Abstracts tagged "Systemic JIA"

Abstract Number: L06 • 2019 ACR/ARP Annual Meeting
Interferon-gamma (IFN-γ) Neutralization with Emapalumab and Time to Response in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (s-JIA) who failed High-Dose Glucocorticoids
Fabrizio De Benedetti¹, Paul Brogan ², Alexei A. Grom ³, Pierre Quartier ⁴, Rayfel Schneider ⁵, Jordi Antón ⁶, Claudia Bracaglia ⁷, Manuela Pardeo ⁸, Giulia Marucci ⁸, Emanuela Sacco ⁹, Despina Eleftheriou ¹⁰, Charalampia Papadopoulou ¹¹, Philippe Jacqmin ¹², maria ballabio ¹³ and Cristina de Min ¹³, ¹Bambino Gesù Children's Hospital, Rome, Italy, ²Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Necker Hospital, Paris, France, ⁵Hospital for Sick Children, Toronto, ON, Canada, ⁶Hospital Sant Joan de Déu, Barcelona, Spain, ⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁸Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, ¹⁰UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section,, london, United Kingdom, ¹¹UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section, london, United Kingdom, ¹²MnS, Dinant, Belgium, ¹³Sobi AG, basel, Switzerland
Background/Purpose: MAS is a severe complication of rheumatic diseases, most frequently sJIA and adult-onset Still’s disease. It is characterized by fever, hepatosplenomegaly, liver dysfunction, cytopenias,…
Abstract Number: 780 • 2019 ACR/ARP Annual Meeting
Changes in MiR-17-92 Cluster Expression Link Systemic Juvenile Idiopathic Arthritis, Monocyte-to-Macrophage Differentiation, and Interferon Regulation
Divya Takellapti¹, Xiaoling Niu ², Thuy Do ³ and Grant Schulert ³, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Fudan University, Shanghai, Shanghai, China (People's Republic), ³Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: MicroRNAs (miRNAs) are small noncoding RNAs which post-transcriptionally regulate gene expression. The miR-17-92 cluster is well characterized; its overexpression has been found to serve…
Abstract Number: 784 • 2019 ACR/ARP Annual Meeting
Application of Systems Biology-Based In Silico Tools for Optimal Treatment Strategy Identification in Still’s Disease
Cristina Segu-Verges¹, Mireia Coma ¹, Christoph Kessel ², Serge Smeets ³, Dirk Foell ² and Anna Aldea ⁴, ¹Anaxomics, Barcelona, Catalonia, Spain, ²University Hospital Muenster, Muenster, Germany, ³Novartis, Amsterdam, Netherlands, ⁴Novartis, Barcelona, Catalonia, Spain
Background/Purpose: Systemic JIA (sJIA) and Adult Onset Still’s Disease may represent a disease continuum1 of the same autoinflammatory disorder, Still’s Disease. Current challenges in its…
Abstract Number: 813 • 2019 ACR/ARP Annual Meeting
Monocyte and Macrophage Transcriptional Phenotypes in Systemic Juvenile Idiopathic Arthritis Reveal TRIM8 as a Mediator of IFNγ Hyperresponsiveness and Risk for Macrophage Activation Syndrome
Grant Schulert¹, Thuy Do ¹, Sanjeev Dhakal ¹, Ndate Fall ², Mario Medvedovich ³, Sherry Thornton ¹, Nathan salomonis ² and Alexei A. Grom ¹, ¹Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³University of Cincinnati College of Medicine, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a severe and distinct subtype of childhood arthritis. Children with SJIA are at risk for macrophage activation syndrome…
Abstract Number: 915 • 2019 ACR/ARP Annual Meeting
Development and Initial Validation of the Systemic JADAS, a New Composite Disease Activity Score for Systemic Juvenile Idiopathic Arthritis
Jessica Tibaldi¹, Yasser El Miedany ², Pryiankar Pal ³, Soamarat Vilaiyuk ⁴, Raju Khubchandani ⁵, Manuela Pardeo ⁶, Tapas Kumar Sabui ⁷, Sujata Sawhney ⁸, Ricardo Russo ⁹, Flavio Sztajnbok ¹⁰, Rolando Cimaz ¹¹, Francesca Minoia ¹², Motasem O. Alsuweiti ¹³, Ekaterina Alexeeva ¹⁴, Mikhail Kostik ¹⁵, Maria Cristina Maggio ¹⁶, Sulayman Al Mayouf ¹⁷, Claudia Saad ¹⁸, Giovanni Conti ¹⁹, Romina Gallizzi ²⁰, Adele Civino ²¹, Masaki Shimizu ²², Enrico Felici ²³, Angela Pistorio ²⁴, Nicolino Ruperto ²⁵, Alessandro Consolaro ²⁶ and Angelo Ravelli ²⁷, ¹IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genoa, Italy, ²Ain Shams University, Cairo, Egypt, ³Institute of Child Health, Kolkata, India, ⁴Ramathibodi Hospital, Mahidol University, Bangkok, Thailand, ⁵Jaslok Hospital and Research Center, Mumbai, India, ⁶Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁷R G Kar Medical College, Kolkata, Kolkata, India, ⁸Department of pediatric rheumatology, Sir Ganga Ram Hospital, New Delhi, India, New Delhi, India, ⁹Hospital de Pediatría Garrahan, Buenos Aires, Argentina, ¹⁰Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ¹¹University Hospital Meyer, Florence, Italy, ¹²Clinica De Marchi Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, ¹³Queen Rania Children's Hospital, Amman, Jordan, ¹⁴National Medical Research Center of Children`s Health, Moscow, Russia, ¹⁵Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁶Università di Palermo, Dipartimento G. D'Alessandro, Palermo, Italy, ¹⁷King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia, ¹⁸Hospital das Clínicas - Botucatu Medicine University, UNESP, Botucatu, Brazil, ¹⁹AOU G Martino, Messina, Italy, ²⁰Azienda Ospedaliera Universitaria Gaetano Martino, Messina, Messina, Italy, ²¹Pediatric Rheumatology “Vito Fazzi” Hospital, Lecce, Lecce, Italy, ²²Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa, Japan, ²³SS Antonio e Biagio e Cesare Arrigo" Hospital, Alessandria, Italy, ²⁴IRCCS Istituto Giannina Gaslini, Genova, Italy, ²⁵Paediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy, ²⁶Università degli Studi di Genova, Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-Infantili (DiNOGMI), Genoa, Italy and IRCCS Istituto Giannina Gaslini, Clinica Pediatrica e Reumatologia, Genoa, Italy, Genova, Italy, ²⁷IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy
Background/Purpose: The Juvenile Arthritis Disease Activity Score (JADAS) has gained increasing popularity for the measurement of the level of disease activity in patients with juvenile…
Abstract Number: 917 • 2019 ACR/ARP Annual Meeting
Systemic Juvenile Idiopathic Arthritis-Lung Disease: Characterization and Risk Factors
Grant Schulert¹, Shima Yasin ², Brenna Carey ³, Claudia Chalk ³, Thuy Do ¹, Andrew Schapiro ³, Ammar Husami ³, Allen Watts ³, Hermine Brunner ⁴, Jennifer Huggins ³, Elizabeth Mellins ⁵, Esi Morgan ³, Tracy Ting ³, Bruce Trapnell ⁶, Katheryn Wikenheiser-Brokamp ³, Christopher towe ³ and Alexei A. Grom ¹, ¹Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Pediatric Rheumatology Collaborative Study Group (PRCSG), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Stanford University, Palo Alto, ⁶Cincinnati Children's Hospital, Cincinnati
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) is associated with a recently recognized albeit poorly defined and characterized lung disease (LD). Our objective is to describe…
Abstract Number: 918 • 2019 ACR/ARP Annual Meeting
Multiplex Serum Analysis Identifies Potential Biomarkers of Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome, and Associated Pulmonary Alveolar Proteinosis: Evidence for Independently-regulated Hyperinflammatory and Eosinophilic Inflammation
Guangbo Chen ¹, Grant Schulert ², Adriana De Jesus ³, Vivian Saper ⁴, Corinne Schneider ⁵, Bruce Trapnell ⁶, Alexei A. Grom ², Raphaela Goldbach-Mansky ⁷, Elizabeth Mellins ⁴, Purvesh Khatri ⁸ and Scott Canna⁹, ¹Stanford University, Palo Alto, CA, ²Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³NIH/NIAID/TADS, Bethesda, MD, ⁴Stanford University, Palo Alto, ⁵University of Pittsburgh, Pittsburgh, ⁶Cincinnati Children's Hospital, Cincinnati, ⁷Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD, ⁸Institute for Immunity, Transplantation and Infection, Stanford University, Palo Alto, ⁹UPMC Children's Hospital of Pittsburgh, Pittsburgh
Background/Purpose: Recent experiences suggest a disturbing epidemic of digital clubbing and insidious, Pulmonary Alveolar Proteinosis (PAP)-like lung disease occurring in patients with Systemic Juvenile Idiopathic…
Abstract Number: 920 • 2019 ACR/ARP Annual Meeting
Adenosine Deaminase 2 as a Circulating Biomarker of Macrophage Activation Syndrome
Pui Lee¹, Grant Schulert ², Scott Canna ³, Yuelong Huang ⁴, Jacob Sundel ⁴, Mary Beth Son ¹, Lauren Henderson ¹, Peter Nigrovic ⁵, Jane Newburger ⁶, Fatma Dedeoglu ⁶, Mindy Lo ⁶, Esra Meidan ⁶, Ying Li ⁴, Olha Halyabar ⁶, Margaret Chang ⁷, Kacie Hoyt ⁶, Thuy Do ², Robert Sundel ⁸ and Rachel Blaustein ⁴, ¹Boston Children's Hospital, Boston, MA, ²Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁴Brigham and Women's Hospital, Boston, ⁵Department of Medicine, Division of Rheumatology, Immunology and Allergy, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA ; Department of Medicine, Division of Immunology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, USA., Boston, ⁶Boston Children's Hospital, Boston, ⁷Division of Immunology, Boston Children's Hospital, Boston, ⁸Boston Children’s Hospital, Harvard Medical School, Boston
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (sJIA) characterized by a vicious cycle of immune cell activation and…
Abstract Number: 2694 • 2019 ACR/ARP Annual Meeting
Inpatient Treatment Variation in New-Onset Systemic Juvenile Idiopathic Arthritis
Rosemary Peterson¹, Rui Xiao ², Hannah Katcoff ¹, Brian Fisher ¹ and Pamela F. Weiss ¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²Children's Hospital of Philadelphia, Philadelphia
Background/Purpose: The effectiveness of IL-1 and IL-6 inhibitors as first-line therapy in patients with new-onset systemic JIA has led to the concept of a “window…
Abstract Number: 2698 • 2019 ACR/ARP Annual Meeting
Reasons for Initiation of Canakinumab of Patients with Systemic Juvenile Idiopathic Arthritis: A Retrospective Medical Chart Review from the United States
Peter Hur¹, Raluca Ionescu-Ittu ², Ameur M. Manceur ², Kathleen G. Lomax ¹, Jordan Cammarota ³, Jipan Xie ⁴, Navneet Sanghera ⁵ and Alexei A. Grom ⁶, ¹Novartis Pharmaceuticals Corporation, East Hanover, NJ, ²Analysis Group, Inc., Montreal, QC, Canada, ³Analysis Group Inc.,, Washington, DC, ⁴Analysis Group, Inc., Los Angeles, CA, ⁵Novartis Pharmaceuticals Corporations, East Hanover, NJ, ⁶Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a rare autoinflammatory disease characterized by fever and arthritis, often accompanied by rash. Canakinumab (CAN) was approved in…
Abstract Number: 1416 • 2018 ACR/ARHP Annual Meeting
Risk Score of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Simone Carbogno¹, Denise Pires Marafon², Giulia Marucci³, Manuela Pardeo³, Antonella Insalaco³, Virginia Messia³, Rebecca Nicolai³, Fabrizio De Benedetti⁴ and Claudia Bracaglia³, ¹University of Milan, Milan, Italy, ²Pediatric Unit, Fondazione IRCCS Ca’ Grande Ospedale Maggiore Policlinico, Milan, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁴IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of rheumatic diseases in childhood, particularly of systemic Juvenile Idiopathic Arthritis (sJIA), occurring in approximately…
Abstract Number: 1426 • 2018 ACR/ARHP Annual Meeting
The Utilization of S100 Proteins Testing in Pediatric Rheumatology Patients in a Tertiary Care Institution and Implications for Care
Najla Aljaberi¹, Angela Merritt², Alexei Grom¹, Grant Schulert³, Jennifer L. Huggins⁴, Michael Henrickson⁵ and Hermine I. Brunner¹, ¹Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²Pediatric Rheumatology, Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Rheumatology, Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁴Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Cincinnati Children’s Medical Center, Cincinnati, OH
Background/Purpose: S100 proteins are calcium-binding proteins of increasing value as biomarkers in various inflammatory conditions (e.g. autoinflammatory diseases, vasculitides, inflammatory bowel disease). The two highly…
Abstract Number: 1437 • 2018 ACR/ARHP Annual Meeting
Use,Safety and Efficacy of Thalidomide from a Tertiary Level Pediatric Rheumatology Centre in India
Manjari Agarwal¹ and Sujata Sawhney², ¹Institute of Child Health, Attending Consultant, New Delhi, India, ²Paediatric rheumatology, Senior Consultant, New Delhi, India
Background/Purpose: Thalidomide is an effective agent for several pediatric rheumatic diseases: SOJIA, Behcet's disease and recalcitrant skin disease in cSLE to name a few. Systemic…
Abstract Number: 2864 • 2018 ACR/ARHP Annual Meeting
Identification of Optimal Subcutaneous Doses of Tocilizumab in Children with Systemic Juvenile Idiopathic Arthritis
Hermine I. Brunner¹, Nicola Ruperto², Daniel J Lovell¹, Gerd Horneff³, María Luz Gámir-Gámir⁴, Markus Hufnagel⁵, Joy Hsu⁶, Min Bao⁶, Wendy Douglass⁷, Navita L. Mallalieu⁶, Chris Wells⁷, Christopher M. Mela⁸ and Fabrizio De Benedetti⁹, ¹Pediatric Rheumatology Collaborative Study Group (PRCSG), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²Pediatric Rheumatology International Trial Organization (PRINTO), Istituto Giannina Gaslini Pediatria II-Reumatologia, Genoa, Italy, ³Asklepios Clinic Sankt Augustin, and University Hospital of Cologne, Cologne, Germany, ⁴Hospital Ramon y Cajal Unidad de Reumatologia Pediatrica, Madrid, Spain, ⁵Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, ⁶Roche Innovation Center, New York, NY, ⁷Roche Products Ltd., Welwyn Garden City, United Kingdom, ⁸Roche Products Ltd, Welwyn Garden City, United Kingdom, ⁹IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Background/Purpose: Efficacy and safety of intravenous (IV) tocilizumab (TCZ) were shown in patients (pts) with systemic JIA (sJIA) in the phase 3 TENDER study.1 This…
Abstract Number: 2865 • 2018 ACR/ARHP Annual Meeting
Canakinumab, on a Reduced Dose or a Prolonged Dose Interval without Concomitant Corticosteroids and Methotrexate, Maintains Efficacy in Systemic Juvenile Idiopathic Arthritis Patients in Clinical Remission
Pierre Quartier¹, Ekaterina Alexeeva², Carine Wouters³, Inmaculada Calvo⁴, Tilmann Kallinich⁵, Bo Magnusson⁶, Nico Wulffraat⁷, Xiaoling Wei⁸, Alan Slade⁹, Ken Abrams⁹ and Alberto Martini¹⁰, ¹AP-HP, Institut des Maladies Génétiques (IMAGINE), and Université Paris-Descartes, Necker-Enfants Malades Hospital, Paris, France, ²National Medical Research Center of Children's Health and Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russian Federation, ³Gasthuisberg University Hospital, Leuven, Belgium, ⁴Hospital Universitario La Fe, Valencia, Spain, ⁵Charité Berlin Campus Virchow, Berlin, Germany, ⁶Karolinska University Hospital, Stockholm, Sweden, ⁷University Medical Center Utrecht, Utrecht, Netherlands, ⁸China Novartis Institutes for Biomedical Research Co., Ltd, Beijing, China, ⁹Novartis Pharmaceuticals Corporation, East Hanover, NJ, ¹⁰Universita di Genova Pediatria II, Genova, Italy
Background/Purpose: Treatment with canakinumab (CAN), a selective, human anti-IL-1β monoclonal antibody, has shown sustained therapeutic effect along with corticosteroid dose reduction/discontinuation in patients with systemic…

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