Abstracts tagged "macrophage activation syndrome"

Abstract Number: 1156 • ACR Convergence 2020
Comparison of Immunological Biomarkers and Lung Histology in Patients with Elevated IL18 – Pulmonary Alveolar Proteinosis and Recurrent Macrophage Activation Syndrome (IL-18PAP-MAS) and Other Inflammatory Lung Diseases
Alhanouf Alsaleem¹, Adriana de Jesus², Sofia Torreggiani³, Chyi-Chia Lee⁴, Les Folio⁵, Huy Do⁶, Andrew Oler⁷, Caroline Kim³, Stewart Levine⁸, Anthony Suffredini⁹, Cem Gabay¹⁰, Joseph Fontana¹¹, Scott Canna¹² and Raphaela Goldbach-Mansky¹³, ¹Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁴Pathology Department/NCI/NIH, Bethesda, MD, ⁵Radiology and Imaging Services/NIH, Bethesda, MD, ⁶Radiology and Imaging Sciences, Bethesda, MD, ⁷Bioinformatics and Computational Biosciences Branch/NIAID/NIH, Bethesda, MD, ⁸Laboratory of Asthma and Lung Inflammation, Division of Intramural Research, NHLBI, NIH,, Bethesda, MD, ⁹Critical Care Medicine Department, Clinical Center, NIH, Bethesda, MD, ¹⁰University Hospitals of Geneva, Geneva, Switzerland, ¹¹NHLBI/NIH, Bethesda, MD, ¹²University of PIttsburgh, Pittsburgh, PA, ¹³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: Recently, pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS) have been reported in rare patients (pts) with systemic juvenile idiopathic arthritis (SJIA)…
Abstract Number: 1496 • ACR Convergence 2020
Outcomes of an Evidence Based Guideline for the Treatment of Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome
Kacie Hoyt¹, Olha Halyabar², Joseph Han³, Siobhan Case⁴, Margaret Chang¹, Craig Platt⁵, Ezra Cohen¹, Megan Day-Lewis⁵, Fatma Dedeoglu¹, Jonathan Hausmann⁶, Erin Janssen², Pui Lee⁷, Jeffrey Lo¹, Gregory Priebe⁵, Mindy Lo¹, Esra Meidan⁸, Peter Nigrovic⁹, Jordan Roberts¹, Mary Beth Son¹, Robert Sundel², Mark Gorman¹, Barbara Degar¹⁰, Melissa Hazen¹ and Lauren Henderson¹¹, ¹Boston Children's Hospital, Boston, MA, ²Children's Hospital/Boston Medical Center, Boston, MA, ³Mount Sinai School of Medicine, New York, NY, ⁴Brigham and Women's Hospital, Boston, MA, ⁵Boston Children's Hospital, Boston, ⁶Boston Children's Hospital / Beth Israel Deaconess Medical Center, Cambridge, MA, ⁷1.Boston Children's Hospital;2.Brigham and Women's Hospital;3.Harvard Medical School, Newton, MA, ⁸Boston Children's Hospital, Somerville, MA, ⁹Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA; Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA, Boston, ¹⁰Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston, ¹¹Boston Children's Hospital, Watertown, MA
Background/Purpose: Rapid identification of HLH/MAS coupled with a multidisciplinary approach to management is essential to improve patient outcomes. We describe our experience with a newly…
Abstract Number: 1634 • ACR Convergence 2020
Etiologies and Management of Hemophagocytic Lymphohistiocytosis: Is It Time for an Updated Protocol and Targeted Treatments?
Therese Posas-Mendoza¹, Cara McLeod¹, William Davis² and Robert Quinet², ¹Ochsner Medical Center, New Orleans, LA, ²Dept of Rheumatology, Ochsner Medical Center, New Orleans, LA
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease characterized by immune-overstimulation and a hyperinflammatory response resulting in cytokine storm and multi-organ failure.1 Secondary HLH…
Abstract Number: 1671 • ACR Convergence 2020
Identifying Rare Genetic Variants in Childhood-onset Monogenic Systemic Lupus Erythematosus
Melissa Misztal¹, Fangming Liao², Sergey Naumenko³, Andrea Knight⁴, Daniela Dominguez⁵, JingJing Cao², Declan Webber⁶, Bhooma Thiruvahindrapuram⁷, Deborah Levy⁶, Andrew Paterson², Earl D. Silverman⁸ and Linda Hiraki⁹, ¹Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Oakville, ON, Canada, ²Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Toronto, Canada, ³The Centre for Computational Medicine, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada, ⁴Division of Rheumatology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, ON, Canada, ⁵Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada, ⁶Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ⁷The Centre for Applied Genomics, The Hospital for Sick Children, Toronto, ⁸Division 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, ⁹Division 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: Among children diagnosed with systemic lupus erythematosus (SLE), there exists monogenic forms of SLE, where rare variants in a single gene lead to disease.…
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…

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