Abstracts tagged "Autoinflammatory diseases"

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: 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: 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: 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: 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: 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…
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…

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