cochin Hospital, APHP, PARIS, France, ⁴Internal medicine, Nantes University Hospital, Nantes, France, ⁵Université Paris Cité, Paris, France, ⁶CHU, Montpelliers, France, ⁷CHU, Lilles, France, ⁸CHU, Lyon, France, ⁹Limoges, Limoges, France, ¹⁰CHU Rennes - University Rennes, Rennes, France, ¹¹CHU, Dijon, France, ¹²Department of Rheumatology, University Hospital Strasbourg, France, Strasbourg, France, ¹³AP HP, Parsi, France, ¹⁴Univ. Lille, Inserm, CHU Lille, Department of Internal Medicine and Clinical Immunology, Reference Center for Rare Autoimmune and Autoinflammatory diseases (CERAINOM), U¹²⁸⁶ - INFINITE - Institute for Translational Research in Inflammation, Lille, France. National Reference Center for Pulmonary Arterial Hypertension (Pulmotension), Lille, France, Lille Cedex, France, ¹⁵Bordeaux University Hospital, Bordeaux, France
Background/Purpose: Total lung capacity (TLC) is seldom assessed in the prediction of systemic sclerosis (SSc) disease severity.Herein, we utilized the French SSc national database to…
Abstract Number: 0884 • ACR Convergence 2025
Risk Score for Early Mortality to stratify for Intensive SSc Therapy
Ann-Christin Pecher¹, Boubaya Marouane², Oliver Distler³, Vanessa Smith⁴, Jeska de Vries-Bouwstra⁵, Radim Bečvář⁶, Gianluca Moroncini⁷, David Launay⁸, Yannick Allanore⁹, Maria De Santis¹⁰, Kamal Solanki¹¹, Carlomaurizio Montecucco¹², Luca Idolazzi¹³, Nihal Fathi¹⁴, Przemyslaw Kotyla¹⁵, Muriel Elhai¹⁶ and Jörg Henes¹⁷, ¹Department of Internal Medicine II, Hematology, Oncology, Clinical Immunology, and Rheumatology, University Hospital Tübingen, Tübingen, Germany, ²Unit of Clinical Research, Paris Seine Saint Denis University, Bobigny, France, Paris, France, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland, ⁴Ghent University Hospital, Gent, Belgium, ⁵Leiden University Medical Center, Leiden, Netherlands, ⁶Department of Internal Medicine, Charles University,, Prague, Czech Republic, ⁷Department of Internal Medicine, Marche University Hospital, Clinica Medica, Ancona, Italy, Ancona, Italy, ⁸Univ. Lille, Inserm, CHU Lille, U¹²⁸⁶ – INFINITE – Institute for Translational Research in Inflammation, Lille, France ; CHU Lille, Département de Médecine interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM), Lille France, Lille, France, ⁹Department of Rheumatology, Université Paris Cité UFR de Médecine, Paris, France, ¹⁰Humanitas University, Pieve Emanuele, Italy, ¹¹Waikato Hospital, Hamilton, New Zealand, ¹²Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy, Pavia, Italy, ¹³Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy, Verona, Italy, ¹⁴Department of Rheumatology ,Rehabilitation & physical medicine, Assiut University Hospital, Assiut, Egypt, Assiut, Egypt, ¹⁵Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland, ¹⁶University Hospital zurich, Zürich, Switzerland, ¹⁷Department of Internal Medicine II, Hematology, Oncology, Clinical Immunology, and Rheumatology, University Hospital Tübingen, Tuebingen, Germany
Background/Purpose: Systemic sclerosis (SSc) is a rare disease that often leads to severe complications and premature mortality. Recent advancements in the field have led to…
Abstract Number: 0032 • ACR Convergence 2025
Protein Language Model-Guided Homology Identifies Microbial Enzymes Linked to Fibrosis-Prone IgG4-RD and Crohn’s Disease
Kumar Thurimella¹, Ahmed Mohamed², Chenhao Li³, Tommi Vatanen⁴, Daniel Graham³, Roisin Owens⁵, Sabina Leanti La Rosa⁶, Damian Plichta³, Sergio Bacallado⁵ and Ramnik Xavier⁷, ¹University of Colorado School of Medicine, Aurora, CO, ²Broad Institute, Boston, ³Broad Institute, Cambridge, MA, ⁴University of Helsinki, Helsinki, Finland, ⁵University of Cambridge, Cambridge, United Kingdom, ⁶NMBU, As, Norway, ⁷Harvard Medical School, Boston, MA
Background/Purpose: Uncharacterized microbial enzymes in metagenomics are difficult to annotate, especially in fibrosis-prone conditions like IgG4-related disease (IgG4-RD) and Crohn’s disease (CD), where microbial carbohydrate…
Abstract Number: 2498 • ACR Convergence 2025
Effect of autologous myeloablative hematopoietic stem cell transplantation on skin and peripheral blood mononuclear cells in systemic sclerosis patients with interstitial lung disease
Poulami Dey¹, Rachael Bogle², Qinmengge Li³, Olesya Plazyo⁴, Rosemary Gedert⁴, Carleigh Zahn⁴, Pei-Suen Tsou⁴, John Varga⁴, Lam Tsoi⁴, Johann Gudjonsson⁴ and Dinesh Khanna⁴, ¹Michigan Medicine, Ann Arbor, MI, ²University of Michigan, Holland, OH, ³University of Michigan, Ypsilanti, MI, ⁴University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is characterized by skin fibrosis, dysregulated immune response, and vascular system dysfunction. Interstitial lung disease (ILD) is one of the leading…
Abstract Number: 2473 • ACR Convergence 2025
Nailfold Capillaroscopy Improves Cardiovascular Risk Stratification in Systemic Sclerosis: An Adjustment to the SCORE2 Algorithm
Carlos Valera Ribera¹, Javier Narváez², Joaquin Lacasa Molina³, Adrián Mayo-Juanatey⁴, Paula Mencio⁵, Patricia Leon Torralba⁵, Pablo Andujar Brazal⁵, Ignacio Vázquez Gómez⁶ and JUAN JOSE ALEGRE SANCHO⁷, ¹Hospital Universitario Doctor Peset, València, Spain, ²Hospital Universitario de Bellvitge, Barcelona, Spain, ³Hospital Universitario Doctor Peset, Valencia, Spain, ⁴Hospital Universitario Doctor Peset, Valencia, Comunidad Valenciana, Spain, ⁵Hospital Universitario Doctor Peset, València, Comunidad Valenciana, Spain, ⁶H.U. Doctor Peset, Valencia, ⁷Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain
Background/Purpose: Systemic sclerosis (SSc) is associated with both micro- and macrovascular damage, increasing cardiovascular (CV) risk. The SCORE2 algorithm estimates CV event risk in the…
Abstract Number: 1863 • ACR Convergence 2025
Bag3 in systemic sclerosis: possible therapeutic target and biomarker for pulmonary fibrosis
Claudia Iannone¹, Margot De Marco², Antonina Minniti³, Giuseppe Armentaro⁴, Antonia Falco², Angela D'Ardia², paola Manzo², Liberato Marzullo⁵, Anna Basile², Alessandra Rosati², Maria Caterina Turco², Roberto Caporali⁶ and Nicoletta Del Papa⁷, ¹University of Milan, Gaetano Pini CTO, Milano, Milan, Italy, ²Department of Medicine, Surgery and Dentistry "Schola Medica Salernitana", University of Salerno, Baronissi, Italy, Baronissi, Campania, Italy, ³ASST Gaetano Pini, milano, Milan, Italy, ⁴ASST Pini-CTO, Clinical Rheumatology Unit, Milano, Italy, ⁵Department of Medicine, Surgery and Dentistry "Schola Medica Salernitana", University of Salerno, Baronissi, Italy, Baronissi, Italy, ⁶University of Milan and ASST Gaetano Pini-CTO, Milano, Italy, ⁷ASST Pini CTO, Milano, Milan, Italy
Background/Purpose: BAG3 (Bcl2-associated athanogene 3) regulates cellular pathways including apoptosis and autophagy, and induces fibroblast-to-myofibroblast transformation. In systemic sclerosis (SSc), myofibroblasts drive fibrosis of skin…
Abstract Number: 1571 • ACR Convergence 2025
Spatial Transcriptomics of Perivascular Subepidermal Regions in Very Early Systemic Sclerosis Unveils Cellular and Mitochondrial Stress-Driven Innate Immune Signatures that Initiate Stromal Remodeling
Ifeoluwa Emmanuel Bamigbola¹, Jayakumar Vadakekolathu¹, Stefano Di Donato², Vishal Kakkar³, Rebecca Ross⁴, Yasser El-Sherbiny¹ and Francesco Del Galdo³, ¹¹Nottingham Trent University, Department of Biosciences, School of Science and Technology, Nottingham, United Kingdom, ²University of Leeds, Canosa Sannita, Chieti, Italy, ³University of Leeds, Leeds, United Kingdom, ⁴Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: The skin from patients with Very Early Diagnosis of Systemic Sclerosis (VEDOSS) already exhibits fibrotic alterations such as collagen deposition and perivascular infiltration (1),…
Abstract Number: 1079 • ACR Convergence 2025
Sociodemographic, Clinical, and Community-Level Deprivation Factors are Associated with Health-Related Quality of Life in Systemic Sclerosis
Sarah Smith¹, Paula Ramos², Dulaney Wilson³, Diane Kamen⁴ and Richard Silver¹, ¹Medical University of South Carolina, Charleston, SC, ²Emory University School of Medicine, Atlanta, GA, ³Medical University of South Carolina, Charleston, ⁴Medical University of South Carolina, Johns Island, SC
Background/Purpose: Systemic sclerosis (SSc) is a multisystem autoimmune disease with heterogeneous manifestations and significant impairment in health-related quality of life (HRQoL). SSc outcomes are shaped…
Abstract Number: 0959 • ACR Convergence 2025
Longitudinal Multiomic Study of Skin, Peripheral Blood, and Serum: Serum Proteome Reflects the Disease Process at the End-organ Level and Predicts the Course of Modified Rodnan Skin Score
Bich Na Choi¹, Claudia Pedroza², Brian Skaug³, Maureen Mayes⁴ and Shervin Assassi⁵, ¹UTHealth Houston Institute for Clinical Research & Learning Health Care, Houston, TX, ²UTHealth Houston Institute for Clinical Research & Learning Health Care, Texas, TX, ³UTHealth Houston Division of Rheumatology, Houston, TX, ⁴UT Health Houston Division of Rheumatology, Houston, TX, ⁵Division of Rheumatology, UTHealth Houston, Houston, Texas, USA, Houston, TX
Background/Purpose: Clinically useful biomarkers for systemic sclerosis (SSc) are needed. While obtaining samples from affected organs like the skin and lungs is invasive and cannot…

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