Abstracts tagged "interferon"

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…
Abstract Number: 1618 • ACR Convergence 2021
Disease Flares in CANDLE/PRAAS with Dose Reductions of Baricitinib
Kader Cetin Gedik¹, Grace Materne², Ana Ortega-Villa³, Gina Montealegre Sanchez⁴, Adam Reinhardt⁵, Paul Brogan⁶, Yackov Berkun⁷, Sara Murias⁸, Maria Robles⁹, Susanne Schalm¹⁰, Adriana Almeida de Jesus¹¹ and Raphaela Goldbach-Mansky¹², ¹Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Diseases Section, NIAID/NIH, Bethesda, TN, ³Biostatistics Research Branch, Division of Clinical Research, NIAID/NIH, Bethesda, ⁴NIAID/NIH, Bethesda, MD, ⁵Boys Town National Research Hospital, Omaha, NE, ⁶UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁷Hadassah Hebrew University Medical Center, Jerusalem, Israel, ⁸Hospital Infantil La Paz, Madrid, Spain, ⁹Eskenazi Health Center, IndianaPolis, IN, ¹⁰Rheumatologie im Zentrum, Munich, Germany, ¹¹TADS/NIAID/NIH, Silver Spring, MD, ¹²NIH/NIAID, Potomac, MD
Background/Purpose: Patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures /proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS) respond to treatment with baricitinib but require higher exposure…
Abstract Number: 0961 • ACR Convergence 2021
Proteomic Profiling of MIS-C Patients Reveals Heterogeneity Relating to Interferon Gamma Dysregulation and Vascular Endothelial Dysfunction
Caroline Diorio¹, Rawan Shraim¹, Laura Vella¹, Josephine Giles², Amy Baxter², Derek Oldridge², Scott Canna¹, Sarah Henrickson¹, Kevin Mcnerney¹, Frances Balamuth¹, Chakkapong Burudpakdee¹, Jessica Lee¹, Tomas Leng¹, Alvin Farrell¹, Michele Lambert¹, Kathleen Sullivan¹, John Wherry³, David Teachey¹, Hamid Bassiri¹ and Edward Behrens¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³UPenn, Philadelphia, PA
Background/Purpose: Multi-system Inflammatory Syndrome in Children (MIS-C) is a major complication of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic in pediatric patients. Children…
Abstract Number: 1643 • ACR Convergence 2021
Increased Incidence of Pediatric SLE and Other Interferon Activated Diseases During COVID-19 Pandemic
Xinyu Dou¹, David Kaelber² and Hulya Bukulmez¹, ¹MetroHealth Medical Center, Case Western Reserve, Cleveland, OH, ²The MetroHealth System Campus of Case Western Reserve University, Cleveland, OH
Background/Purpose: COVID-19, as a member of the Coronavirus family, has been described to trigger host immune response via type I interferon (IFN) signaling pathways with…
Abstract Number: 0996 • ACR Convergence 2021
The Stimulator of Interferon Genes (STING) Protects from Bone Loss Through Regulation of Tonic and Induced Type I Interferon Pathways
Susan MacLauchlan¹, Priyanka Kushwaha¹, Albert Tai², Jia (Sijia) Chen³, Catherine Manning¹, Katherine Fitzgerald⁴, Shruti Sharma² and Ellen Gravallese⁵, ¹Brigham and Women's Hospital, Boston, MA, ²Tufts University School of Medicine, Boston, MA, ³Brigham and Women's Hospital, Cambridge, MA, ⁴University of Massachusetts Medical School, Worcester, MA, ⁵Brigham and Women's Hospital, Harvard Medical School, Chestnut Hill, MA
Background/Purpose: The intracellular DNA sensing Stimulator of Interferon Genes (STING) pathway is critical for detection of viral and bacterial pathogen DNA. Hyperactivating mutations in this…
Abstract Number: 1733 • ACR Convergence 2021
Classification of Patients with Systemic Lupus Erythematosus Enrolled in 2 Phase 3 Trials by EULAR/ACR 2019 Criteria
Martin Aringer¹, Ian N. Bruce², Richard Furie³, Eric Morand⁴, Emmanuelle Maho⁵, Catharina Lindholm⁶ and Raj Tummala⁷, ¹University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany, ²University of Manchester and NIHR Manchester Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom, ³Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ⁴Monash University, Melbourne, Australia, ⁵BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁶BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ⁷BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD
Background/Purpose: The TULIP-1 and TULIP-2 trials of anifrolumab, an anti–type I IFN receptor mAb, enrolled autoantibody-positive (ANA, anti-dsDNA, and/or anti-Smith [anti-Sm]) patients, who fulfilled the…
Abstract Number: 1011 • ACR Convergence 2021
Validation of Bioinformatics Pipeline to Detect NEMO-Deleted Exon 5 Autoinflammatory Syndrome (NEMO-NDAS) and Preliminary Clinical and Immunologic Characterization
Adriana Almeida de Jesus¹, Bin Lin², Eric Karlins³, Dana Kahle⁴, Andre Rastegar², Jacob Mitchell², Sofia Torreggiani², Farzana Bhuyan², Sara Alehashemi⁵, Kader Cetin Gedik⁶, Kat Uss², Chyi-Chia Lee⁷, Hyesun Kuehn⁸, Sergio Rosenzweig⁸, Katherine Calvo⁸, Magdalena Walkiewicz⁹, Justin Lack¹⁰, Eric Hanson¹¹, Amer Khojah¹², Eveline Wu¹³, Christiaan Scott¹⁴, Timothy Ronan Leahy¹⁵, Emma MacDermott¹⁵, Orla Kileen¹⁵, Thaschawee Arkachaisri¹⁶, Zoran Gucev¹⁷, Kathryn Cook¹⁸, Vafa Mammadova¹⁹, Gulnara Nasrullayeva¹⁹, Scott Canna²⁰, Douglas Kuhns²¹, Clifton Dalgard²², Timothy Moran²³, Andrew Oler³ and Raphaela Goldbach-Mansky²⁴, ¹TADS/NIAID/NIH, Silver Spring, MD, ²TADS/NIAID/NIH, Bethesda, MD, ³BCBB/NIAID/NIH, Bethesda, MD, ⁴National Institutes of Health, Chevy Chase, MD, ⁵TADS/NIAID/NIH, Clarksville, MD, ⁶Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ⁷NCI/NIH, Bethesda, MD, ⁸CC/DLM/NIH, Bethesda, MD, ⁹CSI/NIAID/NIH, Bethesda, MD, ¹⁰NCBR/NIAID/NIH, Bethesda, MD, ¹¹Indiana University School of Medicine, Indianapolis, IN, ¹²Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ¹³UNC Chapel Hill, Chapel Hill, NC, ¹⁴Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa, ¹⁵Children’s Health Ireland (CHI) at Crumlin, Dublin, Ireland, ¹⁶KK Women's and Children's Hospital, SingHealth, Singapore, Singapore, ¹⁷University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, ¹⁸Akron Childrens Hospital, Copley, OH, ¹⁹Azerbaijan Medical University, Baku, Azerbaijan, ²⁰Children's Hospital of Philadelphia, Philadelphia, PA, ²¹Frederick National Laboratory for Cancer Research/NIH, Frederick, MD, ²²TAGC/USUHS, Bethesda, MD, ²³University of North Carolina School of Medicine, Chapel Hill, NC, ²⁴NIH/NIAID, Potomac, MD
Background/Purpose: Splice site variants in IKBKG that lead to exon 5 deletion cause NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS). NEMO-NDAS clinically mimics the interferonopathy chronic…
Abstract Number: 1739 • ACR Convergence 2021
SLE Treatment History and Anifrolumab Efficacy by Baseline Standard Therapies in Patients with SLE from 2 Phase 3 Trials
Susan Manzi¹, Richard Furie², Eric Morand³, Yoshiya Tanaka⁴, Gabriel Abreu⁵, Catharina Lindholm⁵ and Raj Tummala⁶, ¹Allegheny Health Network, Pittsurgh, PA, ²Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ³Monash University, Melbourne, Australia, ⁴University of Occupational and Environmental Health, Kitakyushu, Japan, ⁵BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ⁶BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD
Background/Purpose: In the phase 3 TULIP-1 and TULIP-2 trials, anifrolumab, a type I IFN receptor mAb, improved disease activity versus placebo in patients with moderate…
Abstract Number: 1096 • ACR Convergence 2021
CSF-specific CD8 T Cell Clonal Expansion in Neurosarcoidosis
Michael Paley¹, Brandi Baker², Steven Dunham³, Nicole Linskey³, Elisha Roberson³, David Clifford³ and Wayne Yokoyama³, ¹Washington University in St. Louis, Olivette, MO, ²Washington University in St. Louis, St. Louis, MO, ³Washington University School of Medicine, St. Louis, MO
Background/Purpose: Neuroinflammation is a severe manifestation of the systemic inflammatory disorders. Sarcoidosis, which leads to neurologic disease in 5-10 % of cases, has traditionally been…

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