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  • 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: 1150 • ACR Convergence 2020

    Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis

    Arianna De Matteis1, Denise Pires Marafon1, Ivan Caiello1, Manuela Pardeo1, Giulia Marucci1, Emanuela Sacco1, Giusi Prencipe1, Fabrizio De Benedetti2 and Claudia Bracaglia1, 1Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 2Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy

    Background/Purpose: Macrophage Activation Syndrome (MAS) and secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm, in which IFNγ plays a pivotal role.…
  • 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: 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: 1153 • ACR Convergence 2020

    Trends in Timing of Biologic Use for Treatment of Systemic Juvenile Idiopathic Arthritis in the CARRA Registry

    Ginger Janow1, Timothy Beukelman2, Yukiko Kimura3, Rayfel Schneider4, Shalini Mohan5, Gail Rodich6 and Mary Beth Son7, 1Joseph M. Sanzari Children's Hospital at Hackensack Meridian Health, Hackensack, NJ, 2University of Alabama at Birmingham, Birmingham, AL, 3Hackensack Meridian School of Medicine, Hackensack, NJ, 4University of Toronto, The Hospital for Sick Children, Toronto, Canada, 5Genentech, Inc., South San Francisco, CA, 6Genentech, Mill Valley, CA, 7Boston Children's Hospital, Boston, MA

    Background/Purpose: The treatment of systemic juvenile idiopathic arthritis (SJIA) has changed dramatically over the past decade, associated with overall improvement in functional outcomes.  There may…
  • 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: 1155 • ACR Convergence 2020

    Long-term Safety Profile of Anakinra in Patients with Systemic Juvenile Idiopathic Arthritis

    Gabriella Giancane1, Riccardo Papa1, Sebastiaan Vastert2, Francesca Bagnasco1, Joost Swart1, Pierre Quartier3, Jordi Anton4, Isabelle Kone Paut1, Sylvia Kamphuis1, Troels Herlin5, Helga Sanner1, Fabrizio De Benedetti6, Elena Tsitsami7, Susan Mary Nielsen1, Estefania Moreno1, Chiara Pallotti1, Karin Franck-Larsson8, Håkan Malmström8, Susanna Cederholm9, Nico Wulffraat1 and Nicolino Ruperto10, 1IRCCS Istituto Giannina Gaslini, PRINTO, Genoa, Italy, Genova, Italy, 2University Medical Center Utrecht, Utrecht, Netherlands, 3Necker-Enfants Malades University Hospital, Assistance Publique-Hopitaux de Paris, Paris, France, 4Sant Joan de Déu Hospital, Madrid, Spain, 5Aarhus University Hospital, Aarhus, Denmark, 6Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 7Aghia Sophia Childrens Hospital, Athens, Greece, 8Swedish Orphan Biovitrum, Stockholm, Sweden, 9Sobi, Stockholm, Sweden, 10PRINTO, Istituto Giannina Gaslini, Genova, Italy

    Background/Purpose: To evaluate the long-term safety profile of anakinra in patients with systemic juvenile idiopathic arthritis (sJIA)Methods: Data from patients with sJIA according to the…
  • 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: 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: 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: 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: 1160 • ACR Convergence 2020

    Treatment Intensity and Impact on Bone Lesion Evolution and Distribution Patterns in Severe Chronic Recurrent Multifocal Osteomyelitis

    Aleksander Lenert1, T. Shawn Sato2, Sedat G Kandemirli1, Patrick Ten Eyck1 and Polly Ferguson3, 1University of Iowa, Iowa City, IA, 2University of Iowa, Iowa City, 3University of Iowa Carver College of Medicine, Iowa City, IA

    Background/Purpose: To compare bone lesion evolution and bone lesion distribution patterns identified by whole body magnetic resonance imaging (WB-MRI) by treatment intensity in patients with…
  • 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: 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: 1163 • ACR Convergence 2020

    Predictors of Colchicine Response in Patients with Undefined Systemic Autoinflammatory Diseases

    Mariana Correia Marques1, Edwin Anderson1, Kathryn Williams2, Jonathan Hausmann3 and Fatma Dedeoglu4, 1Boston Children`s Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, 2Biostatistics and Research Design Center ICCTR Boston Children`s Hospital, Boston, MA, 3Boston Children’s Hospital, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, 4Boston Children's Hospital, Boston, MA

    Background/Purpose: Systemic autoinflammatory diseases (SAIDs) result from dysregulation of the innate immune system. Many patients with SAIDs have specific mutations that lead to the release…
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All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

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