Abstracts tagged "macrophage activation syndrome"

Abstract Number: 1681 • ACR Convergence 2020
Hemophagocytic Lymphohistiocytosis (HLH) Gene Variants in Childhood-onset SLE (cSLE) with Macrophage Activation Syndrome (MAS)
Piya Lahiry¹, Sergey Naumenko², Fangming Liao³, Daniela Dominguez⁴, Andrea Knight⁵, Deborah Levy⁶, Melissa Misztal⁷, Lawrence Ng⁸, Earl D. Silverman⁹ and Linda Hiraki¹⁰, ¹Hospital for Sick Children, Toronto, ON, Canada, ²The Centre for Computational Medicine, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada, ³Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Toronto, Canada, ⁴Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada, ⁵Division of Rheumatology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, ON, Canada, ⁶Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ⁷Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Oakville, ON, Canada, ⁸Division of Rheumatology, Hospital for Sick Children, Toronto, Canada, ⁹Division of Rheumatology, The Hospital for Sick Children, Translational Medicine, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada, ¹⁰Division of Rheumatology, The Hospital for Sick Children, Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada
Background/Purpose: Familial Hemophagocytic lymphohistiocytosis (fHLH) is an autosomal recessive, hyper-inflammatory, life-threatening disease. Macrophage activation syndrome (MAS) is also known as secondary HLH due to the…
Abstract Number: 0087 • ACR Convergence 2020
Hemophagocytic Syndrome. Clinical Characteristics and Prognostic Factors of a Series of 30 Clinical Cases
César Antonio Egües Dubuc¹, Jaime Calvo-Alén², Andrea de Diego Sola³, Elizabeth Patricia Cabrera-Miranda⁴, Nerea Alcorta⁵, Luis Lopez Dominguez⁶, Olga Maiz⁵, Esther Uriarte Isacelaya⁶, Jorge Cancio Fanlo⁷, Jesus Alejandro Valero Jaimes⁶ and Joaquín Belzunegui¹, ¹Hospital Universitario Donostia, San Sebastián, Spain, ²Hospital Universitario Araba, Vitoria-Gasteiz, Pais Vasco, Spain, ³Hospital Universitario Donostia, San Sebasti�n, Spain, ⁴Hospital 12 de Octubre, Madrid, ⁵Hospital Universitario Donostia, San Sebastian, ⁶Hospital Universitario Donostia, SAN SEBASTIAN, Spain, ⁷Hospital Universitario Donostia, San Sebastian, Pais Vasco, Spain
Background/Purpose: Hemophagocytic syndrome (HPS) is classified into primary and secondary. The secondary form is mainly associated with hematological malignancies (HN) such as lymphomas, and autoimmune…
Abstract Number: 0088 • ACR Convergence 2020
The Spectrum of Hemophagocytic Lymphohistiocytosis: Autoimmunity vs. Malignancy
Samuel Good¹, Stefanie Wade¹ and Vasileios Kyttaris¹, ¹Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS), a life-threatening condition resulting from aberrant immune activation, is a form of hemophagocytic lymphohistiocytosis (HLH) that develops in patients with…
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: 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: 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…
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…

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