Abstracts tagged "Systemic JIA"

Abstract Number: 007 • 2020 Pediatric Rheumatology Symposium
Dense Genotyping of Immunologic Loci Identifies CXCR4 as a Novel Susceptibility Locus for Systemic Juvenile Idiopathic Arthritis
Emily Shuldiner ¹, Elaine Remmers ², Miranda Marion ³, Marc Sudman ⁴, Colleen Satorius ⁵, International Childhood Arthritis Genetics Consortium (INCHARGE), Juvenile Arthritis Consortium for the Immunochip (JACI), Wendy Thomson ⁶, Michael Ombrello¹, Patricia Woo ⁷, Carl Langefeld ⁸, Sampath Prahalad ⁹ and Susan Thompson ¹⁰, ¹NIAMS, NIH, Bethesda, ²National Human Genome Research Institute, Bethesda, ³Wake Forest University, Winston-Salem, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, ⁵NHGRI, NIH, Bethesda, ⁶Manchester Academic Health Science Centre, Manchester, United Kingdom, ⁷London, United Kingdom, ⁸Winston Salem, ⁹Emory + Children's Pediatric Institute, Atlanta, ¹⁰Cincinnati Children's Hospital Medical Center/Univ of Cincinnati College of Medicine, Cincinnati
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a severe, potentially lethal inflammatory condition. It accounts for a disproportionate share of morbidity and mortality among childhood…
Abstract Number: 009 • 2020 Pediatric Rheumatology Symposium
Emapalumab (Anti-Interferon-Gamma Monoclonal Antibody) in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA)
Fabrizio De Benedetti¹, Paul Brogan ², Claudia Bracaglia ¹, Manuela Pardeo ¹, Giulia Marucci ¹, Emanuela Sacco ¹, Despina Eleftheriou ³, Charalampia Papadopoulou ³, Alexei Grom ⁴, Pierre Quartier ⁵, Rayfel Schneider ⁶, Philippe Jacqmin ⁷, Rikke Frederiksen ⁸, Maria Ballabio ⁹ and Cristina de Min ⁸, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Section Head Infection, Immunology, and Rheumatology, London, United Kingdom, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, ⁵Paris-Descartes University, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁶The Hospital for Sick Children, Toronto, Canada, ⁷MnS Modelling and Simulation, Dinant, Belgium, ⁸Swedish Orphan Biovitrum AG (Sobi), Basel, Basel-Stadt, Switzerland, ⁹Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a severe complication of rheumatic diseases and occurs most frequently in patients with sJIA. Data from animal models and from observational studies…
Abstract Number: 072 • 2020 Pediatric Rheumatology Symposium
Allogenic Hematopoietic Stem Cell Transplantation for Refractory Childhood Rheumatic Diseases
Camille Beaufils¹, Catherine Proulx ², Annaliesse Blincoe ³, Pierre Teira ⁴, Henrique Bittencourt ⁴, Sonia Cellot ⁵, Michel Duval ⁴, Johannes Roth ⁶, Marie-Paule Morin ⁷, Jean Jacques De Bruycker ⁷, Julie Couture ⁷, Kathryn Samaan ⁸, Helene Decaluwe ⁷, Fabien Touzot ⁷, Elie Haddad ⁷ and Julie Barsalou ⁷, ¹Division of Pediatric Rheumatology-Immunology, CHU Sainte-Justine, Montreal, Quebec, Canada, ²Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada, ³Division of Pediatric Rheumatology-Immunology, CHU Sainte-Justine, Montreal, Canada, ⁴Division of Pediatric Hematology-Oncology, CHU Sainte-Justine, Department of Pediatrics, University of Montreal, Montreal, Quebec, Canada, ⁵Division of Pediatric Hematology-Oncology, CHU Sainte-Justine, Department of Pediatrics, University of Montreal, Montreal, Canada, ⁶Division of Pediatric Rheumatology, Children Hospital of Eastern Ontario, Department of Pediatrics, University of Ottawa, Ottawa, Ontario, Canada, ⁷Division of Pediatric Rheumatology-Immunology, CHU Sainte-Justine, Department of Pediatrics, University of Montreal, Montreal, Quebec, Canada, ⁸Division of Pediatric Rheumatology-Immunology,-Allergy, CHU Sainte-Justine, Department of Pediatrics, University of Montreal, Montreal, Quebec, Canada
Background/Purpose: Patients with refractory rheumatic diseases face poor quality of life, long-term sequelae and life-threatening complications. With advances in allogenic hematopoietic stem cell transplantation (allo-HSCT),…
Abstract Number: 076 • 2020 Pediatric Rheumatology Symposium
How Do Pediatric Rheumatologists Diagnose Macrophage Activation Syndrome in Systemic Onset Juvenile Idiopathic Arthritis? An Examination of the CARRA Registry
Taha Moussa¹, Moussa Abdelhak ² and Cuoghi Edens ³ for the CARRA investigators, ¹University of Chicago Medicine, Chicago, ²Cambridge GPVTS, Cambridge, United Kingdom, ³University of Chicago, Chicago
Background/Purpose: Macrophage activation syndrome (MAS) is a feared complication in~ 10% of those systemic onset juvenile idiopathic arthritis (sJIA). Due to the similarity of MAS…
Abstract Number: 079 • 2020 Pediatric Rheumatology Symposium
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Arianna De Matteis ¹, Denise Pires Marafon ², Ivan Caiello ², Manuela Pardeo ², Giulia Marucci ², Emanuela Sacco ², Giusi Prencipe ², Fabrizio De Benedetti ² and Claudia Bracaglia², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Lazio, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) and Secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm. Prompt recognition and early treatment are essential…
Abstract Number: 081 • 2020 Pediatric Rheumatology Symposium
Risk Score of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Simone Carbogno ¹, Denise Pires Marafon ², Giulia Marucci ², Manuela Pardeo ², Antonella Insalaco ², Virginia Messia ², Emanuela Sacco ², Ferhat Demir ³, Betul Sozeri ³, Natasia Cekada ⁴, Marija Jelusic ⁴, Olga Vougiouka ⁵, Mikhail Kostik ⁶, Alenka Gagro ⁷, Christoph Kessel ⁸, Francesca Minoia ⁹, Fabrizio De Benedetti ² and Claudia Bracaglia², ¹Pediatric Area, University of Milan, Milan, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ³University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology, Istanbul, Turkey, ⁴Division of Paediatric Rheumatology and Immunology, University of Zagreb, Zagreb, Croatia, ⁵Second Department of Paediatrics, P. & A. Kyriakou Children’s Hospital, Athens, Greece, ⁶Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ⁷Children's Hospital Zagreb, Zagreb, Croatia, ⁸University Hospital Muenster, Muenster, Nordrhein-Westfalen, Germany, ⁹Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, 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: 093 • 2020 Pediatric Rheumatology Symposium
Treatment of Systemic Juvenile Idiopathic Arthritis in the CARRA Registry
Ginger Janow ¹, Timothy Beukelman ², Yukiko Kimura ³, Rayfel Schneider ⁴, Shalini Mohan ⁵, Gail Rodich ⁶ and Mary Beth Son⁷ for the CARRA investigators, ¹Joseph M. Sanzari Children's Hospital at Hackensack Meridian Health, Hackensack, New Jersey, ²University of Alabama at Birmingham, Birmingham, ³Hackensack Meridian School of Medicine, Hackensack, ⁴The Hospital for Sick Children, Toronto, Canada, ⁵Genentech, Inc., South San Francisco, ⁶Genentech, Mill Valley, ⁷Boston Children's Hospital, Boston, Massachusetts
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: 098 • 2020 Pediatric Rheumatology Symposium
Early Treatment with Anakinra in Systemic Juvenile Idiopathic Arthritis
Manuela Pardeo¹, Claudia Bracaglia ¹, Emanuela Sacco ¹, Denise Pires Marafon ¹, Antonella Insalaco ¹, Giulia Marucci ¹, Rebecca Nicolai ², Virginia Messia ¹ and Fabrizio De Benedetti ¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) should be considered as a polygenic autoinflammatory disease. Interleukin 1 (IL-1) has been shown to be a major mediator…
Abstract Number: 140 • 2020 Pediatric Rheumatology Symposium
Serum Biomarkers in a German Cohort of Patients with Systemic Juvenile Idiopathic Arthritis and Their Relationship to Response to Interleukin-1 Blockade
Claas Hinze¹, Tanja Hinze ², Helmut Wittkowski ¹, Christoph Kessel ¹, Sabrina Fuehner ¹ and Dirk Foell ¹, ¹University Hospital Muenster, Muenster, Nordrhein-Westfalen, Germany, ²Muenster, Nordrhein-Westfalen, Germany
Background/Purpose: Most, but not all, patients with systemic juvenile idiopathic arthritis (systemic JIA) respond to therapy with interleukin (IL)-1 blocking agents but predictive factors have…
Abstract Number: 159 • 2020 Pediatric Rheumatology Symposium
Interleukin-18 as a Key Cytokine to Understand Pathology and to Decide Appropriate Therapeutic Strategy in Chronic Arthritic Systemic Juvenile Idiopathic Arthritis
Takako Miyamae¹, Yumi Tani ¹, Manabu Kawamoto ², Takayuki Kishi ³ and Masayoshi Harigai ², ¹Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, ²Institute of Rheumatology, Tokyo Women's Medical University, TOKYO, Japan, ³Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan
Background/Purpose: Long-term outcomes of systemic JIA are highly variable. approximately half of the patients have chronic persistent arthritis requiring extended anti-inflammatory therapy, sometimes into adulthood.…
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…

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