Abstracts tagged "Fibroblasts, Dermal"

Abstract Number: 0949 • ACR Convergence 2025
The Cancer-associated Female-biased Factor VGLL3 Drives Autoimmunity and Fibrosis
Mehrnaz Gharaee-Kermani¹, Poulami Dey², Vincent van Drongelen¹, Joanna Rew¹, Rachael Bogle³, Marisa Hildebrandt¹, Benjamin Klein¹, Rezvan Moallemian¹, Monique E Verhaegen¹, Andrzej A Dlugosz⁴, Lam Tsoi¹, J. Michelle Kahlenberg¹, Johann Gudjonsson¹ and Allison C Billi¹, ¹University of Michigan, Ann Arbor, MI, ²Michigan Medicine, Ann Arbor, MI, ³University of Michigan, Holland, OH, ⁴University of Michigan, Ann Arbor
Background/Purpose: Autoimmune disease is far more common in women than men. We have determined that the skin of healthy women is primed for autoimmunity due…
Abstract Number: 0679 • ACR Convergence 2025
Inhibition of TGFb3 in Systemic Sclerosis Patients Does Not Result in TGFb Pathway Modulation in Skin Biopsies or Circulation
Parisa Mazrooei, Xuting Rebecca Sheng, Xiaoyun Yang, Lyrialle Han, Samira Jamalian, Daniel Repplinger, Jingxuan He, Evelin Logis, Jeongsup Shim, Angela Hendricks, Lena Wang, Andrew Thorley and Sara Glickstein, Genentech/Roche, South San Francisco
Background/Purpose: There are 3 isoforms of Transforming Growth Factor Beta (TGFb), a cytokine frequently upregulated in fibrosis. Chronic targeting of TGFb1 and TGFb2 for fibrotic…
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: 2048 • ACR Convergence 2025
DDR2 auto-phosphorylation in patients with Warburg-Cinotti syndrome promotes differentiation of fibroblasts into myofibroblasts and expansion of immature B cells with antibody production.
Sang Jin LEE and Yun Jeong Lee, Kyungpook National University School of Medicine, Daegu, Republic of Korea
Background/Purpose: Warburg-Cinotti syndrome (WCS) is a rare connective tissue disorder caused by activating mutations in the DDR2 gene, characterized by progressive corneal neovascularization, keloid-like plaques,…
Abstract Number: 1870 • ACR Convergence 2025
Paracrine WNT Signaling Modulates Profibrotic Macrophage Metabolic Activation in Systemic Sclerosis
emily Morris¹, Helen Jarnagin¹, Chen-Yu Wang¹, Michael Whitfield² and Patricia Pioli³, ¹Dartmouth College, Lebanon, NH, ²Geisel School of Medicine, Lebanon, NH, ³Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: An immune fibrotic axis consisting of activated macrophages (MØs) and fibroblasts has been identified in autoimmune systemic sclerosis (SSc) that drives disease across affected…
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: 0975 • ACR Convergence 2025
Linezolid prevents fibroblast activation and ameliorates tissue fibrosis by inhibition of mitochondrial translation
Xuezhi Hong¹, Yanhua Xiao², shihao zhu³, Tim Filla⁴, Andrea-Hermina Györfi⁵, Yi-Nan Li⁶, Meilin Xu⁷, Langxian Zhi², Thuong Trinh-Minh⁸, Clara Dees⁹, Georg Schett¹⁰, Jörg Distler¹¹ and Alexandru-Emil Matei¹², ¹Department of Rheumatology, University Hospital Dusseldorf, Medical Faculty of Heinrich Heine University, Dusseldorf, Germany, Düsseldorf, Germany, ²Medical Faculty of Heinrich Heine University, Dusseldorf, Germany, ³Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany, ⁴Department 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, ⁵Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University., Düsseldorf, Germany, ⁶University Hospital of Düsseldorf, Düsseldorf, Germany, ⁷Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany, Düsseldorf, Germany, ⁸Clinic 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, ⁹Department of Internal Medicine ³, Rheumatology and Clinical Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ¹⁰Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, ¹¹University Hospital Duesseldorf and HHU, Duesseldorf, Germany, ¹²Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer Cluster of Excellence for Immune Mediated Diseases CIMD, Frankfurt am Main, Germany, Düsseldorf, Germany
Background/Purpose: Systemic sclerosis (SSc) is a progressive fibrotic disease characterized by fibroblast activation and immune dysregulation, with limited therapeutic options. Mitochondrial dysfunction is increasingly implicated…
Abstract Number: 0971 • ACR Convergence 2025
Unraveling the role of the hippo pathway in systemic sclerosis: A focus on TEADs and VGLL3
Neha Khanna¹, Alyssa Rosek¹, Alexander Cai¹, Olesya Plazyo², Johann Gudjonsson², Dinesh Khanna² and Pei-Suen Tsou², ¹Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, ²University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by excessive collagen production, leading to the thickening and hardening of skin and internal organs.…
Abstract Number: 0970 • ACR Convergence 2025
Genomic instability in systemic sclerosis is promoted by metabolic remodelling via a FOXO1-dependent axis
Lamia Khan¹, Junqin Wang², Aishwarya Iyer², Desiree Redmond², Dylan Hennessey², Sandra O'Keefe², Jan Storek³, Charmaine van Eeden², Robert Gniadecki² and Mohammed Osman¹, ¹University of Alberta, Edmonton, AB, Canada, ²Department of Medicine, University of Alberta, Edmonton, AB, Canada, ³University of Calgary, Calgary, AB, Canada
Background/Purpose: Systemic sclerosis (SSc) is a life-threatening autoimmune disease with limited treatment options, including autologous hematopoietic stem cell transplantation (AHSCT). We recently showed that dermal…
Abstract Number: 0969 • ACR Convergence 2025
FOXO1 Mediated Polysialic Acid Dysregulation in Severe Systemic Sclerosis (SSc): A Novel Biomarker and Therapeutic Target?
Lamia Khan¹, Lisa Willis², Jan Storek³, Desiree Redmond⁴, Robert Gniadecki⁴ and Mohammed Osman¹, ¹University of Alberta, Edmonton, AB, Canada, ²Department of Biological Sciences, University of Alberta, Edmonton, AB, Canada, ³University of Calgary, Calgary, AB, Canada, ⁴Department of Medicine, University of Alberta, Edmonton, AB, Canada
Background/Purpose: Systemic sclerosis (SSc) is a life-threatening autoimmune disease where fibroblasts (FB) contribute to disease severity by resisting apoptosis and driving excessive skin fibrosis. Among…
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: 0966 • ACR Convergence 2025
Integrated Bulk and Single Cell Analysis Confirms Differential Upregulation of the Proton Sensing Receptor GPR68 in Systemic Sclerosis Across Disease Stage and Subset
kristina clark¹, Xu Shiwen², Xue Li², Voon H. Ong³ and Christopher Denton⁴, ¹Kennedy Institute of Rheumatology, University of Oxford, London, United Kingdom, ²University College London, London, United Kingdom, ³Division of Medicine, Centre for Rheumatology, University College London, London, United Kingdom, ⁴University College London, UK, London, United Kingdom
Background/Purpose: The cell surface protein GPR68 (ovarian cancer G protein-coupled receptor 1, OGR1) is a proton-sensing G-protein-coupled receptor (GPCR) that responds to extracellular acidity and…
Abstract Number: 0967 • ACR Convergence 2025
Single-Cell RNA Sequencing Reveals a Prominent Pro-Inflammatory Gene Signature of Dermal Fibroblasts in Pre-Stages of SSc
Lumeng Li¹, Elena Pachera², Rucsandra Dobrota³, Sinziana Muraru⁴, Kristina Bürki³, Carina Mihai⁵, Muriel Elhai⁶, Laura Much⁷, Astrid Hofman³, Pietro Bearzi¹, Anna-Maria Hoffmann-Vold⁸ and Oliver Distler⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, the LOOP Zurich, Zürich, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, the LOOP Zurich, Zurich, Switzerland, ⁴University of Zurich, University Hospital Zurich, Zürich, Switzerland, ⁵University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁶University Hospital zurich, Zürich, Switzerland, ⁷Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁸Oslo University Hospital, Oslo, Norway, ⁹Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: The poor prognosis of SSc patients demands an urgent need to prevent disease onset. Recently, we reported a global pro-inflammatory gene signature of dermal…
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: 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…

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