Abstracts tagged "immunology"

Abstract Number: 1936 • ACR Convergence 2022
A De Novo Dominant-negative PSMB8 mutation is Linked to a More Severe CANDLE-like Phenotype
Sara Alehashemi¹, Adriana Almeida de Jesus², Jonas Johannes Papendorf³, Farzana Bhuyan⁴, Kat Uss⁵, Anvitha Metpally⁶, Bin Lin⁷, Sophia Park⁸, Thais C.L. Moura⁹, Renata R Dias⁹, Mayra B. Dorna⁹, Katia Kozu⁹, Adriana Sallum⁹, Lucia M. A. Campos⁹, Gijs Santen¹⁰, Jesper Kers¹⁰, Onno Teng¹¹, Karin Palmblad¹², Tom Huizinga¹⁰, Frédéric Ebstein¹³, Elke Krüger¹³ and Raphaela Goldbach-Mansky¹⁴, ¹NIH/NIAID/TADS, Clarksville, MD, ²NIAID, NIH, Silver Spring, MD, ³Institut für Medizinische Biochemie und Molekularbiologie, Universitätsmedizin Greifswald, Greifswald, Germany, ⁴National Institutes of Health, Bethesda, MD, ⁵National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁶NIAID, NIH, Bethesda, MD, ⁷NIH, Bethesda, MD, ⁸NIAID, Bethesda, MD, ⁹Children's Hospital of the University of São Paulo, São Paulo, Brazil, ¹⁰Leiden University Medical Center, Leiden, Netherlands, ¹¹Leiden University Medical Center, Leiderdorp, Netherlands, ¹²Karolinska University Hospital, Stockholm, Sweden, ¹³Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, ¹⁴NIH/NIAID, Potomac, MD
Background/Purpose: Homozygote (HM)/compound heterozygote (CH) and digenic (DG) mutations in proteasome subunits cause the autoinflammatory syndrome CANDLE, or proteasome-associated autoinflammatory syndrome (PRAAS). Inflammation is mediated…
Abstract Number: 0040 • ACR Convergence 2022
Plasma Proteomic Signatures in Juvenile Dermatomyositis Highlight Novel Proteins and a Protein Module That Associate with Skin and Global Disease Activity
Marianne Kerski¹, Celine Berthier¹, Alex Tsoi¹, Sarah Vandenbergen¹, Madison McClune¹, Corey Powell¹, J. Michelle Kahlenberg¹ and Jessica Turnier², ¹University of Michigan, Ann Arbor, MI, ²University of Michigan, Saline, MI
Background/Purpose: Juvenile dermatomyositis (JDM) patients display heterogenous disease phenotypes and treatment response patterns. Our study used a multiplexed proteomics assay for measurement of 3072 proteins…
Abstract Number: 0608 • ACR Convergence 2022
Wnt Pathway Regulators R-spondin 3 and Dickkopf-related Protein 3 Demarcate a Transcriptional Gradient That Drives Synovial Fibroblast Inflammatory Pathology in Rheumatoid Arthritis
Alisa Mueller¹, Angela Zou², SABA NAYAR³, Emily Taylor³, Triin Major³, David H Gardner³, Gerald FM Watts¹, Adam Croft³, Roche Fibroblast Network Consortium⁴, Andrew Filer³, Christopher Buckley⁵, Kevin Wei⁶, ilya Korsunsky¹, Soumya Raychaudhuri¹ and Michael Brenner⁷, ¹Brigham and Women's Hospital, Boston, MA, ²Harvard Medical School, Boston, MA, ³University of Birmingham, Birmingham, United Kingdom, ⁴Roche, Basel, Switzerland, ⁵University of Oxford, Oxford, United Kingdom, ⁶Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ⁷Brigham and Women's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Synovial fibroblasts are key players in rheumatoid arthritis (RA) where they secrete inflammatory cytokines and directly instigate cartilage and bone destruction. However, most treatments…
Abstract Number: 0760 • ACR Convergence 2022
Prevalence of Autoimmune Conditions in Metastatic Melanoma Patients Starting Checkpoint Inhibitors
Alexandra Ladouceur¹, Marina Amaral De Avila Machado², Marie Hudson¹, Cristiano Moura³ and Sasha Bernatsky³, ¹McGill University, Montréal, QC, Canada, ²Research Institute of McGill University Health Center, Montréal, QC, Canada, ³Research Institute of the McGill University Health Centre, Montréal, QC, Canada
Background/Purpose: Since patients with pre-existing autoimmune conditions are commonly excluded from clinical trials with immune checkpoint inhibitors (ICI), we aimed to describe the characteristics of…
Abstract Number: 1174 • ACR Convergence 2022
Cellular Immunotherapy for Systemic Sclerosis
Chanhyuk Park¹, Rajan Bhandari², Jason Gunn³, Timothy Sullivan², Gretel Torres², Joana Murad³, Michael Whitfield⁴ and Patricia Pioli², ¹Geisel School of Medicine at Dartmouth, West Lebanon, NH, ²Geisel School of Medicine at Dartmouth, Lebanon, NH, ³Celdara Medical, Lebanon, ⁴Geisel School of Medicine, Lebanon, NH
Background/Purpose: Pro-fibrotic macrophages (MØs) are implicated in the pathogenesis of systemic sclerosis (SSc). In prior work, we and others have shown that MØs derived from…
Abstract Number: 1717 • ACR Convergence 2022
Deficiency of IL-23 Receptor in Podocytes of MRL/lpr lupus-prone Mice Abrogates the Development of Lupus Nephritis Despite IgG Deposition in the Glomeruli
Afroditi Boulougoura¹, Hao Li¹, Rhea Bhargava¹, Wenliang Pan¹, Abhigyan Satyam¹, Isaac Stillman² and George Tsokos¹, ¹Division of Rheumatology and Clinical Immunology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, ²Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA
Background/Purpose: Interleukin 23 (IL-23) is involved in the pathogenesis of systemic lupus erythematosus (SLE). IL-23 is elevated in the sera of patients with active SLE…
Abstract Number: 1981 • ACR Convergence 2022
Rho-GTPase Expression Recruits CD14+ Cells to Joints in Rheumatoid Arthritis and Predicts Good Response to JAK-inhibitor Treatment
Eric Malmhäll-Bah¹, Karin Andersson², Nina Oparina², Sofia Töyrä Silfverswärd³, Malin Erlandsson² and Maria Bokarewa³, ¹Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden, ²Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Goteborg, Sweden, ³Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Göteborg, Sweden
Background/Purpose: While examining arthritis development in GGTaseI-deficient mice, we found that hyperactive Rho-GTPases regulate the thymic egress of CD4+ T cells to the peripheral lymphoid…
Abstract Number: 0047 • ACR Convergence 2022
Cenerimod, a Novel Selective S1P1 Receptor Modulator, Prevents or Attenuates Joint Inflammation in Several Preclinical Models of Rheumatoid Arthritis
Thomas Hoyler, Jeremy Scherer, Maxime Bulle, Conrad Wyss, Sylvie Froidevaux and Marianne Martinic, Idorsia Pharmaceuticals Ltd., Allschwil, Switzerland
Background/Purpose: Targeting the sphingosine-1-phosphate (S1P) pathway has become an attractive target for therapeutic intervention in autoimmune diseases due to its selective impact on the migration…
Abstract Number: 0615 • ACR Convergence 2022
Semaphorin 5A Suppresses Ferroptosis Through Activation of PI3K-AKT-mTOR Signaling in Rheumatoid Arthritis
Qi Cheng, Mo chen, Mengdan Liu, Xin chen, Huaxiang Wu and Yan Du, The Second Affiliated Hospital Of Zhejiang University School Of Medicine, Hangzhou, China
Background/Purpose: Rheumatoid arthritis (RA) is a systemic autoimmune disease with synovial inflammation as the main pathological feature, and can eventually lead to irreversible joint or…
Abstract Number: 0769 • ACR Convergence 2022
Heterologous Vector versus Homologous mRNA COVID-19 Booster Vaccination in Non-seroconverted Immunosuppressed Patients: A Randomized Controlled Trial
Daniela Sieghart¹, Daniel Mrak¹, Elisabeth Simader¹, Selma Tobudic², Helga Radner¹, Peter Mandl³, Lisa Göschl¹, Maximilian Koblischke⁴, Nikolaus Hommer⁵, Lukas Hartl⁶, Philipp Hofer⁷, Felix Kartnig¹, Thomas Hummel¹, Andreas Kerschbaumer³, Thomas Deimel³, Antonia Puchner¹, Renate Thalhammer⁸, Andreas Zuckermann⁹, Karin Stiasny⁴, Thomas Perkmann⁸, Helmuth Haslacher⁸, Markus Zeitlinger⁵, Ursula Wiedermann¹⁰, Judith Aberle⁴, Daniel Aletaha¹¹, Leonhard Heinz¹ and Michael Bonelli¹, ¹Division of Rheumatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria, ²Division of Infectious Diseases and Tropical Medicine, Department of Internal Medicine I, Medical University of Vienna, Vienna, Austria, ³Medical University of Vienna, Vienna, Austria, ⁴Center for Virology, Medical University of Vienna, Vienna, Austria, ⁵Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria, ⁶Division of Gastroenterology, Medical University of Vienna, Vienna, Austria, ⁷Department of Pathology, Medical University of Vienna, Vienna, Austria, ⁸Department of Laboratory Medicine, Medical University of Vienna, Vienna, Austria, ⁹Department of Cardiac Surgery, Medical University of Vienna, Vienna, Austria, ¹⁰Institute of Specific Prophylaxis and Tropical Medicine, Center of Pathophysiology, Infectiology & Immunology, Medical University Vienna, Vienna, Austria, ¹¹Medical University Vienna, Wien, Austria
Background/Purpose: Patients under immunosuppressive therapy have a high risk for severe COVID-19 disease courses. However, efficacy of vaccination and therefore the right vaccination strategy remains…
Abstract Number: 1180 • ACR Convergence 2022
Deficiency and Altered Phenotype of Mucosal-associated Invariant T Cells in Systemic Sclerosis
Manon Lesturgie-Talarek¹, Virginie Gonzalez¹, Camelia Frantz², Noémie Sénot¹, Zouriatou Gouda¹, Camille Rousseau¹, Lucie Beaudoin¹, Agnès Lehuen¹, Jérôme Avouac³ and Yannick Allanore⁴, ¹INSERM U1016, Institut Cochin, Paris, France, ²Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ³University of Paris, Paris, France, ⁴Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs such as the lung. Mucosal-associated invariant T (MAIT)…
Abstract Number: 1718 • ACR Convergence 2022
Parenchymal INFγ Response Regulates Murine Lupus Nephritis
Minjung Kim¹, Anthony Marinov¹, Mark Shlomchik² and Jeremy Tilstra², ¹University of Pittsburgh, Pittsburgh, ²University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Lupus nephritis is the most common life-threatening end-organ complication of SLE. Interstitial infiltrates, specifically T cells, are major predictors of disease outcomes. We recently…
Abstract Number: 2030 • ACR Convergence 2022
Designing of a Phase 2, Multicenter, Randomized, Placebo-Controlled, Double-Blind Study to Assess the Efficacy and Safety of Nipocalimab, an FcRn Inhibitor, in Adults with Primary Sjögren’s Syndrome
Jonathan Hubbard¹, Kim Campbell¹, Kathy Sivils¹, Robert Hoffman¹, Kim Hung Lo¹, Jocelyn Leu¹, Sophia Liva¹, Qing Zuraw¹, Anne Stevens¹, Leona Ling², Keith Karcher³, Sindhu Ramchandren³, Hong Sun³, Hal Scofield⁴, Daniel Wallace⁵ and Raphaèle Seror⁶, ¹Janssen Research and Development, LLC, Spring House, PA, ²Janssen Research & Development, LLC, Cambridge, MA, ³Janssen Research & Development, LLC, Titusville, NJ, ⁴Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁵Cedars-Sinai Medical Center, Los Angeles, CA, ⁶University Hospital Paris-Saclay, Le Kremlin Bicêtre, France
Background/Purpose: Dysregulated humoral immunity is a hallmark of primary Sjögren's Syndrome (pSS). This dysregulation involves aberrant B-lymphocyte activity resulting in abnormally high immunoglobulin G (IgG)…
Abstract Number: 0053 • ACR Convergence 2022
Natural Microbial Exposure Increases Susceptibility of C57BL/6 Mice to Collagen-induced Arthritis
Sahar Lotfi-Emran¹ and David Masopust², ¹University of Minnesota, Minneapolis, MN, ²University of Minnesota, Department of Microbiology and Immunology, Minneapolis, MN
Background/Purpose: Mice exposed to natural microbes (NME) develop an appropriately mature immune system, one that resembles that of a non-neonatal human.1,2 Collagen induced arthritis is…
Abstract Number: 0618 • ACR Convergence 2022
Regulation of IgA Secretion Through PAD2-mediated Citrullination of MZB1
Bo Sun¹, Benjamin Geary², Santanu Mondal³, Ronak Tilvawala⁴, Konstantin Tsoyi⁵, Ivan O. Rosas⁵, Paul Thompson³ and I-Cheng Ho², ¹Brigham and Women's Hospital, Lexington, MA, ²Brigham and Women's Hospital, Boston, MA, ³UMass Medical School, Worcester, MA, ⁴University of Kansas, Lawrence, KS, ⁵Baylor College of Medicine, Houston, TX
Background/Purpose: The secretion of antibody requires concerted action of several endoplasmic reticulum proteins (ERPs) in B cells. For example, marginal zone B and B1 cell-specific…

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