ACR Meeting Abstracts

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  • Abstract Number: 1146 • ACR Convergence 2020

    Pilot Study of an Internet-Based Pain Coping Skills Training Program for Patients with Systemic Lupus Erythematosus

    Kelli Allen1, Tyler Beuchamp2, Rebecca Cleveland3, Kimberlea Grimm1, David Hu1, Katie Huffman1, Frances Keefe4, Julie Norfleet1, Christine Rini5, Andres Santana1, Shruti Saxena Beem6 and Saira Sheikh7, 1University of North Carolina at Chapel Hill, Chapel HIll, NC, 2Medical College of Georgia, Augusta, GA, 3University of North Carolina, Chapel Hill, NC, 4Duke University Medical Center, Durham, NC, 5Northwestern University, Chicago, IL, 6University of North Carolina at Chapel Hill, Chapel Hill, 7University of North Carolina Thurston Arthritis Research Center, Chapel Hill

    Background/Purpose: Patients with Systemic Lupus Erythematosus (SLE) have increased symptoms of fatigue, chronic pain, depression and anxiety, which are associated with negative impacts on quality…
  • 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: 1151 • ACR Convergence 2020

    Implementation and Initial Experience with a Screening Protocol for Inflammatory Hyperferritinemia

    Michael Zhang1, Corinne Schneider2, Vinh Dang2 and Scott Canna3, 1Children's Hospital UPMC, Pittsburgh, PA, 2University of Pittsburgh, Pittsburgh, 3University of PIttsburgh, Pittsburgh, PA

    Background/Purpose: Macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) epitomize a diverse and deadly group of inflammatory hyperferritinemic syndromes. Early biomarkers distinguishing these syndromes, especially…
  • 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: 1159 • ACR Convergence 2020

    Novel STING1 Mutations Including in the Transmembrane Linker Region Cause STING-associated Vasculopathy with Onset in Infancy (SAVI)

    Bin Lin1, Dana Kahle1, Adriana Almeida de Jesus1, Sofia Torreggiani2, Jacob Mitchell2, Alexander Aue1, Zheng Ji3, Tengchuan Jin3 and Raphaela Goldbach-Mansky4, 1Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, 2Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, 3University of Science and Technology of China, Hefei, China (People's Republic), 4Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD

    Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease caused by gain-of-function (GOF) mutations in STING1/TMEM173 that encodes stimulator of interferon genes,…
  • Abstract Number: 1154 • ACR Convergence 2020

    Distinct Gene Signature Predicts Strong Clinical Responses to Canakinumab in Children with Systemic Juvenile Idiopathic Arthritis

    Emely Verweyen1, Alex Pickering2, Alexei Grom3 and Grant Schulert4, 1Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Harvard Medical School, Boston, MA, 3Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Canakinumab is a human anti-IL1β blocking agent that effectively neutralizes IL1β mediated signaling and is used to treat diseases such as systemic juvenile idiopathic…
  • Abstract Number: 1149 • ACR Convergence 2020

    Risk Score of Macrophage Activation Syndrome (MAS) in Patients with Systemic Juvenile Idiopathic Arthritis (sJIA)

    Simone Carbogno1, Denise Pires Marafon2, Giulia Marucci2, Emanuela Sacco2, Manuela Pardeo2, Alhanouf Alsaleem3, Sarka Fingerhutova4, Ferhat Demir5, Nastasia Cekada6, Mikhail Kostik7, Christoph Kessel8, Olga Vougiouka9, Alenka Gagro10, Jessica Tibaldi11, Francesca Minoia12, Ilaria Maccora13, Rayfel Schneider14, Pavla Dolezalova15, Betul Sozeri16, Marija Jelusic6, Antonella Insalaco2, Fabrizio De Benedetti17 and Claudia Bracaglia2, 1Pediatric Area, University of Milan, Milan, Italy, 2Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 3Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, 4Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, Charles University in Prague, Dlouha 534, Kladno, 27201, Czech Republic, 5University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology, Istanbul, Turkey, Istanbul, Turkey, 6University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, 7Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, Sto arrivando!, Russia, 8Department of Pediatric Rheumatology and Immunology, University Hospital Muenster, Muenster, Germany, Muenster, Germany, 9Second Department of Paediatrics, P. & A. Kyriakou Children’s Hospital, Athens, Greece, Athens, Greece, 10Children's Hospital Zagreb, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, 11UOC Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Italy, 12Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, 13Paediatric Rheumatology Unit, Anna Meyer Children's University Hospital, School of Human Health Science, Florence, Italy, Florence, Italy, 14University of Toronto, The Hospital for Sick Children, Toronto, Canada, 15Paediatric Rheumatology and Autoinflammatory Diseases Unit, General University Hospital, Prague, Czech Republic, Prague, Czech Republic, 16University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology Istanbul, Turkey, Istanbul, Turkey, 17Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy

    Background/Purpose: Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of Systemic Juvenile Idiopathic Arthritis (sJIA) with a significant mortality. A score that identify sJIA…
  • Abstract Number: 1162 • ACR Convergence 2020

    Comparison of Clinicopathologic and Imaging Features Between Chronic Nonbacterial Osteomyelitis and Its Mimickers: A Multi-national 450 Case-Control Study

    Yongdong Zhao1, Raymond Naden2, Melissa Oliver3, Zhaoyi Wang4, Eveline Wu5, Cassyanne Aguiar6, Jonathan Akikusa7, Ozge Basaran8, Kevin Cain1, Martina Capponi9, Nathan Donaldson10, Emily Fox11, Antonella Insalaco12, Annette Jansson13, Ummusen Kaya Akca14, Tzielan Lee15, Edoardo Marrani16, Kamran Mahmood17, Elizabeth Murray18, Farzana Nuruzzaman19, Karen Onel20, Manuela Pardeo12, Lauren Potts21, Nathan Rogers10, Anja Schnabel22, Gabriele Simonini23, Jennifer Soep10, Sara Stern24, Alexander Theos25, Yujuan Zhang26, Polly Ferguson27, Christian Hedrich28, Fatma Dedeoglu29, Hermann Girschick30, Ronald Laxer31 and Seza Ozen32, 1University of Washington, Seattle, WA, 2Department of Medicine, Middlemore Hospital, Auckland, Auckland, New Zealand, 3Department of Pediatric Rheumatology, Riley Children's Hospital, Carmel, IN, 4University of Washington, Bothell, WA, 5UNC Chapel Hill, Chapel Hill, NC, 6Children's Hospital of the King's Daughters/EVMS, Norfolk, VA, 7PRINTO, Istituto Giannina Gaslini, Genova, Italy, 8Hospital for Special Surgery, New York City, NY, 9IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, 10Children's Hospital Colorado, Aurora, CO, 11Children's Mercy Kansas City, Kansas City, MO, 12Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 13Dr. von Hauner Children's Hospital, Munich, Germany, 14Hacettepe University, Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Ankara, Turkey, 15Stanford University School of Medicine, Palo Alto, CA, 16University of Florence, Firenze, Italy, 17Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, England, United Kingdom, 18CRMO Patient Partner, New York, NY, 19Stony Brook Children's Hospital, Stony Brook, NY, 20Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, 21CRMO Patient Partner, Fort Collins, CO, 22Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany, 23Anna Meyer Children's Hospital, Firenze, Italy, 24University of Utah and Primary Children's Hospital, Salt Lake City, UT, 25Georgetown University, DERWOOD, MD, 26Tufts Medical Center, Boston, MA, 27University of Iowa Carver College of Medicine, Iowa City, IA, 28University of Liverpool, Liverpool, United Kingdom, 29Boston Children's Hospital, Boston, MA, 30Vivantes Children’s Hospital, Wuerzburg, Germany, 31The Hospital for Sick Children, Toronto, ON, Canada, 32Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Ankara, Turkey

    Background/Purpose: Chronic nonbacterial osteomyelitis (CNO)/chronic recurrent multifocal osteomyelitis (CRMO) predominantly affects children and young adults. Classification criteria are not available and diagnostic criteria that have…
  • Abstract Number: 1165 • ACR Convergence 2020

    Validation of Healthcare Claims Algorithms for Identification of Herpes Zoster Among Children with Autoimmune/Autoinflammatory Disease

    Beth Rutstein1, Sabrina Gmuca2, Jeffrey S. Gerber2 and Alexis Ogdie3, 1The Children's Hospital of Philadelphia, Philadelphia, PA, 2Children's Hospital of Philadelphia, Philadelphia, PA, 3University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Herpes zoster (HZ) is a known serious infectious complication in children with autoimmune/autoinflammatory disease and potentially exacerbated by immunomodulatory medications. Information on HZ burden…
  • Abstract Number: 1166 • ACR Convergence 2020

    Use of Rituximab to Treat COPA Syndrome: A Multi-Institutional Cohort

    Leigh Stubbs1, Isabella Osuna2, Tarin Bigley3, Michal Cidon4, Kristen Mengwasser5, Simon Helfgott6, Marietta DeGuzman7, Manuel Silva-Carmona8 and Tiphanie Vogel9, 1Baylor College of Medicine, Houston, TX, 2Department of Pediatrics, Division of Rheumatology, Baylor College of Medicine and Texas Children’s Hospital, Houston, Texas, USA, Houston, TX, 3Washington University in St. Louis, Saint Louis, MO, 4CHLA, Los Angeles, CA, 5Department of Medicine, Division of Rheumatology, Immunology, and Allergy, Harvard Medical School and Brigham and Women’s Hospital, Boston, Massachusetts, USA, Boston, MA, 6BWH- HMS, Boston, MA, 7Baylor College of Medicine, Houston, 8Department of Pediatrics, Division of Pulmonology Medicine, Baylor College of Medicine and Texas Children’s Hospital, Houston, Texas, USA, Houston, TX, 9Baylor College of Medicine/Texas Children's Hospital, Houston, TX

    Background/Purpose: Coatomer protein complex subunit α (COPA) syndrome is a rare genetic multisystem autoimmune disorder inherited in an autosomal dominant manner. Onset typically occurs in…
  • Abstract Number: 1161 • ACR Convergence 2020

    Perspectives of Radiologist Physicians in the Imaging of Chronic Nonbacterial Osteomyelitis

    Farzana Nuruzzaman1, Mingqian Huang2, Christian Hedrich3, Hermann Girschick4, Julie Cherian5, T. Shawn Sato6, Karen Onel7, Polly Ferguson8 and Yongdong Zhao9, 1Stony Brook Children's Hospital, Stony Brook, NY, 2Mount Sinai Hospital, 10003, NY, 3University of Liverpool, Liverpool, United Kingdom, 4Vivantes Children’s Hospital, Wuerzburg, Germany, 5Stony Brook Children�s Hospital, Stony Brook, NY, 6University of Iowa, Iowa City, 7Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, 8University of Iowa Carver College of Medicine, Iowa City, IA, 9University of Washington, Seattle, WA

    Background/Purpose: Radiological imaging is integral to the diagnosis of chronic nonbacterial osteomyelitis (CNO) and has been included as a central component in suggested diagnostic criteria…
  • Abstract Number: 1172 • ACR Convergence 2020

    Cardiovascular involvement as a clue for diagnosis of Juvenile Systemic Sclerosis sine scleroderma

    Gloria Lanzoni1, Giorgia Martini1, Alessandra Meneghel1, Fabio Vittadello2, Biagio Castaldi1, Elisabetta Zanatta3 and Francesco Zulian1, 1Department of Woman's and Child's Health, University of Padova, Padua, Italy, 2Centro Studi Statistici Explora, Padua, Italy, 3Department of Medicine-DIMED, University of Padova, Padua, Italy

    Background/Purpose: Juvenile Systemic Sclerosis (JSSc) is a rare condition in childhood and its variety with no skin involvement, systemic sclerosis sine scleroderma (JSSSS) is anecdotal…
  • Abstract Number: 1169 • ACR Convergence 2020

    Three-Dimensional Analysis of Facial Asymmetry in Craniofacial Scleroderma

    Daniel Glaser1, Christopher Liu2 and Kathryn Torok3, 1UPMC Children's Hospital of Pittsburgh, Piitsburgh, PA, 2Children's Hospital of Pittsburgh, Pittsburgh, PA, 3University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Localized scleroderma (LS) is a rare, progressive autoimmune disease of the skin and underlying connective tissue that can result in devastating functional impairment and cosmetic damage in children. …
  • Abstract Number: 1164 • ACR Convergence 2020

    Frequency of Genetic Diagnosis in an Autoinflammatory Disease Natural History Protocol Cohort of Patients

    Katelin R. Honer1, Kim Johnson1, Gema Souto Adeva1, Gina Montealegre Sanchez2, Jenna Wade3, Jacob Mitchell1, Katherine Townsend3, Adriana de Jesus4 and Raphaela Goldbach-Mansky5, 1Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, 2Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Rockville, MD, 3Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, 4Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, 5Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD

    Background/Purpose: Monogenic autoinflammatory diseases (AID) are caused by innate immune dysregulation resulting in systemic inflammation and variable organ-specific clinical manifestations. The Translational Autoinflammatory Disease Section…
  • Abstract Number: 1056 • ACR Convergence 2020

    Descriptive Data Analysis of Patients with Anti Jo1 Syndrome (AJS) and Lung Involvement

    Mohamed Alalwani1, Bassam Alhaddad2, Basem Zraik3, Christopher O'Rourke3, Ruchi Yadav3, Ali Askari1, Charles Malemud1 and Soumya Chatterjee4, 1Case Western Reserve University / University Hospitals Cleveland Medical Center, Cleveland, OH, 2MetroHealth Medical Center, Westlake, OH, 3Cleveland Clinic, Cleveland, OH, 4Cleveland Clinic, Richmond Heights, OH

    Background/Purpose: To evaluate the patterns, severity and, prognostic parameters of interstitial lung disease (ILD) in Anti Jo1 Syndrome (AJS).Methods: We identified 51 anti-Jo-1 patients with…
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