Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0161 • ACR Convergence 2020
Validation of Patient-reported Outcomes (PRO) Lung Questionnaires for Systemic Juvenile Idiopathic Arthritis (SJIA) Patients at Risk for Lung Disease
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) involves dysregulation of inflammation and innate immunity, and can cause life-threatening complications including lung disease (LD). However, there are…
Abstract Number: 1063 • ACR Convergence 2020
Spirulina Stimulates Inflammatory Cytokine Production Through the STING and TLR Pathways in Dermatomyositis in Vitro
Christina Bax¹, Yubin Li¹, Spandana Maddukuri², Adarsh Ravishankar³, Jay Patel³, Daisy Yan¹, Josef Symon Concha¹ and Victoria Werth¹, ¹University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, PA, ²University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Montville, NJ, ³University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia
Background/Purpose: Spirulina, a popular herbal supplement, stimulates the immune system, as determined by in vitro and in vivo studies. Our recent epidemiologic data suggest that Spirulina…
Abstract Number: 1578 • ACR Convergence 2020
Rituximab Hypersensitivity in Rheumatic and Inflammatory Diseases: Role of Skin Testing
Sravani Penumarty¹, Javier Quintero Betancourt², Eugenio Capitle² and Reena Khianey², ¹Rutgers, Eastvale, CA, ²Rutgers, Newark, NJ
Background/Purpose: Infusion-related reactions have been reported with rituximab, a monoclonal antibody targeting the CD20 antigen on B cells, and may result in discontinuation of the…
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…
Abstract Number: 1158 • ACR Convergence 2020
Clinical Features and Outcomes in STING-Associated Vasculopathy with Onset in Infancy (SAVI)
Sofia Torreggiani¹, Sara Alehashemi², Jacob Mitchell¹, Gema Souto Adeva¹, Bin Lin¹, Jenna Wade¹, Gina Montealegre Sanchez³, Abdulrahman Alrasheed⁴, Sibel Balci⁵, Roberta Berard⁶, Borzutzky Arturo⁷, Jürgen Brunner⁸, Bjoern Buehring⁹, Al Adba Buthaina¹⁰, Caterina Cancrini¹¹, John Carter¹², Mireia Corbeto Lopez¹³, Fabrizio De Benedetti¹⁴, Huy Do¹⁵, Gregor Dueckers¹⁶, Les Folio¹⁵, Antonella Insalaco¹⁷, Rabia Miray Kisla Ekinci⁵, Michael Miller¹⁸, Marco Montes Cano¹⁹, Marie-Paule Morin²⁰, Seza Ozen²¹, Lucia Pacillo¹¹, Suzanne Ramsey²², Adam Reinhardt²³, Dax Rumsey²⁴, Laisa Santiago²⁵, Grant Schulert²⁶, Benjamin Wright²⁷, Adriana de Jesus²⁸ and Raphaela Goldbach-Mansky²⁹, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ³NIAID/NIH, Rockville, MD, ⁴King Abdullah Specialized Children Hospital, Riyadh, Riyadh, Saudi Arabia, ⁵Department of Pediatric Rheumatology, Cukurova University Faculty of Medicine, Adana, Turkey, ⁶London Health Sciences Centre, London, ON, Canada, ⁷Pontificia Universidad Católica de Chile, Santiago, Chile, ⁸Tirol Kliniken, Innsbruck, Innsbruck, Austria, ⁹Rheumazentrum Ruhrgebiet, Ruhr-University-Bochum, Herne, Germany, ¹⁰Sidra Medicine, Doha, Doha, Qatar, ¹¹Unit of Immune and Infectious Diseases, Scientific Institute for Research and Healthcare (IRCCS) Childrens’ Hospital Bambino Gesù, University Department of Pediatrics (DPUO); Department of Systems Medicine, University of Rome Tor Vergata, Roma, Italy, ¹²University of South Florida, Tampa, FL, ¹³Vall d’Hebron Hospital Universitari, Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain, ¹⁴Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹⁵Radiology and Imaging Sciences, Clinical Center, NIH, Bethesda, ¹⁶Helios Kliniken - Kinderklinik, HELIOS Klinikum Krefeld, Germany, Krefeld, Germany, ¹⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹⁸Feinberg School of Medicine, Northwestern University Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, ¹⁹Hospital Universitario Virgen del Rocío, Sevilla, Sevilla, Spain, ²⁰Université de Montréal, CHU Sainte-Justine, Montréal, Canada, ²¹Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Ankara, Turkey, ²²IWK Health Centre, Dalhousie University, Halifax, NS, Canada, ²³Boys Town National Research Hospital, Omaha, Omaha, NE, ²⁴Alberta Health Services – Edmonton Zone (Stollery Children’s Hospital), University of Alberta, Edmonton, AB, Canada, ²⁵Johns Hopkins All Children's Hospital, St. Petersburg, FL, ²⁶PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²⁷Mayo Clinic, Phoenix, AZ, ²⁸Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ²⁹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: STING-Associated Vasculopathy with Onset in Infancy (SAVI) is an autoinflammatory interferonopathy caused by gain-of-function mutations in STING1, characterized by peripheral vasculopathy and interstitial lung…
Abstract Number: 1950 • ACR Convergence 2020
Elevated Serum Gasdermin D N-terminal Implicates Macrophage Pyroptosis in Adult-onset Still’s Disease and Systemic Juvenile Idiopathic Arthritis
Hideto Nagai¹, Yohei Kirino², Hiroto Nakano³, Yosuke Kunishita¹ and Michael Ombrello⁴, ¹Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan, ²Yokohama City University Graduate School of Medicine, Yokohama, Japan, ³NIAMS, NIH, Bethesda, ⁴Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD
Background/Purpose: Elevation of serum IL-18 in adult-onset Still's disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA) suggests involvement of one or more inflammasome in these…

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