Abstracts tagged "Autoinflammatory diseases"

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: 0162 • ACR Convergence 2020
Identifying Sleep Problems in Systemic Juvenile Idiopathic Arthritis (SJIA) Patients with Patient-reported Outcomes (PRO) Questionnaires
Kim Nguyen¹, Christopher Towe², Shima Yasin³, Alexei Grom⁴, Hermine I Brunner⁵ and Grant Schulert⁵, ¹Veterans Affairs Cincinnati, University of Cincinnati Medical Center, Cincinnati, ²Cincinnati Children's Medical Hospital Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) can cause severe and chronic multisystem involvement. Medical therapies including high-dose corticosteroids can have significant side effects affecting sleep.…
Abstract Number: 1152 • ACR Convergence 2020
IL-18: A Biomarker That Reflects Disease Activity, Could It Be the Next Disease Activity Measure in Systemic Juvenile Idiopathic Arthritis?
Shima Yasin¹, Thuy Do², Sanjeev Dhakal², Elizabeth Baker², Alexei Grom³ and Grant Schulert⁴, ¹Cincinnati Children's Hospital Medical Center, Liberty twp, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with prominent innate immune activity. Disease presentation and flares could largely mimic infections with fever…
Abstract Number: 1636 • ACR Convergence 2020
8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab
Dror Mevorach¹ and Netanel Karbian¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…
Abstract Number: 0164 • ACR Convergence 2020
What’s in a Name? Patient and Family Perspectives on the Naming of Systemic Juvenile Idiopathic Arthritis
Mariana Correia Marques¹, Rashmi Sinha², Karen Durrant³, Sivia Lapidus⁴, Nicole Tennermann⁵, Saskya Angevare⁶, Leah Bush⁷, Kari Cupp⁸, Jonathan Hausmann⁹, David Maher¹⁰, Shalla Newton¹⁰, Michael Ombrello¹¹, Phillip Reardon⁸, Rebecca Trachtman¹², Fatma Dedeoglu⁵ and Grant Schulert¹³, ¹Boston Children`s Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, ²SJIA Foundation, Cincinnati, ³Autoinflammatory Alliance, San Francisco, CA, ⁴The Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Montclair, NJ, ⁵Boston Children's Hospital, Boston, MA, ⁶Autoinflammatory Alliance, Amersfoort, Netherlands, ⁷Systemic JIA Foundation, Cincinnati, OH, ⁸Systemic JIA Foundation, Cincinnati, ⁹Boston Children's Hospital / Beth Israel Deaconess Medical Center, Cambridge, MA, ¹⁰Still's Disease, the 411, National organization, ¹¹Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD, ¹²Icahn School of Medicine at Mount Sinai, New York, NY, ¹³PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: The childhood inflammatory disorder systemic juvenile idiopathic arthritis (SJIA) has historically had several names, including Still’s disease and systemic juvenile rheumatoid arthritis. While its…
Abstract Number: 1156 • ACR Convergence 2020
Comparison of Immunological Biomarkers and Lung Histology in Patients with Elevated IL18 – Pulmonary Alveolar Proteinosis and Recurrent Macrophage Activation Syndrome (IL-18PAP-MAS) and Other Inflammatory Lung Diseases
Alhanouf Alsaleem¹, Adriana de Jesus², Sofia Torreggiani³, Chyi-Chia Lee⁴, Les Folio⁵, Huy Do⁶, Andrew Oler⁷, Caroline Kim³, Stewart Levine⁸, Anthony Suffredini⁹, Cem Gabay¹⁰, Joseph Fontana¹¹, Scott Canna¹² and Raphaela Goldbach-Mansky¹³, ¹Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁴Pathology Department/NCI/NIH, Bethesda, MD, ⁵Radiology and Imaging Services/NIH, Bethesda, MD, ⁶Radiology and Imaging Sciences, Bethesda, MD, ⁷Bioinformatics and Computational Biosciences Branch/NIAID/NIH, Bethesda, MD, ⁸Laboratory of Asthma and Lung Inflammation, Division of Intramural Research, NHLBI, NIH,, Bethesda, MD, ⁹Critical Care Medicine Department, Clinical Center, NIH, Bethesda, MD, ¹⁰University Hospitals of Geneva, Geneva, Switzerland, ¹¹NHLBI/NIH, Bethesda, MD, ¹²University of PIttsburgh, Pittsburgh, PA, ¹³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: Recently, pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS) have been reported in rare patients (pts) with systemic juvenile idiopathic arthritis (SJIA)…
Abstract Number: 1711 • ACR Convergence 2020
Musculoskeletal Sarcoidosis Learning Module for Internal Medicine Trainees: Developing a Rheumatology Curriculum
Kristen Fadel¹, Evan Dombrosky², Huzaefah Syed³, Joshua Gavin¹, Abhishek Nandan⁴, Stamatina Danielides⁵, Beth Rubinstein⁶, Yashswee KC⁷ and Sandra Johnson⁸, ¹Virginia Commonwealth University School of Medicine, Richmond, ²Virginia Commonwealth University School of Medicine, Glen Allen, VA, ³VCU Health, Richmond, VA, ⁴VCUHS and VA Medical Center- Richmond, VA, Richmond, VA, ⁵Virginia Commonwealth University School of Medicine, Richmond, VA, ⁶Virginia Commonwealth University, Richmond, VA, ⁷Virginia Commonwealth University School of Medicine, Henrico, VA, ⁸Virginia Commonwealth University Health Systems, Richmond
Background/Purpose: Sarcoidosis is a systemic, multi-organ disease frequently overlooked in the development of a differential diagnosis. Although typically considered a pulmonary disease, management decisions often fall to the Rheumatologist especially in the context of sarcoid-related arthritis or the initiation of immunosuppressive therapy. We…
Abstract Number: 0171 • ACR Convergence 2020
Interferon Response Gene Expression Differs in Whole Blood, Peripheral Blood Mononuclear Cells, Monocytes, T Cells, B Cells, and NK Cells in Patients with the Autoinflammatory Interferonopathies, CANDLE and SAVI
Jacob Mitchell¹, Sara Alehashemi², Bernadette Marrero³, Yan Huang⁴, Sofia Torreggiani¹, Lena Bichell¹, Gina Montealegre Sanchez⁵, Raphaela Goldbach-Mansky⁶ and Adriana de Jesus⁷, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ³Computational Systems Biology Section/NIAID/NIH, Bethesda, MD, ⁴NIH, Bethesda, ⁵NIAID/NIH, Rockville, MD, ⁶Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD, ⁷Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD
Background/Purpose: The disease progression of patients (pts.) with type-I interferon (IFN)-mediated diseases undergoing treatment with JAK1 and JAK2 inhibitors is monitored in part by measuring…
Abstract Number: 1157 • ACR Convergence 2020
Health and Socioeconomic Outcomes in a Neonatal-Onset Multisystem Inflammatory Disease (NOMID) Cohort Followed for a Median of Fifteen Years
Sara Alehashemi¹, Megha Garg², Kim Johnson³, Kelly King⁴, Chris Zalewski⁴, Debbie Payne⁵, Adriana de Jesus⁶, Joseph Snow⁷, Wadih Zein⁵, M. Teresa Magone⁵, Rachel Bishop⁸, Carmen Brewer⁴, Jeff Kim⁴, Scott Paul⁹, John Butman¹⁰ and Raphaela Goldbach-Mansky¹¹, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ²NIH/NIAID, Rochester, NY, ³NIH, NIAID, Bethesda, ⁴NIH, NIDCD, Bethesda, MD, ⁵NIH/NEI, Bethesda, MD, ⁶Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ⁷NIH, NIMH, Bethesda, MD, ⁸NIH, NEI, Bethesda, MD, ⁹NIH, CC/RMD, Bethesda, MD, ¹⁰NIH, CC/DRD, Bethesa, MD, ¹¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: Patients (pts) with NOMID have systemic inflammation and organ damage such as sensorineural hearing loss, hydrocephalus, optic nerve atrophy and growth plate defects. IL-1 blocking…
Abstract Number: 1855 • ACR Convergence 2020
Elevated Calprotectin Levels Reveal Loss of Vascular Pattern and Atrophy of Villi in Ileum Using Digital Chromo-endoscopy and Magnification Colonoscopy in Patients with Spondyloarthritis Without Inflammatory Bowel Disease
Consuelo Romero-Sanchez¹, Cristian Florez-Sarmiento², Valerie Khoury-Rosas³, Wilson Bautista-Molano⁴, Magaly Chamorro-Melo⁵, Diego Alejandro Jaimes⁶, Adriana Beltran-Ostos⁷, Juliette De Avila⁸, Alejandro Ramos-Casallas⁸, Juan Manuel Bello-Gualtero⁹, Jaiber Gutierrez⁵, Cesar Pacheco Tena¹⁰, Philipe Chalem Choueka¹¹ and Viviana Parra-Izquierdo¹², ¹Hospital Militar Central, Rheumatology and Immunology Department, Universidad Militar Nueva Granada / Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada /Universidad El Bosque, Cellular and Molecular Immunology Group -InmuBo-, School of Dentistry, Bogotá D.C., Colombia, ²Universidad El Bosque, Cellular and Molecular Immunology Group -InmuBo- / Grastroadvanced SAS, Bogotá D.C., Colombia, ³Universidad EL Bosque / School of Medicine, Bogotá D.C., Colombia, ⁴University Hospital Fundación Santa Fé de Bogotá and Universidad El Bosque, Bogotá, Colombia, ⁵Hospital Militar Central, Rheumatology and Immunology Department, School of Medicine, Bogotá D.C., Colombia, ⁶Clínicos IPS- Universidad de la Sabana, Bogotá D.C., Colombia, ⁷Hospital Militar Central, Subdirección de docencia e Investigación, Bogotá D.C., Colombia, ⁸Universidad El Bosque, Cellular and Molecular Immunology Group -InmuBo-, School of Dentistry, Bogotá D.C., Colombia, ⁹Hospital Militar Central, Rheumatology and Immunology Department, Universidad Militar Nueva Granada/ Clinical Immunology Group, Hospital Militar Central, School of Medicine, Universidad Militar Nueva Granada, Bogotá D.C., Colombia, ¹⁰Universidad Autonoma de Chihuahua, Chihuahua, Chihuahua, Mexico, ¹¹Fundacion Instituto de Reumatología Fernando Chalem, Universidad El Rosario, Bogotá D.C., Colombia, ¹².Universidad El Bosque, Cellular and Molecular Immunology Group -InmuBo- / Grastroadvanced SAS, Bogotá D.C., Colombia
Background/Purpose: Fecal calprotectin (FC) is a well bio-marker related to mucosal inflammation. Digital chromo-endoscopy (DCE) with magnification is a technique to identify microscopic inflammation. The…
Abstract Number: 0172 • ACR Convergence 2020
Early Treatment and IL1RN Single Nucleotide Polymorphisms Affect Response to Anakinra in Systemic Juvenile Idiopathic Arthritis
Marianna Nicoletta Rossi¹, Manuela Pardeo², Denise Pires Marafon², Emanuela Sacco², Chiara Passarelli³, Claudia Bracaglia², Chiara Perrone³, Anna Tulone⁴, Giusi Prencipe⁵ and Fabrizio De Benedetti⁶, ¹Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Lazio, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ³U.O.C. Laboratory of Medical Genetics, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁴Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁵Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ⁶Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) represents 10-20% of all chronic arthritis during childhood. The interleukin 1 (IL-1) play a pivotal role in the pathogenesis…

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