Abstracts tagged "autoimmune diseases"

Abstract Number: 034 • 2023 Pediatric Rheumatology Symposium
STAT3 Gain-of-function Syndrome Mutations Are Susceptible to JAK Inhibition Despite a Spectrum of Potency
Herda Ona¹, Justin Branch², Isabella Osuna¹, Priscilla vasquez², Anaid Reyes¹, Phillip Baker¹, Michael Clowers¹, Stephanie Wood¹ and Tiphanie Vogel¹, ¹Baylor College of Medicine, Houston, TX, ²Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: STAT3 gain-of-function (GOF) syndrome is a rare inborn error of immunity that leads to early-onset lymphoproliferation, autoimmune cytopenias, multi-solid organ autoimmunity, hypogammaglobulinemia, and short…
Abstract Number: 065 • 2023 Pediatric Rheumatology Symposium
Relationship Between Arthritis and Uveitis Disease Activity in Children with JIA
Meghana Karumuri¹, Megan Quinlan-Waters², Alexandra Duell², Kelly Rogers², Sheila Angeles-Han³ and Patricia Vega-Fernandez², ¹Michigan State University, Novi, MI, ²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Cincinnati Children's Hospital, Cincinnati, OH
Background/Purpose: Uncontrolled uveitis can lead to visual complications in 50% of children with JIA associated uveitis (JIA-U). While arthritis and uveitis are not considered to…
Abstract Number: 078 • 2023 Pediatric Rheumatology Symposium
Periodic Fever Syndrome and Myelodysplastic Syndrome: Possible Connections Between Two Disorders
Ivanna Romankevych¹, Nicole Torres², Imani Sanders¹, Laurent King¹ and Pedro Garcia Rodriguez¹, ¹Jackson Health System, University of Miami Miller School of Medicine, Miami, FL, ²University of Miami Miller School of Medicine, Miami, FL
Background/Purpose: Background: associations between autoimmune, immunodeficiency and hematological disorders are well known as well as with autoinflammatory conditions. Modern genetic testing allows to link these…
Abstract Number: 099 • 2023 Pediatric Rheumatology Symposium
The Impact of the COVID-19 Pandemic on Juvenile Dermatomyositis
Tresa Ambooken¹, Sangati Kadakia¹, Tara Lozy¹, Brianna Bulbin², Suhas Ganguli³, Dawn Wahezi⁴ and Sivia Lapidus¹, ¹Department of Pediatrics, Joseph M. Sanzari Children's Hospital, Hackensack University Medical Center, Hackensack, NJ, ²Hackensack Meridian School of Medicine, Hackensack, NJ, ³Department of Pediatrics, K. Hovnanian Children's Hospital, Jersey Shore University Medical Center, Neptune City, NJ, ⁴Children's Hospital at Montefiore, New York, NY
Background/Purpose: Juvenile Dermatomyositis (JDM) is theorized to occur in a genetically susceptible individual as a response to an environmental trigger, leading to autoimmunity and inflammation.…
Abstract Number: 112 • 2023 Pediatric Rheumatology Symposium
Adverse Childhood Experiences: Prevalence and Relationship to Disease in Childhood-onset Lupus
Olivia Hendrikx¹, Stephanie Fevrier¹, Ibrahim Mohamed¹, Chelsea DeCoste², Paris Moaf¹, Lawrence Ng¹, Deborah Levy¹, Linda Hiraki¹, Alene Toulany¹ and Andrea Knight¹, ¹The Hospital for Sick Children, Toronto, ON, Canada, ²IWK Health Centre, Halifax, NS, Canada
Background/Purpose: Adverse Childhood Experiences (ACEs) measure traumatic experiences in childhood. ACEs are associated with epigenetic changes, are known to increase stress response and inflammation, and…
Abstract Number: L10 • ACR Convergence 2022
Efgartigimod Prevents Necrosis and Allows for Muscle Fiber Regeneration in a Humanized Mouse Model of Immune-mediated Necrotizing Myopathy (IMNM)
Sarah Julien¹, Emma Briand¹, Bas van der Woning², Leentje de ceuninck³, Rachid zoubari¹, olivier benveniste⁴, laurent drouot¹ and olivier boyer⁵, ¹INSERM U1234, PAn’THER FOCIS Center of Excellence, Rouen, France, ²argenx, Boston, MA, ³argenx, Ghent, Belgium, ⁴AP-HP, Pitié-Salpêtrière University Hospital, Department of Internal Medicine and Clinical Immunology, Paris, France, ⁵INSERM U1234, PAn’THER FOCIS Center of Excellence; Rouen University Hospital, Department of Immunology and Biotherapy, Rouen, France
Background/Purpose: Immune-mediated necrotizing myopathy (IMNM) is a severe form of myositis characterized by muscle weakness and elevated creatine kinase levels in serum. The most frequent…
Abstract Number: L04 • ACR Convergence 2022
Psoriatic Arthritis Disease Subtypes Mediated by CD8 T Cells Are Phenocopied in a Novel Humanized Murine Model of Psoriasis and Arthritis
Maria de la Luz Garcia-Hernandez¹, Javier Rangel-Moreno¹, Ananta Paine², Soumyaroop Bhattacharya³, Jeffrey Fox⁴, Ernest Meyer⁵, Brian Isett⁵, Riyue Bao⁶, Tullia Bruno⁶ and Christopher Ritchlin⁷, ¹University of Rochester, Rochester, NY, ²ORNA Tx, Southborough, MA, ³Department of Pediatrics, University of Rochester Medical Center, Rochester, NY, ⁴CMSR, University of Rochester Medical Center, Rochester, NY, ⁵UPMC Hillman Cancer Center, Pittsburgh, PA, ⁶University of Pittsburgh, Pittsburgh, PA, ⁷University of Rochester Medical Center, Rochester, NY
Background/Purpose: A pivotal clinical characteristic of psoriatic arthritis (PsA) is disease manifestations in several domains (skin, entheses, joints, spine) and multiple domain involvement is prevalent,…
Abstract Number: 0014 • ACR Convergence 2022
The Expanded CD21 Low B Cell Subpopulation in Ankylosing Spondylitis Consists Mainly of Antigen-Inexperienced Cells
Rick Wilbrink¹, Linda van der Weele², Anneke Spoorenberg¹, Niek De Vries², Frans Kroese¹ and Gwenny Verstappen¹, ¹University Medical Center Groningen, Groningen, Netherlands, ²University Medical Center Amsterdam, Amsterdam, Netherlands
Background/Purpose: The role of B cells in the pathogenesis of ankylosing spondylitis (AS) remains relatively understudied. Nevertheless, available evidence shows presence of B cells at…
Abstract Number: 0157 • ACR Convergence 2022
Brepocitinib for the Treatment of Dermatomyositis: Pharmacologic and Clinical Rationale
Rohit Aggarwal¹, Brendan Johnson², Jolie Feldman³, Austin Gromatzky⁴ and Paul N Mudd Jr.², ¹Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ²Priovant Therapeutics, Durham, NC, ³Priovant Therapeutics, New York, NY, ⁴Priovant Therapeutics, Washington, DC
Background/Purpose: Brepocitinib is a novel, orally available, TYK2/JAK1 inhibitor in Phase 3 development for the treatment of dermatomyositis (DM), a chronic immune-mediated disease of the…
Abstract Number: 0506 • ACR Convergence 2022
Immunophenotypic Categorization of Systemic Immune-mediated Diseases
Shinji Izuka¹, Toshihiko Komai¹, Takahiro Itamiya¹, Mineto Ota², Saeko Yamada¹, Yasuo Nagafuchi², Hirofumi Shoda¹, Kosuke Matsuki³, Kazuhiko Yamamoto⁴, Tomohisa Okamura² and Keishi Fujio¹, ¹Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Bunkyo City, Tokyo, Japan, ²Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Department of Functional Genomics and Immunological Diseases, Graduate School of Medicine, The University of Tokyo, Bunkyo City, Tokyo, Japan, ³Research Division, Chugai Pharmaceutical Co., Ltd., Kamakura, Japan, ⁴Laboratory for Autoimmune Diseases, Center for Integrative Medical Sciences, the Institute of Physical and Chemical Research (RIKEN), Wako, Saitama, Japan
Background/Purpose: Systemic immune-mediated diseases are heterogeneous, and the pathogenesis varies among and within each disease. Some studies tried to stratify patients with immune-mediated diseases into…
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: 0740 • ACR Convergence 2022
Real-World Demographics, Clinical Characteristics, and Treatment Patterns of Patients Treated with Emapalumab for Secondary Hemophagocytic Lymphohistiocytosis in the United States: The REAL-HLH Study
Carl Allen¹, Shanmuganathan Chandrakasan², Michael Jordan³, Jennifer Leiding⁴, Abiola Oladapo⁵, Priti Pednekar⁶, Kelly Walkovich⁷ and John Yee⁸, ¹Baylor College of Medicine, Houston, TX, ²Emory University School of Medicine, Atlanta, GA, ³University of Cincinnati, Cincinnati, OH, ⁴Johns Hopkins University, Baltimore, MD, ⁵Swedish Orphan Biovitrum AB, Boston, MA, ⁶PRECISIONheor, Los Angeles, CA, ⁷University of Michigan Health, Ann Arbor, MI, ⁸Sobi - North America, Waltham, MA
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome caused by overproduction of proinflammatory cytokines, e.g., interferon gamma (IFNγ). Secondary HLH (sHLH), a subtype…
Abstract Number: 0790 • ACR Convergence 2022
Sustainability of Immunogenicity of BNT162b2 Vaccine in Patients with Autoimmune Rheumatic Disease, a Retrospective Comparative Study
Omar Alsaed, Masautso Chaponda, Eman Satti, Hadil Ashour, Muna Almaslamani and Samar Al emadi, Hamad Medical Corporation, Doha, Qatar
Background/Purpose: The sustainability of the immunogenicity of BNT162b2 anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine in patients with autoimmune rheumatic diseases (ARDs) receiving immunomodulators…
Abstract Number: 0987 • ACR Convergence 2022
A Randomized Placebo-Controlled Phase 1 Study in Healthy Adult Volunteers of the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of ALPN-303, a Potent Dual BAFF/APRIL Antagonist for the Treatment of Systemic Lupus Erythematosus and Other Autoantibody-Associated Diseases
Stacey Dillon¹, Pille Harrison², Jason Lickliter³, Kristi Manjarrez¹, Alina Smith¹, Mary Lessig¹, Lori Blanchfield¹, Russell Sanderson¹, Allison Chunyk¹, Tiffany Blair¹, Amanda Enstrom¹, Martin Wolfson¹, Mark Rixon¹, Hany Zayed⁴, Rupert Davies¹ and Stanford Peng¹, ¹Alpine Immune Sciences, Seattle, WA, ²Alpine Immune Sciences, Taunton, United Kingdom, ³Nucleus Network, Melbourne, Australia, ⁴Alpine Immune Sciences, San Francisco, CA
Background/Purpose: B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL) are tumor necrosis factor (TNF) superfamily members that bind TACI (transmembrane activator and CAML…
Abstract Number: 1200 • ACR Convergence 2022
Vitamin D and Marine n-3 Fatty Acid Supplementation for Prevention of Autoimmune Disease in the VITAL Randomized Controlled Trial: Outcomes over 7 Years
Karen Costenbader¹, Nancy R Cook², I-Min Lee², Jill Hahn³, Joseph Walter¹, Vadim Bubes¹, Gregory Kotler¹, Nicole Yang¹, Sonia Friedman¹, Erik K Alexander¹ and JoAnn E Manson², ¹Brigham and Women's Hospital, Boston, MA, ²Harvard TH Chan School of Public Health, Brigham and Women's Hospital, Boston, MA, ³Brigham and Womens' Hospital, Boston, MA
Background/Purpose: Strong biologic rationale supports potential effects of both vitamin D and of marine omega-3 (n3) fatty acids for autoimmune disease prevention. Within the randomized…

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