Abstracts tagged "interferon"

Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Kailey Brodeur¹, Liang Chen¹, zhengping huang², Yan Du¹, Holly Wobma³, Maria Taylor⁴, Joyce Chang³, Megan Day-Lewis³, Fatma Dedeoglu³, Olha Halyabar³, Mindy Lo³, Jane W. Newburger⁵, Mary Beth F. Son³, Robert Sundel³, Peter Nigrovic³, lauren henderson³ and Pui Lee³, ¹Boston Children's Hospital, Boston, MA, ²Guangdong Second Provincial Hospital, Guangzhou, China, ³Division of Immunology, Boston Children's Hospital, Boston, MA, ⁴Division of Immunology, Boston Children's Hospital, Brighton, MA, ⁵Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
Abstract Number: 0641 • ACR Convergence 2022
IL-4 Receptor Signaling and IKAROS Transcriptomic Program Maintains Naïve B-cell Quiescence and Antagonizes SLE
John Mountz, Min Gao, Shanrun Lui, Walter Chatham and Hui-Chen Hsu, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Factors that promote B-cell quiescence and homeostasis at the transitional (Tr) and naive stages of B cells to prevent excessive TLR7 and type I…
Abstract Number: 1629 • ACR Convergence 2022
AUR200: An Improved BAFF/APRIL Inhibitor with Increased Potency and Safety for the Treatment of B Cell-Mediated Diseases
Shawn Morales, Jennifer Cross and Robert Huizinga, Aurinia Pharmaceuticals, Inc., Victoria, BC, Canada
Background/Purpose: AUR200 targets both B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL), which are key mediators in the pathogenesis of B cell-mediated autoimmune…
Abstract Number: 2264 • ACR Convergence 2022
Dysregulated Interferon Gamma Signals Promote Systemic Autoimmunity in STAT1 Gain of Function Syndrome
Andrea Largent¹, Jane Buckner², David Rawlings¹ and Shaun Jackson¹, ¹Seattle Children's Research Institute, Seattle, WA, ²Benaroya Research Institute at Virginia Mason, Seattle, WA
Background/Purpose: Heterozygous gain-of-function mutations in STAT1 promote a complex disorder of immune dysregulation including chronic mucocutaneous candidiasis (CMC) and systemic autoimmunity. However, given widespread expression…
Abstract Number: 0644 • ACR Convergence 2022
Long-lived Plasma Cells in the Lupus Bone Marrow Are Imprinted by Interferon
Jennifer Barnas¹, Diana Alzamareh², Neha Nandedkar-Kulkarni², Jennifer Albrecht², Nida Meednu², Cameron Baker², Andrew McDavid¹ and Jennifer Anolik², ¹University of Rochester, Rochester, NY, ²University of Rochester Medical Center, Rochester, NY
Background/Purpose: Systemic lupus erythematous (SLE) is a complex autoimmune disorder with heterogeneous disease presentation and a multi-pronged pathogenesis. Although autoreactive plasma cells (PC) play a…
Abstract Number: 1664 • ACR Convergence 2022
TRIM21 as a Regulator of UVB-Driven IFN Responses in Lupus
Richard Moore, Gantsetseg Tumurkhuu, Daniel Wallace and Caroline Jefferies, Cedars-Sinai Medical Center, Los Angeles, CA
Background/Purpose: Patients with systemic lupus erythematosus (SLE) experience photosensitivity where exposure to ultraviolet light B (UVB) drives lupus flares and systemic symptoms. Although the mechanisms…
Abstract Number: 2267 • ACR Convergence 2022
Plasmacytoid Dendritic Cells Are Not Major Producers of Type 1 Interferons in Cutaneous Lupus
Thomas Vazquez¹, Nilesh Kodali¹, DeAnna Diaz², Jay Patel³, Emily Keyes², Grant Sprow², Meena Sharma¹, Mariko Ogawa-Momohara², Madison Grinnell², Josh Dan² and Victoria Werth², ¹Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ²Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, ³Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PN
Background/Purpose: Type 1 interferons (IFN-1) are major drivers of disease activity in systemic (SLE) and cutaneous lupus erythematosus (CLE). Plasmacytoid dendritic cells (pDCs) are the…
Abstract Number: L20 • ACR Convergence 2021
Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA) Who Had Failed High-Dose Glucocorticoids (GCs)
Fabrizio De Benedetti¹, Alexei Grom², Paul Brogan³, Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci¹, Despina Eleftheriou³, Charalampia Papadopoulou³, Pierre Quartier⁴, Jordi Antón⁵, Rikke Frederiksen⁶, Veronica Asnaghi⁶ and Cristina De Min⁶, ¹Bambino Gesù Children's Hospital IRCCS, Rome, Italy, ²Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁴Université de Paris, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁵Hospital Sant Joan de Déu, Division of Pediatric Rheumatology, University of Barcelona, Barcelona, Spain, ⁶Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a life-threatening complication of rheumatic diseases, occurring most frequently in sJIA. The mainstay of MAS treatment is high dose GCs; however, GCs…
Abstract Number: 0501 • ACR Convergence 2021
Immunogenicity of a Single Dose of Covid-19 Vaccination in Patients with Systemic Sclerosis with or Without Immunosupression
Vishal Kakkar¹, Rebecca Ross², Ranjitha Karanth³, Sumit Lahiri⁴, Panji Mulipa¹, Pamela Hughes⁵, Brendan Clarke⁵, Clive Carter⁵, Mark Lobb⁵, Sinisa Savic⁶ and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²LIRMM University of Leeds, Leeds, United Kingdom, ³Leeds Institute of Rheumatic and Musculoskeletal Medicine, LTHT, Leeds, United Kingdom, ⁴Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁵Transplant and Cellular Immunology, Leeds, United Kingdom, ⁶Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) is a rare connective tissue disease with multi-systemic involvement, which at times requires the use of immunosuppressive medication. None of the…
Abstract Number: 1494 • ACR Convergence 2021
Functional Effects of a Lupus-associated PRKG1 Variant on the RhoA-ROCK Pathway and Response to Type I Interferon
Ruth Fernandez Ruiz¹, Justine Shum², Kayla Van Buren³ and Timothy Niewold⁴, ¹NYU Grossman School of Medicine, New York, NY, ²Marinhealth Medical Center, San Francisco, CA, ³Mnemo Therapeutics, New York, NY, ⁴Colton Center for Autoimmunity NYU School of Medicine, New York, NY
Background/Purpose: Interferon (IFN)-α contributes to susceptibility and severe manifestations in systemic lupus erythematosus (SLE). The PRKG1 rs7897633 variant has been previously identified as the top…
Abstract Number: 0528 • ACR Convergence 2021
Interferon Pathway Lupus Risk Alleles Modulate Risk of Death from Acute COVID-19
Ilona Nln¹, Ruth Fernandez Ruiz², Theresa Wampler Muskardin³, Stephanie Tuminello², Mukundan Attur², Eduardo Itturate², Christopher Petrilli², Steven B. Abramson⁴, Aravinda Chakravarti² and Timothy Niewold¹, ¹Colton Center for Autoimmunity NYU School of Medicine, New York, NY, ²NYU Grossman School of Medicine, New York, NY, ³Colton Center for Autoimmunity, NYU School of Medicine, New York, NY, ⁴New York University School of Medicine, New York, NY
Background/Purpose: Type I interferon (IFN) is critical in our defense against viral infections. Increased type I IFN pathway activation is a genetic risk factor for…
Abstract Number: 1495 • ACR Convergence 2021
Multiplexed Profiling of Treatment Naïve Cutaneous Lupus Skin Stratified by Patient Response to Antimalarials
Thomas Vazquez¹, Jay Patel², Daisy Yan³, Emily Keyes⁴, DeAnna Diaz⁵, Yubin Li⁶, Madison Grinnell⁶, Rui Feng⁷ and Victoria Werth³, ¹FIU Wertheim College of Medicine, Virginia Beach, VA, ²Corporal Michael J. Crescenz VAMC, Department of Dermatology, U Penn, Philadelphia, NJ, ³Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ⁴Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania and Philadelphia VAMC, Philadelphia, PA, ⁵Philadelphia College of Medicine, Philadelphia, PA, ⁶Corporal Michael J. Crescenz VAMC, Department of Dermatology, U Penn, Philadelphia, PA, ⁷University of Pennsylvania Department of Biostatistics, Philadelphia, PA
Background/Purpose: Lupus erythematous (LE) is a systemic autoimmune disease with a variety of cutaneous manifestations. Antimalarials are first-line systemic therapy, yet not all patients respond…
Abstract Number: 0705 • ACR Convergence 2021
Mitochondrial Calcification-Induced Inflammation in Human Skeletal Muscle and Immune Cells
Bhargavi Duvvuri¹, Lauren Pachman², TING WANG¹, Payton Hermanson¹ and Christian Lood¹, ¹University of Washington, Seattle, WA, ²Northwestern's Feinberg School of Medicine. Ann and Robert H. Lurie Children's Hospital of Chicago; Stanley Manne Children's Research Institute of Chicago, Lake Forest, IL
Background/Purpose: Children with juvenile dermatomyositis (JDM) have decreased autophagy, as also confirmed by our RNA seq data in JDM muscle, which may contribute to accumulation…
Abstract Number: 1504 • ACR Convergence 2021
Association Between Anti-RNP Antibodies and Interferon Gene Expression but Not Complement Consumption in SLE
Erika Hubbard¹, David Pisetsky² and Peter Lipsky¹, ¹AMPEL BioSolutions, Charlottesville, VA, ²Duke University Medical Center, Durham, NC
Background/Purpose: Anti-nuclear antibodies are important serologic features of SLE and facilitate diagnosis. Anti-double stranded DNA (dsDNA) antibodies are routinely monitored for disease prognosis and are…
Abstract Number: 0874 • ACR Convergence 2021
Serum Galectin-9 and CXCL-10 but Not Their Urinary Levels Reflect Lupus Activity
Pankti Mehta, Pratibha Singh and Amita Aggarwal, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
Background/Purpose: SLE is characterized by increased type I IFN signature in the blood and immune cells. Traditionally, type I IFN signature is measured by gene…

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