Abstracts tagged "Fibrosing syndromes"

Abstract Number: 1663 • ACR Convergence 2024
Notch—mediated Fibrogenic Activation Is a Spatial Determinant of RA Treatment Response
Kartik Bhamidipati¹, Ce Gao¹, Ksenia Anufrieva², Shideh Kazerounian³, Roopa Madhu⁴, Miles Tran¹, Vikram Khedgikar¹, Sonia Presti¹, Anna Helena Jonsson⁵, ilya Korsunsky¹ and Kevin Wei⁶, ¹Brigham and Women's Hospital, Boston, MA, ²BWH, Cambridge, MA, ³Brigham & Women's Hospital, Boston, MA, ⁴Brigham and Women's Hospital, Brookline, MA, ⁵University of Colorado School of Medicine, Aurora, CO, ⁶Brigham and Women's Hospital at Harvard Medical School, Boston, MA
Background/Purpose: In RA, poor treatment response has been linked to a fibroid synovial tissue phenotype, characterized by lack of immune cell infiltration and an expansion…
Abstract Number: 2027 • ACR Convergence 2024
Clinical Features and Treatment of Retroperitoneal Fibrosis in a Referral Hospital and Literature Review
Adrián Martín-Gutiérrez¹, Javier Loricera², Fernando Lopez-Gutierrez³ and Ricardo Blanco-Alonso⁴, ¹Hospital Universitario Marques de Valdecilla, IDIVAL, Renedo de Piélagos, Cantabria, Spain, ²Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ³Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Cantabria, Spain, ⁴Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: Retroperitoneal fibrosis (RPF) is usually idiopathic (iRPF), but may be secondary to drugs, neoplasms, infections, or may be part of an IgG4-related disease. Our…
Abstract Number: 2661 • ACR Convergence 2024
Changes in Krebs Von Den Lungen 6 Levels (∆KL6) Predict Fibrosing Progression of Interstitial Lung Disease in Patients with an Underlying Connective Tissue Disease (CTD-ILD)
Fredeswinda Romero-Bueno¹, María Carmen Vegas-Sánchez², lydia Abasolo Alcazar³, Mª Jesus Rodriguez Nieto², Cristina Vadillo-Font⁴, M Asuncion Nieto⁵, Laura Cebrián⁶, Belén López-Muñiz⁶, Jesus Loarce Martos⁷, Juan Rigual⁸, hildegarda Godoy Tundidor⁹, Rosalía Laporta¹⁰, Irene Llorente-Cubas¹¹, Gema Bonilla¹², Luis Gomez Carrera¹³, Rosario García-Vicuña¹⁴, Ana Jaureguizar⁷, Jose Luis Morell Hita⁷, Claudia Valenzuela¹⁵ and Olga Sánchez-Pernaute¹⁶, and the NEREA Study group for Autoimmune ILD, ¹Hospital Universitario Fundación Jiménez Díaz and IIS-FJD. Autonomous University of Madrid, Madrid, Spain, ²Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, ³IdISSC. HCSC, Madrid, Spain, ⁴Hospital Clinico S Carlos, Madrid, Spain, ⁵Hospital Clínico San Carlos, Madrid, Madrid, Spain, ⁶Hospital Universitario Infanta Leonor, Madrid, Spain, ⁷Hospital Universitario Ramón y Cajal - IRYCIS, Madrid, Spain, ⁸Ramón y Cajal University Hospital, Madrid, Madrid, Spain, ⁹Servicio de Reumatología, Hospital Universitario Puerta de Hierro Majadahonda, IDIPHISA , Madrid, Madrid, Spain, ¹⁰Hospital Universitario Puerta de Hierro, Madrid, Spain, ¹¹Hospital Universitario de La Princesa, Madrid, Spain, ¹²H. Universitario La Paz, Madrid, Spain, ¹³Hospital Universitario La Paz, Madrid, Spain, ¹⁴Hospital Universitario de la Princesa, Madrid, Madrid, Spain, ¹⁵Hospital Universitario La Princesa, Madrid, Spain, ¹⁶Department of Rheumatology and Joint and Bone Research Unit. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD. Autonomous University of Madrid, Madrid, Spain
Background/Purpose: Krebs von den Lungen-6 (KL6) is a mucin-1 glycoprotein produced by type 2 alveolar epithelial cells , which increase at peripheral blood show a…
Abstract Number: 2686 • ACR Convergence 2024
Dose Escalation Safety Study of Brentuximab Vedotin for Diffuse Cutaneous Systemic Sclerosis:Clinical Results and Mechanistic Analysis of Skin and Peripheral Blood Mononuclear Cells
David Fox¹, Laura Cooney², Lia Stelzig³, Robert Lafyatis⁴, Johann Gudjonsson⁵, Lam Tsoi⁶, Maureen Mayes⁷, Ankoor Shah⁸, Suzanne Kafaja⁹, William Barry³, Ellen Goldmuntz¹⁰, Dawn Smilek¹¹ and Dinesh Khanna⁶, ¹University of Michigan, Dexter, MI, ²University of Michigan and Immune Tolerance Network, Ann Arbor, MI, ³Rho, Durham, NC, ⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, ⁵Department of Dermatology, University of Michigan, Ann Arbor, MI, ⁶University of Michigan, Ann Arbor, MI, ⁷UTHealth Houston Division of Rheumatology, Houston, TX, ⁸Duke University, Durham, NC, ⁹Division of Rheumatology, University of California, Los Angeles, Los Angeles, CA, ¹⁰Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, Rockville, MD, ¹¹Immune Tolerance Network, UCSF, San Francisco, CA
Background/Purpose: Brentuximab vedotin (ADCETRIS®) is a drug antibody conjugate targeting CD30 approved for treatment of Hodgkin’s lymphoma (HL) and other hematologic malignancies. Association of CD30…
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: 0785 • ACR Convergence 2024
Exploring the Link Between Atgl-Dependent Lipolysis and Dermal Fibrosis in Systemic Sclerosis
Elizabeth Caves¹, Agrani Dixit¹, Anna Jussila², Vivian Lei³, Hailey Edelman⁴, Muhammad Hamdan⁵, Ian Odell⁵, Monique Hinchcliff⁶, Radhika Atit⁷ and Valerie Horsley¹, ¹Yale School of Medicine, New Haven, CT, ²Stanford Medicine, Stanford, CA, ³University of North Carolina School of Medicine, Chapel Hill, ⁴Vanderbilt School of Engineering, New Haven, ⁵Yale School of Medicine, New Haven, ⁶Yale School of Medicine, Westport, CT, ⁷Case Western Reserve University College of Arts and Sciences, Cleveland
Background/Purpose: Resident lipid-filled dermal adipocytes are depleted in both systemic sclerosis (SSc) and scleroderma mouse models, but mechanisms are poorly understood. We undertook studies in mouse…
Abstract Number: 0956 • ACR Convergence 2024
Secreted Frizzled-Related Protein 2 (sFRP2) Regulates Wnt Signaling to Affect Mesenchymal Transition of Lung Epithelial Cells Participates in Interstitial Lung Disease
Yinlan Wu¹, Yanhong Li² and Yi Liu³, ¹West China Hospital of Sichuan University, Chengdu, Sichuan, China, ²West China School of Medicine and West China Hospital, Sichuan University, Cheng Du, Sichuan, China, ³West China Hospital, Sichuan University, Chengdu, China (People's Republic)
Background/Purpose: Investigation of Transcriptional Changes in Interstitial Lung Disease (ILD) and Exploration of Functional Effects of Differential Genes and Expressed Proteins in the Development of…
Abstract Number: 0713 • ACR Convergence 2023
IgG4-Related Disease as a Potencial Etiology of Idiopathic Constrictive Pericarditis
Edwin Ulises Cortez-Domínguez¹, Gabriela Hernandez-Molina¹, Norma Ofelia Uribe-Uribe², María J Lizardo-Thiebaud² and Eduardo Martin-Nares¹, ¹Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ²Department of Pathology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Background/Purpose: Constrictive pericarditis (CP) is a type of diastolic heart failure characterized by impaired cardiac filling due to the presence of an inelastic pericardium. Histologically,…
Abstract Number: 0935 • ACR Convergence 2023
Sodium Pyruvate Improves Mitochondrial Fitness in SSc Fibroblasts to Prevent Fibroblast-to-myofibroblast Transition and Fibrotic Remodeling
Xiang Zhou¹, Thuong Trinh-Minh¹, Alexandru-Emil Matei¹, Honglin Zhu², Hermina Györfi¹, Cuong Tran Manh¹, Xuezhi Hong¹ and Joerg Distler¹, ¹Clinic 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, Germany, ²3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany. 5 Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China
Background/Purpose: Systematic sclerosis (SSc) is an autoimmune disease characteristic by fibroblast transition and overproduction of extracellular matrix, yet with limited treatment. Recent studies provided evidence…
Abstract Number: 0945 • ACR Convergence 2023
The Nuclear Receptor TR4 Orchestrates Cytoskeletal Organization in a Gα12/ROCK-dependent Manner to Promote Myofibroblast Differentiation and Tissue Fibrosis in Systemic Sclerosis
Yun Zhang¹, Lichong Shen², yi-nan Li¹, Hermina Györfi¹ and Joerg Distler¹, ¹Clinic 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, Germany, ²Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany
Background/Purpose: Members of the superfamily of nuclear receptors have been implicated in inflammatory processes and pathologic tissue remodeling and have emerged as attractive targets for…
Abstract Number: 0957 • ACR Convergence 2023
Rationale for Targeting Insulin-like Growth Factor Signalling in Systemic Sclerosis
Voon Ong¹, Shiwen Xu², Li Xue², Brajesh Kumar³ and Christopher Denton², ¹UCL Medical School Royal Free Campus, London, United Kingdom, ²University College London, London, United Kingdom, ³Horizon Therapeutics, South San Francisco, CA
Background/Purpose: Previous studies have shown that insulin like growth factor (IGF) pathways may promote extracellular matrix deposition and be upregulated in systemic sclerosis (SSc). Teprotumumab,…
Abstract Number: 0962 • ACR Convergence 2023
Topological Proteomic Differences in Fibrotic and Unaffected Skin in Scleroderma
Irene Choi¹, Courtney Yu², Francesco Boin¹, Elizabeth Volkmann³, Stephanie Stanford² and Nunzio Bottini⁴, ¹Cedars-Sinai Medical Center, Los Angeles, CA, ²University of California San Diego, San Diego, CA, ³University of California Los Angeles, Los Angeles, CA, ⁴University of California, San Francisco, CA
Background/Purpose: Scleroderma (SSc) is a chronic autoimmune and rheumatic disease characterized by varying levels of tissue fibrosis in the skin and other internal organs. It…
Abstract Number: 1517 • ACR Convergence 2023
Comparing Deep Neural Network to Modified Rodnan Skin Score in a Trial for Belumosudil in Systemic Sclerosis Patients
Baran Gunes¹, Lucy Duran Camacho¹, Shawn Cowper², Gauri Panse², Elizabeth Bundschuh³, Alyssa Williams⁴, Nicolas Page⁵, Mary Karns⁶, Kathleen Aren⁶, Niki Pradhan⁷, Elana Bernstein⁸, Sarah Fantus⁹, Elizabeth Volkmann¹⁰, Heather Bukiri¹⁰, Chase Correia¹¹, Francis Wilson¹, Seamus Mawe¹², J. Matthew Mahoney¹³, Monique Hinchcliff¹⁴ and Rui Wang¹⁵, ¹Yale University School of Medicine, New Haven, CT, ²Yale School of Medicine - Dermatopathology, New Haven, CT, ³Yale University School of Medicine, New York, NY, ⁴Yale University School of Medicine, Winchester, MA, ⁵Yale University School of Medicine, Salem, CT, ⁶Northwestern University Division of Rheumatology, Chicago, IL, ⁷Columbia University Irving Medical Center, New York, NY, ⁸Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁹Kansas City Physician Partners- Center for Rheumatic Disease, Kansas City, MO, ¹⁰University of California Los Angeles, Los Angeles, CA, ¹¹Riverside Medical Group, Hampton, VA, ¹²The Jackson Laboratory, Bar Harbor, ME, ¹³Jackson Laboratory, Bar Harbor, ME, ¹⁴Yale School of Medicine, Westport, CT, ¹⁵Sanofi, Cambridge, MA
Background/Purpose: We previously published a proof-of-principle study demonstrating the potential utility of computer vision (Deep Neural Network/DNN) methods applied to stained skin biopsy sections from…
Abstract Number: 2359 • ACR Convergence 2023
Reversal of the Activation State of Pro-fibrotic CD206 Positive Macrophages by Therapeutic Peptide AUR300 in Systemic Sclerosis
Sandra Lopez¹, Bahja Ahmed Abdi¹, Linda Lei¹, Anas Al-Oweidi¹, Claire Macfadyen¹, Lydia Nagib¹, Sarah Ive¹, Anshul Kumar¹, Teresa Collins¹, Jennifer Cross², Christopher Denton¹, David Abraham¹, Clayton Yates³, Charles Garvin⁴, Henry Lopez⁴, George Martin⁵ and Richard Stratton¹, ¹University College London, London, United Kingdom, ²Aurinia Pharmaceuticals Inc., Rockford, MD, ³Tuskegee University, Tuskegee, AL, ⁴Riptide Bioscience Inc., Valejo, CA, ⁵Riptide Bioscience Inc., Bethesda, MD
Background/Purpose: A spectrum of macrophage activation is seen in human pathologic conditions; M2-like CD206 positive macrophages are pro-fibrotic and proresolution, while M1-like macrophages are proinflammatory,…
Abstract Number: 0037 • ACR Convergence 2022
Dual (and Dueling) Fibroblast Differentiation Is Integral to the Pathogenesis of Anti-Ro Mediated Congenital Heart Block and Provides a Novel Biomarker of Severity
Christina Firl¹, Robert Clancy², Bettina Cuneo³, Nicola Fraser⁴, Marc Halushka⁵, Mala Masson⁶, Amit Saxena¹ and Jill Buyon², ¹NYU Langone Health, New York, NY, ²NYU Grossman School of Medicine, New York, NY, ³University of Colorado School of Medicine, Aurora, CO, ⁴NYU School of Medicine, New York, NY, ⁵Johns Hopkins Medicine, Baltimore, MD, ⁶NYU Langone Medical Center- Division of Rheumatology, New York, NY
Background/Purpose: Fibroblast heterogeneity may provide critical insights into autoimmune mediated tissue damage. Transdifferentiation to a scarring myofibroblast is characterized by expression of alpha-Smooth Muscle Actin…

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