Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1110 • ACR Convergence 2021
Cryopyrin-associated Periodic Syndromes: GOSH and National Amyloidosis Centre Experience
Ovgu Kul Cinar¹, Charalampia Papadopoulou², Amber Putland¹, Karen Wynne¹, Helen J Lachmann³, Despina Eleftheriou⁴ and Paul A. Brogan⁴, ¹Great Ormond Street Hospital NHS Trust, London, United Kingdom, ²UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology, London, United Kingdom, ³National Amyloidosis Centre, Royal Free Campus, UCL Medical School, London, United Kingdom, ⁴Great Ormond Street Hospital NHS Trust, UCL GOS Institute of Child Health, Department of Infection, Immunity & Inflammation, London, United Kingdom
Background/Purpose: CAPS is a rare, heterogenous inflammasomopathy associated with gain-of-function mutations in NLRP3 that encodes cryopyrin. Mutations in NLRP3 result in excessive IL-1ß production that…
Abstract Number: 0192 • ACR Convergence 2021
Influence of Canakinumab Dosing on Efficacy and Safety of Long-term Treatment in Patients with Familial Mediterranean Fever – Interim Analysis of the RELIANCE Registry
Jorg Henes¹, Norbert Blank², Tilmann Kallinich³, Frank Dressler⁴, Gerd Horneff⁵, Ivan Foeldvari⁶, Markus Hufnagel⁷, Birgit Kortus-Goetze⁸, Frank Weller-Heinemann⁹, Florian Meier¹⁰, Julia Weber-Arden¹¹ and Jasmin Kuemmerle-Deschner¹², ¹University Hospital Tuebingen, Tuebingen, Germany, ²Rheumatology, University Hospital Heidelberg, Eppelheim, Germany, ³Charité University Medicine, Nuremberg, Germany, ⁴Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ⁵Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁶Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ⁷Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ⁸Division of Nephrology, University of Marburg, Marburg, Germany, ⁹Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ¹⁰1 Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany 2 Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt am Main, Germany, Frankfurt a.M., Germany, ¹¹Novartis Pharma GmbH, Nuremberg, Germany, ¹²Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany
Background/Purpose: Familial Mediterranean Fever (FMF) is characterized by severe systemic and organ inflammation. Successful treatment with rapid remission of symptoms and normalization of laboratory parameters…
Abstract Number: 1116 • ACR Convergence 2021
Disease Characteristics of Steroid-Induced Spinal Epidural Lipomatosis: A Systematic Review
Haseeb Chaudhary¹, Usama Nasir², Khezar Syed³, Abigayle Sullivan³, Shilla Zachariah³, Christian AkemDimala³, Muhammad Khan⁴ and Anthony Donato³, ¹Tower Health System, Reading, PA, ²Reading Hospital, Tower Health System, Reading, PA, ³Reading Hospital, Tower Health System, Reading, PA, ⁴University of Kentucky, Lexington, KY
Background/Purpose: Steroid-induced epidural lipomatosis (SEL) is a rare phenomenon causing neuronal compression from adipose tissue deposition in the epidural space. We analyzed the available literature…
Abstract Number: 0193 • ACR Convergence 2021
Tocilizumab for the Treatment of Familial Mediterranean Fever – a Randomized, Double Blind, Placebo-controlled Phase II Study
Jorg Henes¹, Sebastian Saur², David Kofler³, Martin Krusche⁴, Theodoros Xenitidis⁵, Christoph Meisner⁶, Claudia Kedor⁷, Ina Koetter⁸, Hendrik Schulze-Koops⁹ and Eugen Feist¹⁰, ¹University Hospital Tuebingen, Tuebingen, Germany, ²Universtiy Hostpiatl Tübingen, Tuebingen, Germany, ³Division of Rheumatology and Clinical Immunology, Department I of Internal Medicine; University Hospital Cologne, Cologne, Germany, ⁴Charite Berlin, Berlin, Germany, ⁵University Hospital Tuebingen, Rheumatology, Tuebingen, Germany, ⁶University Tuebingen, Statistics, Tuebingen, Germany, ⁷Charite University Hospital Berlin, Berlin, Germany, ⁸University Hospital Hamburg Eppendorf, Hamburg, Germany, ⁹Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, University of Munich, Munich, Germany, ¹⁰Helios Department of Rheumatology, Vogelsang-Gommern, Germany
Background/Purpose: Familial Mediterranean Fever (FMF) is the most common auto-inflammatory disease characterized by chronic inflammation, recurrent episodes of fever, abdominal and thoracic pain due to…
Abstract Number: 1157 • ACR Convergence 2021
Patient Perspective on the Non Medical Switch of Originator to Its Biosimilar in Inflammatory Arthritis Using a Social Media Survey
David Vega Morales¹, Alejandro Garza-Alpirez² and Carlos Andrés Díaz-Garza², ¹Hospital Universitario, Garcia, Mexico, ²Tecnologico de Monterrey, Escuela de Medicina y Ciencias de la Salud, San Pedro, Mexico
Background/Purpose: The use of biosimilars instead of its originator is a controversial subject with many implications. It is considered that a non medical switch should…
Abstract Number: 0488 • ACR Convergence 2021
Efficacy and Safety of Brepocitinib (Tyrosine Kinase 2/Janus Kinase 1 Inhibitor) for the Treatment of Active Psoriatic Arthritis: Results from a Phase 2b Randomized Controlled Trial
Philip Mease¹, Philip S Helliwell², Paula Silwinska-Stanczyk³, Malgorzata Miakisz⁴, Andrew Ostor⁵, Elena Peeva⁶, Michael Vincent⁶, Vanja Sikirica⁷, Randall Winnette⁸, Ruolun Qiu⁶, Gang Li⁷, Gang Feng⁶, Jean Beebe⁶ and David Martin⁶, ¹Swedish Medical Center/Providence St. Joseph Health and University of Washington, Seattle, WA, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, ³REUMATIKA – Centrum Reumatologii NZOZ, Warsaw, Poland, ⁴Twoja Przychodnia Centrum Medyczne Nowa Sol, Nowa Sol, Poland, ⁵Monash University, Cabrini Hospital & Emeritus Research, Melbourne, Australia, ⁶Pfizer Inc, Cambridge, MA, ⁷Pfizer Inc, Collegeville, PA, ⁸Pfizer Inc, New York, NY
Background/Purpose: Brepocitinib is a small-molecule tyrosine kinase 2/Janus kinase 1 inhibitor that has shown promising results in an oral formulation for plaque psoriasis and alopecia…
Abstract Number: 1432 • ACR Convergence 2021
Modulation of Auto-Inflammation with a Novel Selective Cyclic GMP-AMP Synthase (cGAS) Inhibitor in a Trex1-/- Model
Kelly Pike¹, Alexandre Caron¹, Emilie Bérubé¹, Ramsay Beveridge¹, Marc-Oliver Boily¹, Jason Burch¹, Valerie Dumais¹, Nadine Fradet¹, Samuel Gaudreault¹, Daniel McKay¹, Marianne Raymond¹, Eleftheria Seliniotakis¹, Daniel Sietsema³, Alexander Skeldon¹, Miguel St.-Onge¹, Li Wang² and Michael Crackower², ¹Ventus Therapeutics, Montréal, QC, Canada, ²Ventus Therapeutics, Waltham, MA

Background/Purpose: The detection of viral nucleic acids (NA) elicits a transient type I interferon (IFN) response central to antiviral immunity. Chronic type I IFN responses…
Abstract Number: 0537 • ACR Convergence 2021
The Clinical, Oral Small Molecule IRAK4 Inhibitor, GS-5718, Improves Survival and Reduces Disease Pathologies by Modulating Multiple Inflammatory Endpoints in the Murine NZB/W Model of Spontaneous Lupus
Adam Yadon¹, Vanessa Gorney², Angie Hammond³, Ethan Grant² and Astrid Clarke³, ¹Gilead Sciences, Inc., Seattle, WA, ²Gilead Sciences, Inc, Foster City, CA, ³Gilead Sciences, Inc, Seattle, WA
Background/Purpose: Lupus is a heterogenous autoimmune disease characterized by loss of immune tolerance, production of nucleic acid:autoantibody immune complexes, immune cell hyperactivation, and increased proinflammatory…
Abstract Number: 1452 • ACR Convergence 2021
COVID-19 Infection Among Autoimmune/Auto-inflammatory Rheumatic Disease Patients: Data from an Observational Study
Clio Mavragani¹, Athanasios-Dimitrios Bakasis², Kyriaki Boki³, Athanasios Tzioufas⁴, Panayiotis Vlachoyiannopoulos¹, Ioanna Stergiou⁵, Fotini Skopouli⁶ and Haralampos Moutsopoulos⁷, ¹National and Kapodistrian University of Athens, Athens, Greece, ²1 Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ³3 Rheumatology Unit, Sismanoglio General Hospital, Athens, Greece, Athens, Greece, ⁴Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, ⁵Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, ⁶Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Athens, Greece, ⁷Athens Academy of Athens, Chair Medical Sciences/Immunology, Athens, Greece
Background/Purpose: The impact of COVID-19 infection in patients with autoimmune/auto-inflammatory rheumatic diseases (AARD) under immunomodulatory treatment is not entirely clear and deeper knowledge is of…
Abstract Number: 0539 • ACR Convergence 2021
Vgll3-transgenic Autoimmune Mice Display Features of Cutaneous Fibrosis
Mehrnaz Gharaee-Kermani¹, Allison Billi², Marisa Hildebrandt², Jacob Martens³, Rachael Wasikowski², Joanne (Michelle) Kahlenberg¹ and Johann Gudjonsson², ¹Internal Medicine - Division of Rheumatology and Department of Dermatology, University of Michigan, Ann Arbor, MI, ²Department of Dermatology, University of Michigan, Ann Arbor, MI, ³Graduate Program in Immunology, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, MI
Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast accumulation, and extracellular matrix remodeling and can lead to disfiguring skin changes. In cutaneous lupus, scar formation…
Abstract Number: 1454 • ACR Convergence 2021
RHAPSODY: Rilonacept, an IL-1α and IL-1β Trap, Resolves Pericarditis Episodes and Reduces Risk of Recurrence in a Phase 3 Trial of Patients with Recurrent Pericarditis
Allan Klein¹, Massimo Imazio², Paul Cremer¹, Antonio Brucato³, Antonio Abbate⁴, Fang Fang⁵, Antonella Insalaco⁶, Martin LeWinter⁷, Basil S. Lewis⁸, David Lin⁹, Sushil A. Luis¹⁰, Stephen J. Nicholls¹¹, Arian Pano⁵, Alistair Wheeler¹², Liangxing Zou⁵ and John F Paolini⁵, ¹Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ²University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy, ³Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁶Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁷Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁸Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ⁹Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ¹⁰Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda
Background/Purpose: Recurrent pericarditis (RP) is an autoinflammatory disease with no FDA-approved therapies. RHAPSODY, a global Phase 3 study, evaluated rilonacept, a once-weekly IL-1α/IL-1β trap, in…
Abstract Number: 0565 • ACR Convergence 2021
Increased Risk for Inflammatory Arthritis in Veterans with Depression or Anxiety
Brian Breviu¹, Shaobo Pei¹, Matthew Kirkpatrick², Bingjian Feng¹ and Jessica Walsh³, ¹Salt Lake City Veteran Affairs and University of Utah Medical Centers, Salt Lake City, UT, ²University of Utah, Salt Lake City, UT, ³Salt Lake City Veteran Affairs Medical Center (VAMC)/University of Utah Hospital, Salt Lake City, UT
Background/Purpose: Depression and anxiety are common in patients with inflammatory arthritis (IA), and have been reported as risk factors for various inflammatory diseases. The study…
Abstract Number: 1457 • ACR Convergence 2021
Clinical Heterogeneity of the VEXAS Syndrome: A Case Series
Matthew Koster¹, Taxiarchis Kourelis¹, Kaaren Reichard¹, Tanaz Kermani², David Beck³, Daniela Ospina Cardona⁴, Matthew Samec¹, Abhishek Mangaonkar¹, Kebede Begna¹, Christopher Hook¹, Jennifer Oliveira¹, Samih Nasr¹, Benedict Tiong⁵, Mrinal Patnaik¹, Michelle Burke¹, Clement Michet¹ and Kenneth Warrington⁶, ¹Mayo Clinic, Rochester, MN, ²University of California Los Angeles, West Hills, CA, ³National Institutes of Health, Bethesda, MD, ⁴National Institutes of Health, Germantown, MD, ⁵University of California Los Angeles Medical Center, Culver City, CA, ⁶Mayo Clinic, Rochester, Minnesota
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently described myeloid-driven autoinflammatory condition caused by somatic mutations affecting methionine-41 (p.Met41) in the…
Abstract Number: 0947 • ACR Convergence 2021
Cytokine Competent Gut-joint Migratory T Cells Contribute to Inflammation in the Joint
Adam Lefferts¹, David Claypool¹, Eric Norman¹, Uma Kantheti¹ and Kristine Kuhn², ¹University of Colorado, Anschutz Medical Campus, Aurora, CO, ²University of Colorado Anschutz Medical Campus, Aurora, CO
Background/Purpose: Although studies have identified the presence of gut-associated cells in the enthesis of joints affected by spondyloarthritis, a direct link through cellular transit between…
Abstract Number: 1508 • ACR Convergence 2021
Decrease of Angiogenic T Cells Associated to the Presence of Interstitial Lung Disease in Patients with Connective Tissue Diseases
Verónica Pulito-Cueto¹, Sara Remuzgo-Martinez¹, Fernanda Genre¹, Belén Atienza-Mateo², Victor M. Mora-Cuesta³, David Iturbe-Fernández³, Leticia Lera-Gómez¹, Raquel Perez-Fernández¹, Pilar Alonso-Lecue⁴, Javier Rodriguez-Carrio⁵, Diana Prieto-Peña⁶, Virginia Portilla⁶, Ricardo BLANCO⁷, Alfosno Corrales⁶, José M. Cifrián⁸, Raquel López-Mejías¹ and Miguel Ángel gonzalez-Gay⁹, ¹Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Santander, Spain, ²Group "Research in genetic epidemiology and atherosclerosis of systemic diseases and in bone metabolic diseases of the locomotor system", IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System; Department of Pneumology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁴Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Pneumology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁵Deparment of Functional Biology, Immunology Area, Faculty of Medicine, Universidad de Oviedo, Oviedo, Asturias, Spain., Oviedo, Spain, ⁶Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁷Hospital University Marqués de Valdecilla, Santander, Spain, ⁸Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; Department of Pneumology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain, ⁹Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Division of Rheumatology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa
Background/Purpose: Interstitial lung disease (ILD) is one of the most significant complications of connective tissue diseases (CTD) leading to an increase of the morbidity and…

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