Abstracts tagged "Fibroblasts, Dermal"

Abstract Number: 1181 • ACR Convergence 2022
Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis
Feiyang Ma¹, Pei-Suen Tsou¹, Grace Hile¹, John Varga¹, J. Michelle Kahlenberg¹, Allison Billi¹, Johann Gudjonsson¹ and Dinesh Khanna², ¹University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…
Abstract Number: 1183 • ACR Convergence 2022
Functionally Distinct Fibroblast Populations in Systemic Sclerosis Skin Revealed by Explant Tissue Characterization and Single-cell RNAseq
Kristina Clark¹, Alice Cole², Shiwen Xu¹, Voon Ong³, Christopher Buckley⁴ and Chris Denton¹, ¹University College London, London, United Kingdom, ²Royal Free Hospital, London, United Kingdom, ³UCL Medical School Royal Free Campus, London, United Kingdom, ⁴University of Oxford, Oxford, United Kingdom
Background/Purpose: Traditional studies of skin fibroblasts in SSc focus on the early "migratory" fibroblast population derived from explant culture of skin biopsies. Single-cell RNAseq has…
Abstract Number: 1184 • ACR Convergence 2022
Intracellular Calcium Signals Regulate the Pro-fibrotic Phenotype of Scleroderma Fibroblasts via Calcium/calmodulin-dependent Kinase II
Sirapa Vichaikul¹, William Brodie², Megan Mattichak², Qi Wu², Dinesh Khanna² and Eliza Pei-Suen Tsou², ¹Michigan Medicine, Howell, MI, ²University of Michigan, Ann Arbor, MI
Background/Purpose: We performed an in-depth analysis of the involvement of histone reader bromodomain extra-terminal proteins (BETs) in scleroderma (SSc) fibrosis and showed that JQ1, a…
Abstract Number: 1185 • ACR Convergence 2022
Interleukin-11 Promotes Fibrosis Through Classic and Trans-signaling Pathway in Systemic Sclerosis
Wenjing Ye¹, Qian Wang¹, Li Zhao², Changcheng Wang³, Dandan Zhang⁴, Mengyu Zhou⁴, Fangfang Chen², Zaihua Zhu², Weiguo Wang², Wenyu Guo⁵, Yun Liu⁴, Hejian Zou² and Yu Xue², ¹epartment of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China, ²Department of Rheumatology, Huashan Hospital, Fudan University, Shanghai, China, ³Department of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China, ⁴MOE Key Laboratory of Metabolism and Molecular Medicine, Department of Biochemistry and Molecular Biology, School of Basic Medical Sciences, Fudan University, Shanghai, China, ⁵Clinical Development, I-Mab Biopharma (Hangzhou) Co., Ltd, Shanghai, China
Background/Purpose: Interleukin-11 (IL-11) was found significantly upregulated in Systemic sclerosis (SSc), the aim of this study is to explore the pathological role of IL-11 and…
Abstract Number: 1624 • ACR Convergence 2022
Blocking IL-1, IL-33 and IL-36 Signaling with the Anti-IL1RAP Antibody mCAN10 Ameliorates Inflammation and Fibrosis in Preclinical Models of Systemic Sclerosis
Caitriona Grönberg¹, Sara Rattik¹, Meik Kunz², Thoung Trinh-Minh³, Cuong Tran-Manh³, Xiang Zhou³, Petter Skoog¹, David Liberg¹ and Jörg Distler³, ¹Cantargia AB, LUND, Sweden, ²Friedrich-Alexander University (FAU) Erlangen-Nuremberg, Erlangen, Germany, ³Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: The IL-1 receptor accessory protein (IL1RAP) is a co-receptor required for signaling through the IL-1, IL-33, and IL-36 receptors. IL1RAP-dependent signaling has been implicated…
Abstract Number: 0645 • ACR Convergence 2022
Lupus Fibroblasts from Non-lesional Skin Exhibit Exaggerated Responses to Inflammatory Cytokines and Upregulate Pro-fibrotic Collagens in Patients with Scarring Lesions
Suzanne Shoffner-Beck, Lisa Abernathy-Close, Stephanie Lazar, Amy Hurst, Craig Dobry, Deepika Pandian, Rachael Wasikowski, Kelly Arnold, Johann Gudjonsson, Lam Tsoi and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
Background/Purpose: Cutaneous lupus erythematosus (CLE) is a manifestation of systemic lupus erythematosus (SLE) that can cause significant patient distress and disfiguration secondary to scar. Scarring…
Abstract Number: 0661 • ACR Convergence 2022
Cutaneous Type I IFN Responses in Systemic Lupus Erythematosus Are Associated with Inflammatory Phenotype and Altered Wound Healing Function of Lupus Fibroblasts from Non-Lesional Skin
Lisa Abernathy-Close, Suzanne Shoffner-Beck, Annie Lu, Amanda Victory, Amy Hurst, Craig Dobry, Rachael Wasikowski, Johann Gudjonsson, Alex Tsoi and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
Background/Purpose: Cutaneous lupus erythematosus (CLE) is a heterogenous, disfiguring, and difficult-to-treat manifestation of systemic lupus erythematosus (SLE) with scar formation in CLE subtypes such as…
Abstract Number: 1065 • ACR Convergence 2022
Biosamples from VEDOSS Patients Show Pathological Signs of SSc: Opportunity for a Biololgical Diagnosis of Disease
Rebecca Ross¹, Emily Clarke¹, Will Merchant¹, Panji Mulipa¹, Natalia Riobo-DelGaldo¹ and Francesco Del Galdo², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: The VEDOSS study has recently indicated that more than 50% of patients affected by Raynaud’s phenomenon (RP) and specific SSc anti-nuclear antibodies (ANA) and/or…
Abstract Number: 1168 • ACR Convergence 2022
Dermal Fibroblast-derived Exosomes Drive Profibrotic Macrophage Activation in Systemic Sclerosis
Heetaek Yang¹, Rajan Bhandari¹, Noelle Kosarek², Jonathan Garlick³, Michael Whitfield⁴ and Patricia Pioli¹, ¹Geisel School of Medicine at Dartmouth, Lebanon, NH, ²Dartmouth Geisel School of Medicine, Lebanon, NH, ³Tufts University School of Dental Medicine, Boston, MA, ⁴Geisel School of Medicine, Lebanon, NH
Background/Purpose: Macrophage (MØ) activation derives from coordination of signals received in local tissue microenvironments. In prior studies, we demonstrated that cocultured MØs and fibroblasts from…
Abstract Number: 1170 • ACR Convergence 2022
Systemic Sclerosis Dermal Fibroblast-derived Exosomes Trigger a Type 1 Interferon Rresponse in Keratinocytes Through TBK1
Jessica Bryon¹, Christopher Wasson¹, Rebecca Ross¹, Elton Zeqiraj¹ and Francesco Del Galdo², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: Elevated Type I IFN response is present in blood and affected tissues in systemic sclerosis (SSc) and correlates with disease activity and response to…
Abstract Number: 1172 • ACR Convergence 2022
Transcription Analysis of Macrophages Reveals Important Changes Induced by Systemic Sclerosis Fibroblasts
Juan-Pablo Zertuche¹, Taha Dinc², Fatima El Adili³, Giovanni Ligresti¹ and Andreea Bujor¹, ¹Boston University, Boston, MA, ²Boston University School of Medicine, Boston, MA, ³Boston University School of Medicine, Revere, MA
Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune system activation, endothelial dysfunction and widespread tissue fibrosis. Activation of the monocyte/macrophage…
Abstract Number: 1176 • ACR Convergence 2022
Immunoglobulins G from Systemic Sclerosis Patients May Alter the Secretome of Dermal Fibroblasts
Aurélien Chepy¹, Marie Duhamel², Solange Vivier³, lucile Guilbert³, Eric Hachulla⁴, Sylvain Dubucquoi³, David Launay³, Michel Salzet² and Vincent Sobanski³, ¹CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest de France (CeRAINO), Lille, France, ²Univ. Lille, Inserm, CHU Lille, U1192 - Protéomique Réponse Inflammatoire Spectrométrie de Masse - PRISM, Lille, France, ³Univ. Lille, Inserm, CHU Lille, U1286 - INFINITE - Institute for Translational Research in Inflammation, Lille, France, ⁴University of Lille, LILLE, France
Background/Purpose: Autoantibodies (Aab) are frequent in systemic sclerosis (SSc).Recently, it has been shown that immunoglobulins G (IgG) from SSc promoted a proinflammatory and profibrotic phenotype…
Abstract Number: 0542 • ACR Convergence 2021
Functional Characterization of Glycoprotein Nonmetastatic Melanoma Protein B in Scleroderma Fibrosis
Pamela Palisoc¹, Leah Vaikutis¹, Ellen Model¹, Morgan Omara¹, Dinesh Khanna¹, Eliza Pei-Suen Tsou¹ and Amr Sawalha², ¹University of Michigan, Ann Arbor, MI, ²University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Glycoprotein nonmetastatic melanoma protein B (GPNMB) is widely expressed on stromal cells and immune cells. It is involved in various cell functions such as…
Abstract Number: 0544 • ACR Convergence 2021
Targeting CD13/aminopeptidase N as a Novel Therapeutic Approach for Scleroderma Fibrosis
Eliza Pei-Suen Tsou, M.Asif Amin, Phillip Campbell, Mikel Gurrea-Rubio, Morgan Omara, Ellen Model, Pamela Palisoc, Mustafa Ali, Sirapa Vichaikul, Jonatan Hervoso, Jeffrey Ruth, Dinesh Khanna and David Fox, University of Michigan, Ann Arbor, MI
Background/Purpose: Aminopeptidase N, also known as CD13, is a Zn2+-dependent membrane bound ectopeptidase widely expressed in mammalian cells including rheumatoid arthritis (RA) fibroblast-like synoviocytes (FLS),…
Abstract Number: 0551 • ACR Convergence 2021
Metabolic Intermediate Dimethyl-Alpha-Ketoglutarate Is a Novel Repressor of Pathogenic Myofibroblast Reprogramming and Skin Fibrosis in Systemic Sclerosis
Blaž Burja¹, Ishani Banik², Shervin Assassi³, Michael Whitfield⁴, J. Matthew Mahoney⁵, Andreja Erman⁶, Matija Tomšič⁷, Mitchell P. Levesque², Snežna Sodin-Šemrl⁷, Gabriela Kania⁸, Hubert Rehrauer⁹, Ziga Rotar⁷, Oliver Distler¹⁰, Katja Lakota¹¹ and Mojca Frank-Bertoncelj¹⁰, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich and Department of Rheumatology, University Medical Centre Ljubljana, Zurich, Switzerland, ²Department of Dermatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³University of Texas McGovern Medical School at Houston, Houston, TX, ⁴Geisel School of Medicine, Lebanon, NH, ⁵Center for Quantitative Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ⁶Institute for Cell Biology, University Medical Centre Ljubljana, Ljubljana, Slovenia, ⁷Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia, ⁸Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁹Functional Genomics Center Zurich, ETH Zurich and University of Zurich, Zurich, Switzerland, ¹⁰Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich/University of Zurich, Zurich, Switzerland, ¹¹Department of Rheumatology, University Medical Centre Ljubljana, Llubljana, Slovenia
Background/Purpose: Metabolic perturbations drive fibroblast-to-myofibroblast reprogramming and tissue fibrosis. Restoring perturbed metabolism might represent a new antifibrotic strategy. Here we explored the capacity of dimethyl-alpha-ketoglutarate…

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