Abstracts tagged "Systemic JIA"

Abstract Number: 2410 • 2016 ACR/ARHP Annual Meeting
Genetic Architecture of Systemic Juvenile Idiopathic Arthritis Distinguishes It from Oligoarticular and Polyarticular Forms of Juvenile Idiopathic Arthritis
Michael J. Ombrello¹, Victoria Arthur¹, Elaine F. Remmers², Anne Hinks³, Alexei Grom⁴, Dirk Föll⁵, Alberto Martini⁶, Marco Gattorno⁷, Seza Ozen⁸, Sampath Prahalad⁹, Andrew Zeft¹⁰, John F. Bohnsack¹¹, Norman Ilowite¹², Ricardo Russo¹³, Elizabeth D. Mellins¹⁴, Claudio A. Len¹⁵, Maria Odete E. Hilário¹⁶, Sheila Oliveira¹⁷, Rae S.M. Yeung¹⁸, Alan Rosenberg¹⁹, Lucy R. Wedderburn²⁰, Jordi Anton²¹, Johannes Peter Haas²², Angela Rösen-Wolff²³, Klaus Tenbrock²⁴, Susan D Thompson²⁵, Daniel L. Kastner²⁶, Patricia Woo²⁷, Wendy Thomson²⁸ and International Childhood Arthritis Genetics (INCHARGE) Consortium, ¹Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD, ²National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ³ARC Epidemiology Unit, University of Manchester, Manchester, United Kingdom, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵University of Muenster, Muenster, Germany, ⁶Istituto Giannina Gaslini, Genoa, Italy, ⁷UO Pediatria 2, Istituto Giannina Gaslini, Genova, Italy, ⁸Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁹Pediatric Rheumatology, Emory University School of Medicine, Atlanta, GA, ¹⁰Pediatrics Rheumatology, Cleveland Clinic, Cleveland, OH, ¹¹Division of Allergy, Immunology and Pediatric Rheumatology, University of Utah, Salt Lake City, UT, ¹²Rheumatology, Children's Hospital Montefiore, Bronx, NY, ¹³Hospital de Pediatria Garrahan, Buenos Aires, Argentina, ¹⁴Dept of Pediatrics CCSR, Stanford University Med Ctr, Stanford, CA, ¹⁵Pediatrics, Universidade Federal de São Paulo, São Paulo,, Brazil, ¹⁶Pediatric Rheumatology Collaborative Study Group (PRCSG), Cincinnati, OH, ¹⁷Pediatric Rheumatology, Universidade F Rio De Janeiro, Rio De Janeiro, Brazil, ¹⁸Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ¹⁹Pediatrics, Pediatrics, Saskatoon, SK, Canada, ²⁰Institute of Child Health, UCL, London, United Kingdom, ²¹Unitat de Reumatologia Pediàtrica, Hospital Sant Joan de Déu, Barcelona, Spain, ²²German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany, ²³Children's Hospital Dresden, Dresden, Germany, ²⁴University Aachen, Aachen, Germany, ²⁵Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, ²⁶Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ²⁷Great Ormond Street Hospital, University College London Medical School, London, United Kingdom, ²⁸Arthritis Research UK Centre for Genetics and Genomics,The University of Manchester, Manchester, United Kingdom
Background/Purpose: JIA is a heterogeneous group of conditions that are unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA)…
Abstract Number: 2419 • 2016 ACR/ARHP Annual Meeting
Single-Cell Analysis of CD163 mRNA and Protein Expression By Primeflow™ in Polarized Monocyte and Macrophage Populations
Rachel Tan¹, Sherry Thornton², Alyssa Sproles², Thuy Do³, Jonathan Schick⁴, Monica DeLay⁴ and Grant Schulert⁵, ¹University of Cincinnati, Cincinnati, OH, ²Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Cincinnati Children's Hospital, Cincinnati, OH, ⁵Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: CD163 is involved in the regulation and resolution of innate inflammation and the removal of free hemoglobin from the blood via internalization of the…
Abstract Number: 3006 • 2016 ACR/ARHP Annual Meeting
IFN-Gamma (IFNγ), IFNγ-Induced Chemokines and Other Biomarkers in Macrophage Activation Syndrome (MAS)
Claudia Bracaglia¹, Denise Pires Marafon², Ivan Caiello², Kathy de Graaf³, Florence Guilhot³, Walter Ferlin³, Sergio Davì⁴, Grant Schulert⁵, Angelo Ravelli⁴, Alexei Grom⁶, Robert Nelson³, Cristina de Min³ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy, Rome, Italy, ²Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Rome, Italy, ³NovImmune S.A., Geneva, Switzerland, ⁴Istituto Giannina Gaslini, Genoa, Italy, ⁵Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁶Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Evidence in animals and humans points to a pivotal role of IFNγ in primary HLH. We have recently generated data in an animal model…
Abstract Number: 250 • 2016 ACR/ARHP Annual Meeting
Application of the 2016 European Leage Against Rheumatism (EULAR) /American College of Rheumatology (ACR)/Paediatric Rheumatology International Trials Organisation (PRINTO) Classification Criteria of Macrophage Activation Syndrome in Patients with Adult Onset Still’s Disease
Sung Soo Ahn¹, Seung Min Jung², Sang-Won Lee¹, Yong-Beom Park¹ and Jason Jungsik Song¹, ¹Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea, ²Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea, The Republic of
Background/Purpose: Macrophage activation syndrome (MAS) is acute systemic inflammation arising in the context of various autoimmune and autoinflammatory conditions, which is often life-threatening. In 2016,…
Abstract Number: 2376 • 2016 ACR/ARHP Annual Meeting
The Disease Burden of Systemic Juvenile Idiopathic Arthritis for Patients and Caregivers: An International Health Related Quality of Life Survey and Retrospective Chart Review
Susan Shenoi¹, Gerd Horneff², Michal Cidon³, Athimalaipet Ramanan⁴, Yukiko Kimura⁵, Pierre Quartier⁶, Ivan Foeldvari⁷, Andrew Zeft⁸, Kathleen G Lomax⁹, Jill Gregson¹⁰, Sarah Campbell¹¹, Jeffrey Weiss¹¹, Nina Marinsek¹¹, Dony Patel¹¹ and Nico Wulffraat¹², ¹Seattle Children's Hospital, Seattle, WA, ²Asklepios Kliniken GmbH, Hamburg, Germany, ³Stanford University, Palo Alto, CA, ⁴University Hospitals Bristol, Bristol, United Kingdom, ⁵Hackensack University Medical Center, Hackensack, NJ, ⁶Hôpital Necker, Paris, France, ⁷Hamburger Zentrum für Kinder-und Jugend Rheumatologie, Hamburg, Germany, ⁸Pediatrics Rheumatology, Cleveland Clinic, Cleveland, OH, ⁹Novartis Pharmaceuticals Corporation, East Hanover, NJ, ¹⁰Novartis Pharma AG, Basel, Switzerland, ¹¹Navigant Consulting, Inc., London, United Kingdom, ¹²Wilhelmina Kinderziekenhuis, Utrecht, Netherlands
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a severe autoinflammatory disease characterized by systemic features including high fevers, rash, and arthritis. SJIA can impose a…
Abstract Number: 960 • 2015 ACR/ARHP Annual Meeting
Efficacy of Canakinumab in Systemic Juvenile Idiopathic Arthritis Patients Previously Exposed to Biologics
Hermine I. Brunner¹, Nicolino Ruperto², Pierre Quartier³, Tamas Constantin², Yackov Berkun², Inmaculada Calvo-Penedes², Müferet Erguven², Laurence Goffin², Michael Hofer², Tilmann Kallinich², Sheila Oliveira², Yosef Uziel², Stefania Viola⁴, Kiran Nistala², Carine Wouters², Karolynn Leon⁵, Antonio Speziale⁶, Karine Lheritier⁶, Guido Junge⁶, Daniel Lovell^1,7 and Alberto Martini², ¹PRCSG, Cincinnati, OH, ²PRINTO-Istituto Gaslini, Genova, Italy, ³Necker-Enfant Malades Hospital, Paris, France, ⁴Istituto G Gaslini, Pediatria II, Reumatologia, Genova, Italy, ⁵Novartis Pharmaceuticals Corporation, East Hanover, NJ, ⁶Novartis Pharma AG, Basel, Switzerland, ⁷Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Efficacy and safety of canakinumab (CAN) in systemic juvenile idiopathic arthritis (SJIA) have been demonstrated in two phase III trials.1 In these trials, over…
Abstract Number: 1026 • 2015 ACR/ARHP Annual Meeting
Microrna-125a-5p Has Increased Expression in Active Systemic Juvenile Idiopathic Arthritis and Is an Essential Modulator of Regulatory Macrophage Phenotypes in Vitro
Grant Schulert¹, Ndate Fall², Nan Shen³, Sherry Thornton² and Alexei A. Grom⁴, ¹Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, ³Center for Autoimmune Genomics and Etiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is an autoinflammatory disease of childhood, characterized by a predominance of mononuclear phagocytic effector cells, compared to the lymphocyte…
Abstract Number: 1246 • 2015 ACR/ARHP Annual Meeting
Gene Expression Analysis of Adult Onset Still’s Disease and Systemic Juvenile Idiopathic Arthritis Suggest a Single Disease Continuum
N. Nirmala¹, Arndt Brachat², Eugen Feist³, Norbert Blank⁴, Christof Specker⁵, Matthias Witt⁶, Jan Zernicke⁷, Alberto Martini⁸ and Guido Junge⁹, ¹Novartis Institutes for Biomedical Research, Cambridge, MI, ²Novartis Institutes for Biomedical Research, Basel, Switzerland, ³Rheumatology and Clinical Immunology, Charite University Hospital, Berlin, Germany, ⁴University of Heidelberg, Heidelberg, Germany, ⁵Kliniken Essen Süd, Essen, Germany, ⁶Division for Rheumatology, University of Munich, Munich, Germany, ⁷Department of Rheumatology and Clinical Immunology, Charité University Medicine, Berlin, Germany, ⁸Department of Pediatrics, G Gaslini Institute, Genova, Italy, ⁹Novartis Pharma AG, Basel, Switzerland
Background/Purpose: Adult-onset Still's disease (AOSD) is a rare auto-inflammatory disorder.1 The superimposable systemic clinical features of AOSD and the pediatric syndrome known as systemic juvenile…
Abstract Number: 1402 • 2015 ACR/ARHP Annual Meeting
Development of Systemic Juvenile Idiopathic Arthritis Manifestations Following Remission of Hemophagocytic Lymphohistiocytosis
Baruch Goldberg¹, Eyal Muscal², Marietta De Guzman³ and Carl Allen⁴, ¹Pediatric Rheumatology, Texas Children's Hospital, Houston, TX, ²Pediatric Rheumatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ³Pediatric Immunology, Allergy, and Rheumatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ⁴Pediatric Hematology and Oncology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal pathologic inflammatory process resulting from impaired immune function due to inherited gene mutations or secondary to…
Abstract Number: 1461 • 2015 ACR/ARHP Annual Meeting
Inflammatory Bowel Disease in Children with Systemic Juvenile Idiopathic Arthritis
Emily Fox¹, Joyce Hsu², Elizabeth Chalom^3,4, Sarah Sertial⁵, KT Park⁶, Julia F Simard⁷, Pierre Quartier⁸, Maria Teresa Terreri⁹, Kevin Baszis¹⁰, Charlotte Borocco¹¹, Sampath Prahalad¹², Adam Reinhardt¹³, Dieneke Schonenberg¹⁴, Elizabeth D. Mellins¹⁵ and Devy Zisman¹⁶, ¹Pediatric Rheumatology, Stanford, Palo Alto, CA, ²Pediatric Rheumatology, Stanford University, Palo Alto, CA, ³Pediatrics, St Barnabas Med Center-ACC, Livingston, NJ, ⁴Pediatric Rheumatology, St. Barnabas Medical Center, West Orange, NJ, ⁵Children's Hospital of New Jersey, Livingston, NJ, ⁶Division of Gastroenterology, Stanford University School of Medicine, Stanford, CA, ⁷Division of Epidemiology, Health Research and Policy Department, and Division of Immunology & Rheumatology, Department of Medicine, Stanford School of Medicine, Stanford, CA, ⁸Hôpital Necker-Enfants Malades, Paris, France, ⁹Pediatrics, Universidade Federal de Sao Paulo, São Paulo, Brazil, ¹⁰Pediatrics, Division of Rheumatology, Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO, ¹¹Necker Hospital for Sick Children, Paris, France, ¹²Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, ¹³Faculty of Physicians of the University of Nebraska Medical Center, College of Medicine, Nebraska, NE, ¹⁴Department of Pediatric Rheumatology and Immunology, Emma Children's Hospital, Academic Medical Center, Amsterdam, Netherlands, ¹⁵Dept of Pediatrics CCSR, Stanford University Med Ctr, Stanford, CA, ¹⁶Rheumatology Unit, Carmel Medical CenterThe Ruth and Bruce Rappaport Faculty of Medicine, Technion, Israel, Haifa, Israel
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease accompanied by systemic symptoms including lymphadenopathy, serositis, and hepatosplenomegaly. Recently, we encountered a patient previously…
Abstract Number: 2424 • 2015 ACR/ARHP Annual Meeting
Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis Patients Treated with Canakinumab: Results from Phase 3 Trial Program
Alexei A. Grom¹, Hermine I. Brunner¹, Nicolino Ruperto², Alberto Martini², Daniel Lovell^1,3, Virginia Pascual⁴, Karine Lheritier⁵, Karolynn Leon⁶, Ken Abrams⁶ and Norman Ilowite⁷, ¹PRCSG, Cincinnati, OH, ²PRINTO-Istituto Gaslini, Genova, Italy, ³Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Baylor Institute for Immunology Research, Dallas, TX, ⁵Novartis Pharma AG, Basel, Switzerland, ⁶Novartis Pharmaceuticals Corporation, East Hanover, NJ, ⁷Pediatrics, Albert Einstein College of Medicine, Bronx, NY
Background/Purpose: Macrophage activation syndrome (MAS), a potentially fatal complication of systemic juvenile idiopathic arthritis (SJIA), was reported as an adverse event in both canakinumab and…
Abstract Number: 2437 • 2015 ACR/ARHP Annual Meeting
Experience with Tocilizumab for Treatment of 56 Children with Systemic Juvenile Idiopathic Arthritis in the German JIA Biologics Register
Gerd Horneff¹, Gerd Ganser², Johannes Peter Haas³, Toni Hospach⁴, Ralf Trauzeddel⁵, Hans-Iko Huppertz⁶, J B. Kuemmerle-Deschner⁷, Kirsten Minden⁸ and BIKER Registry Study Group, ¹Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ²Pediatric Rheumatology, Sankt Josef Stift, Sendenhorst, Germany, ³German Centre for Rheumatology in Children and Young People, Garmisch-Partenkirchen, Germany, ⁴Pediatrics, Olgahospital, Klinikum Stuttgart, Stuttgard, Germany, ⁵HELIOS Klinikum Berlin-Buch Klinik fuer Kinder- und Jugendmedizin, Berlin, Germany, ⁶PRINTO, IRCCS G. Gaslini, Genoa, Italy, ⁷Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ⁸DRFG, Berlin, Germany
Background/Purpose: Tocilizumab (TOC) has become a valuable option for treatment of systemic juvenile idiopathic arthritis (sJIA), which significantly improved the outcome of patients. The aim…
Abstract Number: 2442 • 2015 ACR/ARHP Annual Meeting
The Family Journey to Diagnosis with Systemic Juvenile Idiopathic Arthritis As Evidenced through Changing Social Media Presence
Renee F Modica¹, Kathleen G Lomax², Pamela Batzel³, Leah Shapardanis³, Kimberly A Compton³ and Melissa E Elder⁴, ¹UF Health Shands Children’s Hospital, Gainesville, FL, ²Medical Affairs, Immunology and Dermatology, Novartis Pharmaceuticals Corporation, East Hanover, NJ, ³TREATO, Princeton, NJ, ⁴Pediatrics, Univ of Florida, Gainesville, FL
Background/Purpose: Patients with systemic juvenile idiopathic arthritis (SJIA) frequently encounter delays from symptom onset to SJIA diagnosis (dx), partly due to the broad differential of…
Abstract Number: 3096 • 2015 ACR/ARHP Annual Meeting
Interferon-Gamma (IFNg) in Macrophage Activation Syndrome (MAS): CXCL9 Levels As a Biomarker for IFNg Production in MAS
Claudia Bracaglia¹, Denise Pires Marafon², Ivan Caiello¹, Kathy De Graaf³, Florence Guilhot⁴, Walter Ferlin⁴, Sergio Davì⁵, Grant Schulert⁶, Angelo Ravelli⁵, Alexei A. Grom⁷, Robert Nelson⁴, Cristina de Min⁴ and Fabrizio De Benedetti¹, ¹Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, ²Pediatric Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ³Novimmune S.A., Geneva, Switzerland, ⁴NovImmune S.A., Geneva, Switzerland, ⁵Istituto Giannina Gaslini and University of Genova, Genova, Italy, ⁶Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁷Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: A vast body of evidence in animals and humans points to a pivotal pathogenic role of IFNγ, in primary HLH. The role of IFNg…
Abstract Number: 1901 • 2014 ACR/ARHP Annual Meeting
Interferon-γ (IFNγ) in Macrophage Activation Syndrome (MAS) Associated with Systemic Juvenile Idiopathic Arthritis (sJIA). High Levels in Patients and a Role in a Murine MAS Model
Claudia Bracaglia¹, Ivan Caiello¹, Kathy De Graaf², Giovanni D'Ario², Florence Guilhot², Walter Ferlin², Lidia Meli¹, Giusi Prencipe¹, Sergio Davì³, Grant Schulert⁴, Angelo Ravelli⁵, Alexei Grom⁶, Cristina De Min² and Fabrizio De Benedetti Sr.¹, ¹Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, ²Novimmmune S.A., Plan-Les-Ouates, Geneva, Switzerland, ³Pediatria II, Istituto Giannina Gaslini, Genova, Italy, ⁴Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵Istituto Giannina Gaslini and University of Genova, Genova, Italy, ⁶Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: IFNγ is the pivotal mediator in murine models of primary HLH. Given the similarities between primary and secondary (sHLH), including MAS, we analyzed IFNγ…

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