Abstracts tagged "macrophage activation syndrome"

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: 019 • 2020 Pediatric Rheumatology Symposium
Characterization of DOCK8 as a Novel Gene Associated with Cytokine Storm Syndrome
Mingce Zhang ¹, Remy Cron ¹, Devin Absher ², Prescott Atkinson ¹, W. Winn Chatham ¹ and Randy Cron¹, ¹University of Alabama at Birmingham, Birmingham, ²HudsonAlpha Institute for Biotechnology, Huntsville
Background/Purpose: Cytokine storm syndromes (CSS), such as macrophage activation syndrome (MAS) and secondary hemophagocytic lymphohistiocytosis (HLH), are life threatening conditions that commonly present with unremitting…
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: 080 • 2020 Pediatric Rheumatology Symposium
Therapeutic Interferon Gamma Neutralization with Emapalumab in Patients with NRLC4- and CDC42-Associated Diseases Characterized by Recurrent and Severe Hemophagocytic Lymphohistiocytosis
Claudia Bracaglia¹, Antonella Insalaco ¹, Giulia Marucci ¹, Manuela Pardeo ¹, Emanuela Sacco ¹, Virginia Messia ¹, Giusi Prencipe ¹, Ivan Caiello ¹, Sarka Fingerhutova ², Pavla Dolezalova ², Veronica Asnaghi ³, Maria Ballabio ³, Cristina de Min ³ and Fabrizio De Benedetti ¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²Paediatric Rheumatology and Autoinflammatory Diseases Unit, General University Hospital, Prague, Czech Republic, ³Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: Interferon gamma (IFNγ) is a pivotal mediator of HLH. Emapalumab (a fully human anti-IFNγ monoclonal antibody) is efficacious in patients with primary HLH. In…
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: 108 • 2020 Pediatric Rheumatology Symposium
A Multinational Study of Thrombotic Microangiopathy in Macrophage Activation Syndrome: A Dreadful Condition Which Is Likely Underrecognized
Francesca Minoia¹, Jessica Tibaldi ², Valentina Muratore ³, Romina Gallizzi ⁴, Claudia Bracaglia ⁵, Alessia Arduini ⁶, Elif Comak ⁷, Olga Vougiouka ⁸, Ralf Trauzeddel ⁹, Giovanni Filocamo ¹⁰, Concetta Micalizzi ², Ozgur Kasapcopur ¹¹, Erbil Unsal ¹², Toshiyuki Kitoh ¹³, Elena Tsitsamis ¹⁴, Mikhail Kostik ¹⁵, Randy Cron ¹⁶, Jana Pachlopnik ¹⁷, Despoina Maritsi ¹⁸, Marija Jelusic ¹⁹, Susan Shenoi ²⁰ and Angelo Ravelli ², ¹Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy, ²Istituto Giannina Gaslini, Genoa, Italy, ³IRCCS Policlinico San Matteo, Pavia, Italy, ⁴Università degli Studi di Messina, Messina, Italy, ⁵Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ⁶IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁷Akdeniz University, Antalya, Turkey, ⁸Second Department of Paediatrics, P. & A. Kyriakou Children’s Hospital, Athens, Greece, ⁹Helios Klinikum, Berlin, Germany, ¹⁰Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, ¹¹Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Cerrahpasa, Turkey, ¹²Dokuz Eylul University, Izmir, Turkey, ¹³Aichi Medical University, Nagakute, Japan, ¹⁴Children's Hospital Aghia Sophia, Athens, Greece, ¹⁵State Pediatric Medical Academy, Saint Petersburg, Russia, ¹⁶University of Alabama at Birmingham, Birmingham, ¹⁷Universitats-Kinderspital, Zurich, Switzerland, ¹⁸National and Kopdistrian University of Athens, Athens, Greece, ¹⁹Division of Paediatric Rheumatology and Immunology, University of Zagreb, Zagreb, Croatia, ²⁰Seattle Children's Hospital, Seattle
Background/Purpose: Macrophage activation syndrome (MAS) is a severe complication of rheumatologic conditions, mainly systemic juvenile idiopathic arthritis (sJIA), and is classified among the secondary forms…
Abstract Number: 190 • 2020 Pediatric Rheumatology Symposium
Outcomes of an Evidence Based Guideline for the Treatment of Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome
Kacie Hoyt¹, Olha Halyabar ², Joseph Han ³, Siobhan Case ⁴, Margaret Chang ⁵, Ezra Cohen ⁶, Fatma Dedeoglu ⁵, Mark Gorman ⁷, Jonathan Hausmann ⁸, Erin Janssen ², Pui Lee ⁹, Jeffrey Lo ⁵, Mindy Lo ⁵, Esra Meidan ¹⁰, Peter Nigrovic ¹, Jordan Roberts ⁵, Mary Beth Son ¹, Robert Sundel ², Barbara Degar ⁵, Melissa Hazen ⁷ and Lauren Henderson ¹¹, ¹Boston Children's Hospital, Boston, Massachusetts, ²Children's Hospital/Boston Medical Center, Boston, ³Mount Sinai School of Medicine, New York, ⁴Brigham and Women's Hospital, Boston, Massachusetts, ⁵Boston Children's Hospital, Boston, ⁶, ⁷Boston, ⁸Division of Immunology, Boston Children's Hospital; Rheumatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, ⁹Boston Children's Hospital, Newton, ¹⁰Somerville, ¹¹Boston Children's Hospital, Watertown, Massachusetts
Background/Purpose: Rapid identification of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) coupled with a multidisciplinary approach to management is essential to improve patient outcomes.…
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: 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: 811 • 2019 ACR/ARP Annual Meeting
Monomethyl Fumarate as a Novel Therapy for Macrophage Activation Syndrome: Mechanism of Action in an Animal Model
Chhanda Biswas ¹, Thomas Burn ¹, Niansheng Chu ¹ and Edward Behrens¹, ¹The Children's Hospital of Philadelphia, Philadelphia
Background/Purpose: Macrophage activation syndrome (MAS) is a deadly systemic inflammatory condition marked by an increase in enzymes of iron metabolism including ferritin and heme-oxygenase 1…
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: 916 • 2019 ACR/ARP Annual Meeting
Development and Initial Validation of the MS Score for Diagnosis of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis
Francesca Minoia¹, Francesca Bovis ², Sergio Davì ³, AnnaCarin Horne ⁴, Michel Fischbach ⁵, Michael Frosch ⁶, Adam Huber ⁷, Marija Jelusic ⁸, Sujata Sawhney ⁹, Deborah McCurdy ¹⁰, Clovis Artur Silva ¹¹, Donato Rigante ¹², Erbil Unsal ¹³, Nicolino Ruperto ¹⁴, Alberto Martini ¹⁵, Randy Cron ¹⁶ and Angelo Ravelli ¹⁵, ¹Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Lombardia, Italy, ²Università degli Studi di Genova, Genoa, Italy, ³IRCCS Istituto Giannina Gaslini, Genova, Italy, ⁴Karolinska University Hospital, Stockholm, Sweden, ⁵Hopital Universitaire Hautepierre, Strasbourg, France, ⁶School of Medicine Witten/Herdecke University, Datteln, Germany, ⁷IWK Health Centre, Halifax, NS, Canada, ⁸University of Zagreb, Zagreb, Croatia, ⁹Sir Ganga Ram Hospital, New Delhi, India, ¹⁰University of California, MDCC 12-430, 10833 Le Conte Ave, Los Angeles, 90095, CA, USA, Los Angeles, ¹¹Childrens’ Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil, ¹²Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ¹³Dokuz Eylul University, School of Medicie, Izmir, Turkey, ¹⁴Paediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy, ¹⁵IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy, ¹⁶University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Macrophage activation syndrome (MAS) is the most severe complication of systemic juvenile idiopathic arthritis (sJIA) and its adult equivalent, adult-onset Still’s disease (AOSD). Because…
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…

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