Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1689 • ACR Convergence 2022
Oral Ginger Supplementation Counteracts NETosis in Autoimmune Mouse Models and in Healthy Humans
Ramadan Ali¹, Miela Zahavi², Christine Rysenga¹, Cyrus Sarosh³, Claire Hoy¹, Kristen Demoruelle⁴ and Jason S Knight⁵, ¹University of Michigan, Ann Arbor, MI, ²Universtiy of Michigan, Ann Arbor, MI, ³University of Michigan, Temperance, MI, ⁴University of Colorado Anschutz Medical Campus, Aurora, CO, ⁵University of Michigan, Division of Rheumatology, Ann Arbor, MI
Background/Purpose: It has previously been reported that 6-gingerol, the most abundant phytochemical in ginger root, inhibits neutrophil phosphodiesterase activity and thereby counteracts neutrophil hyperactivity in…
Abstract Number: 1943 • ACR Convergence 2022
Prevalence and Evolution of Undifferentiated Periodic Fever Syndromes in an Academic Pediatric Rheumatology Practice
Jacob Anderson¹, Catherine Bingham², Lisabeth Scalzi³, Natalya Gaffney⁴, Kimberly Hays⁵, Brandt Groh² and Timothy Hahn², ¹Phoenix Children's Hospital, Phoenix, AZ, ²Penn State Children's Hospital, Hershey, PA, ³Penn State Health, Hummelstown, PA, ⁴Self-Employed, Carlisle, PA, ⁵Penn State Health, Hershey, PA
Background/Purpose: Periodic Fever Syndromes (PFS) are common referrals to pediatric rheumatology clinics. While the syndrome of Periodic Fevers, Aphthous stomatitis, Pharyngitis, and Adenitis (PFAPA) is…
Abstract Number: 0134 • ACR Convergence 2022
Combination Treatment with Baricitinib and Pulse Steroids in Severe COVID19: A
Francesco Ferro¹, Elena Elefante², Italiano Nazzareno², Gaetano La Rocca³, Davide Schilirò², Michele Moretti², Roberto Mozzo⁴, De Simone Luigi⁴, Chiara Baldini⁵ and Marta Mosca², ¹Clinical and Experimental Medicine Department, Azienda Ospedaliero-Universitaria Pisana, Pisa, Pisa, Italy, ²University of Pisa, Pisa, Italy, ³University of Pisa, Rheumatology Unit, Palermo, Palermo, Italy, ⁴Anesthesiology & Intensive Care Unit/Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy, ⁵University of Pisa, Pisa, Pisa, Italy
Background/Purpose: Growing evidence from RCTs and real-life experiences has highlighted important similarities between severe COVID19 and rapidly progressive interstitial lung diseases (RP-ILD) occurring in connective…
Abstract Number: 0812 • ACR Convergence 2022
3-years Safety and Efficacy Outcomes of Canakinumab Treatment in Cryopyrin-associated Periodic Syndromes (CAPS) – Data from the RELIANCE Registry
Jasmin B. Kuemmerle-Deschner¹, Birgit Kortus-Goetze², Prasad T. Oommen³, Ales Janda⁴, Juergen Rech⁵, Catharina Schuetz⁶, Tilmann Kallinich⁷, Frank Weller-Heinemann⁸, Gerd Horneff⁹, Ivan Foeldvari¹⁰, Florian Meier¹¹, Michael Borte¹², Tobias Krickau¹³, Julia Weber-Arden¹⁴ and Norbert Blank¹⁵, ¹Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, ²Division of Nephrology, University of Marburg, Marburg, Germany, ³Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁴Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ⁵University Clinic Erlangen, Erlangen, Germany, ⁶Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ⁷Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁸Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ⁹Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ¹⁰Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹¹Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany, and Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt, Germany, ¹²ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, Germany, ¹³Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, ¹⁴Novartis Pharma GmbH, Nuernberg, Germany, ¹⁵Rheumatology, University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are monogenic autoinflammatory diseases with severe systemic inflammation. The IL-1β inhibitor canakinumab (CAN) leads to a rapid remission of CAPS…
Abstract Number: 1698 • ACR Convergence 2022
LINE1 Reverse Transcriptase Inhibitors Abrogate Type 1 Interferon Responses
Daniel Rios¹, Nafeeza Hafeez¹, Brian Desrosiers¹, Shradha Rao¹, Jess Floro¹, Joelle Baddour¹, Nisha Perez², Greg Bisacchi², Oliver Saunders², Donna Romero¹, Menachem Fromer², Enrique Garcia-Rivera², Wilson McKerrow², Julie Behr², Bryan Thornlow², Dennis Zaller², David Proia² and Wenyan Miao², ¹Rome Therapeutics, Cambridge, MA, ²Rome Tx, Cambridge, MA
Background/Purpose: Transposable elements (TEs) are mobile DNA elements that can replicate and move from one position to another within the host genome. Through co-evolution, TEs…
Abstract Number: 2101 • ACR Convergence 2022
Outcomes of Autoimmune Hemolytic Anemia in Patients with Systemic Lupus Erythematosus: A Nationwide Inpatient Sample Study
Ekrem Turk, Vaishali Deenadayalan, Khaldun Obeidat and Pierre Rodriguez, John H. Stroger, Jr. Hospital of Cook County, Chicago, IL
Background/Purpose: Autoimmune Hemolytic Anemia (AIHA) is one of the manifestations of Systemic lupus erythematosus (SLE), an autoimmune connective tissue disease. AIHA may be present in…
Abstract Number: 0138 • ACR Convergence 2022
Patients with Familial Mediterranean Fever (FMF) Under Canakinumab Treatment – Long-term Efficacy and Safety Interim Data of the RELIANCE Registry
Joerg Henes¹, Jasmin B. Kuemmerle-Deschner², Tobias Krickau³, Tilmann Kallinich⁴, Frank Dressler⁵, Gerd Horneff⁶, Florian Meier⁷, Ivan Foeldvari⁸, Frank Weller-Heinemann⁹, Birgit Kortus-Goetze¹⁰, Markus Hufnagel¹¹, Juergen Rech¹², Prasad T. Oommen¹³, Julia Weber-Arden¹⁴ and Norbert Blank¹⁵, ¹Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ²Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, ³Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, ⁴Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁵Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ⁶Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁷Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany, and Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt, Germany, ⁸Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ⁹Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ¹⁰Division of Nephrology, University of Marburg, Marburg, Germany, ¹¹Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹²University Clinic Erlangen, Erlangen, Germany, ¹³Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ¹⁴Novartis Pharma GmbH, Nuernberg, Germany, ¹⁵Rheumatology, University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and serositis, with a risk of severe complications (e. g.…
Abstract Number: 0813 • ACR Convergence 2022
Canakinumab in Patients with Tumor Necrosis Factor Receptor-associated Periodic Syndrome (TRAPS) – Long-term Efficacy and Safety Data from a RELIANCE Registry Interim Analysis
Norbert Blank¹, Catharina Schuetz², Joerg Henes³, Tilmann Kallinich⁴, Prasad T. Oommen⁵, Michael Borte⁶, Markus Hufnagel⁷, Ales Janda⁸, Julia Weber-Arden⁹ and Jasmin B. Kuemmerle-Deschner¹⁰, ¹Rheumatology, University Hospital Heidelberg, Heidelberg, Germany, ²Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ³Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ⁴Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁵Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁶ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, 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, ⁸Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ⁹Novartis Pharma GmbH, Nuernberg, Germany, ¹⁰Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany
Background/Purpose: TRAPS is a rare hereditary autoinflammatory disease characterized by periodic fever and severe systemic and organ inflammation. Successful treatment was achieved with the interleukin-1β…
Abstract Number: 1701 • ACR Convergence 2022
High TNFAIP3-Transfected Cell Activity: An In Vitro Model for Phagocyte Study of Monogenic Behçet’s Disease Pathophysiology
Patricia Pontes Aires¹, André Cunha¹, DANIELA GERENT PETRY PIOTTO¹, Maria Teresa Terreri² and Sandro Perazzio¹, ¹Universidade Federal de São Paulo, São Paulo, Brazil, ²Universidad Federal São Paulo, São Paulo, Brazil
Background/Purpose: A20 haploinsufficiency (A20HA), an important monogenic etiology of Behçet's disease (BD), is caused by loss-of-function mutations in TNFAIP3. Although monocytes play an important role…
Abstract Number: 2194 • ACR Convergence 2022
Is Vaccination Against Covid-19 Associated with Autoimmune Rheumatic Disease Flare? Self-controlled Case Series Analysis Using Data from the Clinical Practice Research Datalink (Aurum)
Georgina Nakafero¹, Matthew J. Grainge¹, Tim Card¹, Christian Mallen², Jonathan S. Nguyen van Tam¹, Hywel C. Williams¹ and Abhishek Abhishek¹, ¹University of Nottingham, Nottingham, United Kingdom, ²Keele University, Keele, United Kingdom
Background/Purpose: It is unclear whether autoimmune rheumatic disease (AIRD) flares are associated with the recent prior Covid-19 vaccination, and whether the association was influenced by…
Abstract Number: 0139 • ACR Convergence 2022
A Clinical Study Designed to Support a Demonstration of Interchangeability Between AVT02 and Reference Adalimumab (Humira®)
Steven Feldman¹, Heimo stroissnig², Richard Kay³, Joanna Sobierska⁴, Roshan Dias⁴, Eric Guenzi⁵, Hendrik Otto², Halimuniyazi Haliduola², Abid Sattar⁶, Ruth Ruffieux⁴, Matjaz Steijger⁴ and Fausto Berti⁴, ¹Wake Forest School of Medicine, Winston-Salem, NC, ²Alvotech, Jülich, Germany, ³RK Statistics, Bakewell, United Kingdom, ⁴Alvotech, Zürich, Switzerland, ⁵UGA Biopharma, Bonn, Germany, ⁶Alvotech, London, United Kingdom
Background/Purpose: To support a demonstration of interchangeability of proposed biosimilar AVT02 and Humira® (reference adalimumab).Methods: Participants with chronic plaque psoriasis were selected for the study…
Abstract Number: 0815 • ACR Convergence 2022
Hyper-IgG4 in Patients with Systemic Autoinflammatory Disease
Brianne Navetta-Modrov and QingPing Yao, Stony Brook University, Stony Brook, NY
Background/Purpose: Hyper-immunoglobulin G4 (IgG4) has been reported in various conditions including autoimmune disease, gastrointestinal disorders, lymphoproliferative/granulomatous diseases and solid tumors. IgG4-related disease is an immune-mediated…
Abstract Number: 1721 • ACR Convergence 2022
Protective Effects of the Natural Antioxidant Taxifolin in Models of Lupus and Antiphospholipid Syndrome
Christine Rysenga¹, Linda May-Zhang², Miela Zahavi³, Jason S Knight⁴ and Ramadan Ali¹, ¹University of Michigan, Ann Arbor, MI, ²Blue California, Rancho Santa Margarita, CA, ³Universtiy of Michigan, Ann Arbor, MI, ⁴University of Michigan, Division of Rheumatology, Ann Arbor, MI
Background/Purpose: Taxifolin, also known as dihydroquercetin, is a bioactive flavonoid commonly found in apples, onions, French maritime bark, and milk thistle. Given its potent anti-inflammatory…
Abstract Number: 2209 • ACR Convergence 2022
Efficacy of Secukinumab in Enthesitis-related Arthritis and Juvenile Psoriatic Arthritis Subtypes of Juvenile Idiopathic Arthritis: Results from a Randomized, Phase 3 Study
Hermine Brunner¹, Elena Chertok², Joke Dehoorne³, Gerd Horneff⁴, Tilmann Kallinich⁵, Ingrid Louw⁶, Maria Alessio⁷, Sandrine Compeyrot-Lacassagne⁸, Bernard Lauwerys⁹, Neil Martin¹⁰, Katherine Marzan¹¹, W Patrick Knibbe¹², Ruvie Martin¹³, Xuan Zhu¹⁴, sarah whelan¹⁵, Luminita Pricop¹⁶, Daniel Lovell¹⁷, Alberto Martini¹⁸ and Nicola Ruperto¹⁹, ¹Division of Rheumatology, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Department of Pediatrics, Cincinnati, OH, ²Voronezh State Medical University, Voronezh, Russia, ³University Hospital Gent, Gent, Belgium, ⁴Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁵Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁶Panaroma Medical Centre, Cape Town, South Africa, ⁷Policlinico Federico II di Napoli, Napoli, Italy, ⁸Great Ormond Street Hospital for Children, London, United Kingdom, ⁹Cliniques Universitaires Saint-Luc, Brussels, Belgium, ¹⁰Royal Hospital for Children, Glasgow, Scotland, United Kingdom, ¹¹Children's Hospital Los Angeles, Los Angeles, CA, ¹²St. Luke's Hospital System, Meridian, ID, ¹³Novartis Pharmaceutical Corporation, East Hanover, NJ, ¹⁴Novartis Pharmaceuticals Corporation, Basking Ridge, NJ, ¹⁵Novartis Ireland Ltd, Crumlin, Dublin, Ireland, ¹⁶Novartis Pharmaceuticals Corporation, East Hanover, NJ, ¹⁷Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹⁸Istituto Giannina Gaslini, Genova, Italy, ¹⁹IRCCS Istituto Giannina Gaslini; PRINTO, Clinica Pediatrica e Reumatologia, Genova, Italy
Background/Purpose: Juvenile idiopathic arthritis (JIA) categories of enthesitis-related arthritis (ERA) and juvenile psoriatic arthritis (JPsA) represent pediatric counterparts of adult non-radiographic axial spondyloarthritis and psoriatic…
Abstract Number: 0142 • ACR Convergence 2022
A Systemic Autoinflammatory Syndrome Caused by NEMO Exon 5 Skipping
Bin Lin¹, Adriana Almeida de Jesus², Eric Karlins¹, Dana Kahle¹, Sophia Park³, Andre Rastegar¹, Jacob Mitchell⁴, sofia Torregiani¹, Farzana Bhuyan⁵, Sara Alehashemi⁶, Kader Cetin Gedik⁷, Kat Uss⁸, Eric Hanson⁹, Amer Khojah¹⁰, Eveline Wu¹¹, Christiaan Scott¹², Timothy Ronan Leahy¹³, Emma J. MacDermott¹³, Orla G. Killeen¹³, Chyi-Chia Richard Lee¹⁴, Thaschawee Arkachaisri¹⁵, Zoran Gucev¹⁶, Kathryn Cook¹⁷, Vafa Mammadova¹⁸, Gulnara Nasrullayeva¹⁸, Mariana Correia Marques¹⁹, Abigail Bosk²⁰, Seza Ozen²¹, Abigail Lang²², Brian Nolan²³, Scott Canna²⁴, Maude Tusseau²⁵, Emilie Chopin²⁵, Guilaine Boursier²⁶, Danielle Fink²⁷, Douglas Kuhns²⁷, Clifton Dalgard²⁸, Alexandre Belot²⁹, Timothy Moran³⁰, Andrew Oler³ and Raphaela Goldbach-Mansky³¹, ¹NIH, Bethesda, MD, ²NIAID, NIH, Silver Spring, MD, ³NIAID, Bethesda, MD, ⁴NIAID NIH, Bethesda, MD, ⁵National Institutes of Health, Bethesda, MD, ⁶NIH/NIAID/TADS, Clarksville, MD, ⁷Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ⁸National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁹Indiana University School of Medicine, Indianapolis, IN, ¹⁰Umm Al-Qura University, Makkah, Saudi Arabia, ¹¹University of North Carolina, Chapel Hill, NC, ¹²University of Cape Town, Red Cross War Memorial Children's Hospital, Cape Town, South Africa, ¹³CHI at Crumlin, Dublin, Ireland, ¹⁴National Cancer Institute, National Institutes of Health, Bethesda, MD, ¹⁵KK Women's and Children's Hospital, SingHealth, Singapore, Singapore, ¹⁶University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, ¹⁷Akron Children's Hospital, Akron, OH, ¹⁸Azerbaijan Medical University, Baku, Azerbaijan, ¹⁹National Institute of Arthritis and Musculoskeletal and Skin Diseases / Children`s National Hospital, Bethesda, MD, ²⁰Children's National Hospital, Washington, DC, ²¹Hacettepe University Faculty of Medicine, Ankara, Turkey, ²²Lurie Children's Hospital, Chicago, IL, ²³Ann & Robert H. Lurie Children's Hospital, Chicago, IL, ²⁴Children's Hospital of Philadelphia, Philadelphia, PA, ²⁵Service de Néphrologie, Rhumatologie, Dermatologie Pédiatriques, Centre de Référence des Rhumatismes Inflammatoires et Maladies Auto-Immunes Rares de l'Enfant (RAISE), Hôpital Femme-Mère-Enfant, Hospices Civils de Lyon, Bron, France, ²⁶Laboratoire de Génétique des Maladies Rares et Autoinflammatoires, Département de Génétique Médicale, Maladies Rares et Médecine Personnalisée, CEREMAIA, CHU de Montpellier, Univ Montpellier, Montpellier, France, ²⁷National Institute of Allergy and Infectious Diseases, National Institutes of Health, Frederick, MD, ²⁸The American Genome Center, Uniformed Services University of the Health Sciences, Bethesda, MD, ²⁹Hospices Civils de Lyon, Collonges au mont d'or, France, ³⁰University of North Carolina School of Medicine, Chapel Hill, NC, ³¹NIH/NIAID, Potomac, MD
Background/Purpose: NF-kB essential modulator (NEMO, encoded by IKBKG) is an essential gene in immune response, development, and cell death regulation. Mutations in NEMO have been…