Abstracts tagged "lupus-like disease"

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: 2404 • ACR Convergence 2024
Nailfold Dermoscopy: A Valuable Tool for Differentiating Dermatomyositis from Systemic Lupus Erythematosus
Caroline Stone¹, Daniella Faden¹, Lillian Xie¹, Lais Lopes Almeida Gomes¹, Joshua Dan¹, Cristina Ricco¹, Ahmed Eldaboush¹ and Victoria Werth², ¹University of Pennsylvania, philadelphia, PA, ²University of Pennsylvania, Wynnewood, PA
Background/Purpose: Dermatoscopes are emerging as efficient, valuable alternatives to capillaroscopy for evaluating rheumatologic conditions. Second to systemic sclerosis, nailfold capillaroscopy abnormalities are most consistently reported…
Abstract Number: 2549 • ACR Convergence 2024
VISTA Deficiency Alters the Skin Immune Cell Composition and Confers Skin Sensitivity to UV Light
Zachary Peters¹, Lindsay Mendyka², Angelique Cortez¹, J'voughnn Blake¹, Alecia Roy¹, William Rigby³, Christopher Burns⁴, Randolph Noelle⁵ and Sladjana Skopelja-Gardner⁶, ¹Dartmouth College, Lebanon, NH, ²Dartmouth Hitchcock Memorial Hospital, Lyme, NH, ³Dartmouth-Hitchcock, Norwich, VT, ⁴Dartmouth Hitchcock Medical Center, Lebanon, NH, ⁵Geisel School of Medicine at Dartmouth, Lebanon, ⁶Dartmouth Geisel School of Medicine, Lebanon, NH
Background/Purpose: Persistent production of type I interferons (IFN-Is) is one of the hallmarks of cutaneous lupus erythematosus (CLE) that is exacerbated by ultraviolet (UV) light.…
Abstract Number: 2599 • ACR Convergence 2024
Xist Deletion in B Cells Results in Systemic Lupus Erythematosus Phenotypes
Claudia Lovell¹, Nikhil Jiwrajka², Hayley Amerman³, Michael Cancro⁴ and Montserrat Anguera⁵, ¹Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ²Hospital of the University of Pennsylvania, Philadelphia, PA, ³University of Pennsylvania School of Veterinary Medicine, Philadelphia, PA, ⁴Perelman School of Medicine, Philadelphia, PA, ⁵University of Pennsylvania, Philadelphia, PA
Background/Purpose: Systemic lupus erythematosus (SLE) exhibits a strong female sex bias but the mechanisms underlying this bias are not well understood. Epidemiological studies demonstrate that…
Abstract Number: PP11 • ACR Convergence 2024
Living Well with Chronic Illness: Decades with MCTD
Carol KAMINSKI, HP (Hewlett Packard) Retired, Wilmington, NC
Background/Purpose: In the fall of 1985, I was not feeling well. I attributed my tiredness, weakness and malaise to stress and carried on. Walking upstairs I…
Abstract Number: 0086 • ACR Convergence 2024
Evaluating the Efficacyof B Cell Targeting Drug Candidates Using a Humanized BAFF Transgenic SLE Mouse Model
Juan Liang, Shuxin Xu, Qiuping Xu and Yuxi Zhang, GemPharmatech, San Diego
Background/Purpose: B-cell-activating factor (BAFF) plays a critical role in B cell survival, and its elevated expression can contribute to the presence of autoreactive B cells,…
Abstract Number: 0089 • ACR Convergence 2024
Single-Cell RNA Sequencing Outlines the Mechanisms of Vgll3-Driven Myofibroblasts Differentiation in Promoting Fibrosis in Cutaneous Lupus
Mehrnaz Gharaee-Kermani, Allison Billi, Poulami Dey, Jacob Martens, Marisa Hildebrandt, J. Michelle Kahlenberg and Johann Gudjonsson, University of Michigan, Ann Arbor, MI
Background/Purpose: Skin fibrosis is characterized by collagen deposition, fibro/myofibroblast accumulation, and extracellular matrix remodeling. Some subtypes of cutaneous lupus erythematosus (CLE), particularly discoid lupus erythematosus…
Abstract Number: 0129 • ACR Convergence 2024
The Efficacy and Safety of Telitacicept Following Rituximab Immunotherapy on Antiphospholipid Syndrome, a Prospective 24- Week Study
Qiang Shu¹, Qing Zuraw², Xiaoyu Zhang³, Guillermo Pons-Estel⁴, Shuning Sun⁵, Qincheng Che¹, Xinyu Li⁶, Jie Li⁶ and Qi Liu⁶, ¹Qilu Hospital, Shandong Provincial Clinical Research Center for Immune Disease and Gout, Jinan, China; Qilu Hospital, Cheeloo College of Medicine, Shandong University, Rheumatology, Jinan, China, Jinan, China (People's Republic), ²RemeGen Biosciences, Inc., South San Francisco, China (People's Republic), ³Qilu Hospital, Shandong Provincial Clinical Research Center for Immune Disease and Gout, Jinan China, Beijing, China, ⁴Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina, ROSARIO, Santa Fe, Argentina, ⁵Qilu Hospital, Shandong Provincial Clinical Research Center for Immune Disease and Gout, Jinan, China; Qilu Hospital, Cheeloo College of Medicine, Shandong University, Pheumatology, Jinan, China, Jinan, China (People's Republic), ⁶Qilu Hospital, Jinan, China (People's Republic)
Background/Purpose: Antiphospholipid syndrome (APS) is a challenging disease to treat and lack effective therapies. Rituximab (RTX) can deplete CD20+ peripheral B cells. Telitacicept (TA) is…
Abstract Number: 0887 • ACR Convergence 2024
Identification of Rare Variants in Lupus-causing Genes in a Mixed Paediatric and Adult Connective Tissue Disease Cohort
Anastasia-Vasiliki Madenidou¹, Gillian Rice², Terence Garner³, Sarah Dyball⁴, Alice Chieng⁵, Ben Parker⁶, Tracy Briggs⁷, Adam Stevens³ and Ian Bruce⁸, ¹Centre for Musculoskeletal Research, The University of Manchester, Manchester, United Kingdom, ²Division of Evolution and Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester Academic Health Science Centre, Manchester, United Kingdom, Manchester, ³Division of Developmental Biology and Medicine, Faculty of Biology, Medicine and Health, University of Manchester and Manchester Academic Health Science Centre, Manchester, United Kingdom, Manchester, United Kingdom, ⁴Centre for Musculoskeletal Research, The University of Manchester, Manchester, UK, Manchester, United Kingdom, ⁵Department of Rheumatology, Royal Manchester Children's Hospital, Manchester, United Kingdom, Manchester, United Kingdom, ⁶Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom, ⁷Division of Evolution, Infection and Genomic Sciences, School of Biological Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom, Manchester, United Kingdom, ⁸Centre for Musculoskeletal Research, Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom
Background/Purpose: Connective tissue diseases (CTDs) are a family of heterogeneous autoimmune diseases with overlapping clinical features. Not all patients with features suggestive of a mendelian…
Abstract Number: 1574 • ACR Convergence 2024
Therapeutic Strategy in Patients with Mixed Connective Tissue Disease: A Multicenter Retrospective Study in the French MCTD Cohort
Kevin Chevalier¹, Benjamin Thoreau², Marc Michel³, Bertrand Godeau⁴, Christian AGARD⁵, Thomas Papo⁶, Karim Sacré⁶, Raphaele Seror⁷, Xavier Mariette⁸, Patrice Cacoub⁹, Ygal Benhamou¹⁰, Hervé Levesque¹⁰, Cécile Goujard¹¹, Olivier Lambotte¹¹, Bernard Bonnotte¹², Maxime SAMSON¹³, Felix Ackermann¹⁴, Jean Schmidt¹⁵, Pierre Duhaut¹⁶, Jean-Emmanuel Kahn¹⁷, Thomas Hanslik¹⁷, Nathalie Costedoat-chalumeau¹⁸, Benjamin Terrier¹⁹, Alexis Régent²⁰, bertrand Dunogue², Pascal cohen²¹, Veronique LE GUERN²², Eric Hachulla²³, Luc Mouthon² and Benjamin Chaigne²⁴, ¹Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Cité, Paris, France, Paris, France, ²Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ³2. Department of Internal Medicine, Henri-Mondor University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Est Créteil (UPEC), Créteil, France., Créteil, France, ⁴Department of Internal Medicine, Henri-Mondor University Hospital, Assistance Publique-Hôpitaux de Paris, Université Paris Est Créteil (UPEC), Créteil, France., Créteil, France, ⁵Nantes University Hospital, Nantes, France, ⁶Université Paris Cité, Paris, France, ⁷Service de Rhumatologie, Hôpital Bicêtre, AP-HP, le Kremlin Bicetre, Ile-de-France, France, ⁸Service de Rhumatologie, Hôpital Bicêtre, AP-HP, Le Kremlin Bicetre, France, ⁹Sorbonne Université, Paris, ¹⁰Department of Internal Medicine, CHU de Rouen, UniRouen, Rouen, France., Rouen, France, ¹¹Université Paris Saclay, Department of Internal Medicine and clinical immunology, Bicêtre hospital, Assistance Publique-Hôpitaux de Paris, UMR1184 Inserm, CEA, Le Kremlin Bicêtre, France, Kremlin-Bicêtre, France, ¹²Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France., Dijon, France, ¹³Dijon University Hospital, Dijon, France, ¹⁴Department of Internal Medicine, Foch Hospital, Suresnes, France., Suresnes, France, ¹⁵Department of Internal Medicine and RECIF, Amiens University Hospital, Université Picardie Jules Verne, Amiens, France., Amiens, France, ¹⁶Department of Internal Medicine and RECIF, Amiens-Picardie University Hospital, AMIENS, France, ¹⁷Department of Internal Medicine, Ambroise Paré Hospital, Assistance Publique - Hôpitaux de Paris, Université de Versailles Saint-Quentin-en-Yvelines, Boulogne-Billancourt, France, Boulogne Billancourt, France, ¹⁸University Paris Cité, Paris, France, ¹⁹Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, ²⁰National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ²¹National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ²²Cochin hospital, Paris, France, ²³Department of Internal Medicine and clinical immunology, North-West National Reference Center for Rare Systemic Autoimmune Diseases iques et Auto-Immunes Rares du Nord-Ouest, Hôpital Claude Huriez, Université de Lille, France, Lille, France, ²⁴Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, Ile-de-France, France
Background/Purpose: Mixed connective tissue disease (MCTD) is a rare systemic disorder that belongs to connective tissue diseases (CTD). Few studies are available on MCTD treatment.…
Abstract Number: 1931 • ACR Convergence 2024
Health System Improvement in Appropriate Test Utilization Involving ANA Testing in the Inpatient and Outpatient Setting
Benjamin Wolff¹, Nayeon Oh², Amy Slenker³, Kathryn Zaffiri³, Rahul Mhaskar⁴ and Marie O'Brien⁵, ¹Lehigh Valley Health Network - Internal Medicine Residency, Macungie, PA, ²Univeristy of South Florida, Allentown, PA, ³Lehigh Valley Health Network, Allentown, PA, ⁴University of South Florida, Tampa, FL, ⁵Lehigh Valley Hospital and Health Network, Orefield, PA
Background/Purpose: To manage patients In the current healthcare environment, objective data and laboratory tests drive medical decision making. This has led to an increase in…
Abstract Number: 0095 • ACR Convergence 2023
Kynurenine Is a Proinflammatory Metabolite That Activates a Positive Feedback Loop of Rab4A-dependent CD98 Expression and mTORC1 and mTORC2 Activation in SLE
Thomas Winans¹, Nick Huang¹, Joshua Lewis¹, Xiaojing Wang¹, Tamas Faludi¹, Daniel Krakko², Laurence Morel³ and Andras Perl⁴, ¹SUNY Upstate Medical University, Syracuse, NY, ²North Carolina State University, Raleigh, NC, ³University of Texas health San Antonio, San Antonio, TX, ⁴SUNY, Syracuse, NY
Background/Purpose: The kynurenine (KYN) pathway has been linked to disease pathogenesis in patients with systemic lupus erythematosus (SLE) (https://pubmed.ncbi.nlm.nih.gov/26366134/). Genetically enforced overexpression of Rab4A activates…
Abstract Number: 0551 • ACR Convergence 2023
Monogenic Lupus: Clinical Phenotypes and Genetic Mutations in a Cohort of Pediatric Patients
Keerthi Vardhan Yerram¹, Kavitha Shanigaaram¹, Gaurang Sudhir Deshpande¹, Akshay Parikh¹, Arjun Ramavath¹, Nanditha Gollakota¹, Ramesh Manthri¹ and Liza Rajasekhar², ¹Department of Clinical Immunology and Rheumatology, Nizams Institute of Medical Sciences, Hyderabad, India, ²Nizam's Institute of Medical Sciences, Madhapur, India
Background/Purpose: Monogenic lupus is associated with specific gene mutations, most commonly reported in TREX1, DNASE1L3, DNASE2, and SAMHD1. However, their phenotypes are not well reported.…
Abstract Number: 0555 • ACR Convergence 2023
Evaluation and Management of the “False Positive” ANA and Undifferentiated Connective Tissue Disease Amongst Rheumatologists
Neha Gupta¹, Sarah Ford², Lane Scheiber², Tangada Rao² and Abhishek Nandan², ¹Virginia Commonwealth University Health Systems, Richmond, VA, ²Virginia Commonwealth University Health Systems; Veterans Affairs Medical Center, Richmond, VA
Background/Purpose: The management of a positive anti-nuclear antibody (ANA) is one of the most common consultations in rheumatology outpatient practice. The prevalence of a positive…
Abstract Number: 0892 • ACR Convergence 2023
Single-Cell RNA Sequencing Reveals Keratinocytes and Fibroblasts Drive Hippo and TGFb Dysregulation and Fibrosis in Epidermal Overexpression of VGLL3 in Cutaneous Lupus
Mehrnaz Gharaee-Kermani¹, Allison Billi¹, Jacob Martens¹, Marisa Hildebrandt¹, Amanda Victory¹, Mitra Maz², Shannon Loftus¹, J. Michelle Kahlenberg¹ and Johann E. Gudjonsson¹, ¹University of Michigan, Ann Arbor, MI, ²University of Michigan Medical School, Ann Arbor, MI
Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast (MYOFB) accumulation, and extracellular matrix remodeling. In some subtypes of cutaneous lupus erythematosus (CLE), particularly discoid lupus…

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