Abstracts tagged "macrophage activation syndrome"

Abstract Number: 1619 • ACR Convergence 2021
Where Do Multisystem Inflammatory Syndrome in Children (MIS-C) and Pediatric COVID-19 Fit Under the Cytokine Storm Syndrome Umbrella?
Randy Cron and Daniel Reiff, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Although COVID-19 has been less severe in the pediatric population, with cases more likely to be mild or asymptomatic, there remains a proportion of…
Abstract Number: 1928 • ACR Convergence 2021
In Vitro and In Vivo Evidence for DOCK8 as a Risk Allele for Cytokine Storm Syndrome, Including COVID-19 and MIS-C

Randy Cron¹, Mingce Zhang¹, Niansheng Chu², Devin Absher³, John Bridges¹, Amanda Schnell¹, Anshul Vagrecha⁴, Shannon Lozinsky⁴, Suchitra Acharya⁴, Carolyn Levy⁴, Winn Chatham¹ and Edward Behrens², ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Pennsylvania, Philadelphia, PA, ³HudsonAlpha Institute for Biotechnology, Huntsville, AL, ⁴Hofstra/Northwell School of Medicine, Hempstead, NY
Background/Purpose: Cytokine storm syndromes (CSS) are frequently fatal complications of a variety of oncologic, rheumatic, and infectious diseases. Many patients with CSS possess heterozygous missense…
Abstract Number: 1930 • ACR Convergence 2021
Single-Cell Genomics Reveals a Shared Monocyte Interferon Program in a Subset of Patients with Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome and Lung Disease
Emely Verweyen¹, Kairavee Thakkar², Kashish Chetal², Sanjeev Dhakal³, Alexei Grom², Nathan Salomonis² and Grant Schulert², ¹Cincinnati Children's Hospital, Cincinnati, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Cincinnati Children hospital, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a clinically heterogenous disease and can be complicated by macrophage activation syndrome (MAS) and lung disease (LD) thought…
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: 0469 • ACR Convergence 2020
IFNγ Is Essential for Alveolar Macrophage Driven Lung Inflammation in Macrophage Activation Syndrome
Denny Gao¹, Maggie Henderlight¹, Christopher Woods¹, Alexei Grom¹, Sherry Thornton¹, Michael Jordan¹, Katheryn Wikenheiser-Brokamp¹ and Grant Schulert², ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening cytokine storm syndrome frequently complicating systemic juvenile idiopathic arthritis (SJIA) and driven by IFNγ. MAS is also…
Abstract Number: 0473 • ACR Convergence 2020
Characterization of DOCK8 as a Novel Gene Associated with Cytokine Storm Syndrome
Mingce Zhang¹, Remy Cron², Devin Absher³, Prescott Atkinson¹, Winn Chatham¹ and Randy Cron¹, ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Alabama at Birmingham, Birmingham, ³HudsonAlpha Institute for Biotechnology, Huntsville, AL
Background/Purpose: Cytokine storm syndrome (CSS), also known as macrophage activation syndrome (MAS) or secondary hemophagocytic lymphohistiocytosis (HLH), is a life threatening condition that commonly presents…
Abstract Number: 0592 • ACR Convergence 2020
A Rheumatology-Driven Protocol and Treatment Algorithm of SARS-CoV-2 Cytokine Release Syndrome and Its Associated Outcomes
Sandy Lee¹, Neha Chiruvolu², Muntarin Karim³, Patil Injean⁴, Loomee Doo⁵, Donna Jose⁶, Deepa Panikkath¹, Micah Yu⁵, Anna Lafian⁵, Wendy De La Pena⁷, Albert Chow⁸, Karina Torralba⁹, Vaneet Sandhu¹⁰, Mehrnaz Hojjati⁵, Marven Cabling⁵ and Christina Downey¹, ¹Loma Linda University Medical Center, Redlands, CA, ²UC Riverside School of Medicine, Riverside, CA, ³Loma Linda University Health System, Loma Linda, CA, ⁴LOMA LINDA UNIVERSITY MEDICAL CENTER - RHEUMATOLOGY, Redlands, CA, ⁵Loma Linda University Medical Center, LOMA LINDA, CA, ⁶Loma Linda University Medical Center, Ontario, CA, ⁷PRCSG, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁸Loma Linda University, San Bernardino, CA, ⁹Loma Linda University School of Medicine, Redlands, CA, ¹⁰Loma Linda University, Loma Linda, CA
Background/Purpose: The newly identified SARS-CoV-2 has brought Cytokine Release Syndrome (CRS) to a level of prominence not often seen in adult medicine. Mortality rates of…
Abstract Number: 0623 • ACR Convergence 2020
Cytokine Storm: Outcomes in SARS-CoV-2 Patients Treated with Biologics in a Rheumatology Cohort
Neha Chiruvolu¹, Muntarin Karim², Patil Injean³, Sandy Lee³, Karina Torralba⁴, Christina Downey³, Mehrnaz Hojjati⁵, Loomee Doo⁵, Donna Jose⁶, Deepa Panikkath³, Micah Yu⁵, Anna Lafian⁵, Marven Cabling⁵ and Vaneet Sandhu⁷, ¹UC Riverside School of Medicine, Riverside, CA, ²Loma Linda University Health System, Loma Linda, CA, ³Loma Linda University Medical Center, Redlands, CA, ⁴Loma Linda University School of Medicine, Redlands, CA, ⁵Loma Linda University Medical Center, Loma Linda, CA, ⁶Loma Linda University Medical Center, Ontario, CA, ⁷Loma Linda University, Loma Linda, CA
Background/Purpose: Cytokine Release Syndrome (CRS) or Macrophage Activation Syndrome (MAS) is a life threating hyperinflammatory condition that can complicate rheumatic disease as well as infections…
Abstract Number: 0639 • ACR Convergence 2020
Immunological Abnormalities in a SARS-CoV-2-Cytokine Release Syndrome Rheumatology Cohort
Patil Injean¹, Sandy Lee¹, Neha Chiruvolu², Muntarin Karim³, Loomee Doo⁴, Deepa Ragesh Panikkath⁵, Donna Jose⁴, Micah Yu⁴, Anna Lafian⁴, Vaneet Sandhu⁶, Karina Torralba⁷, Christina Downey¹, Marven Cabling⁸ and Mehrnaz Hojjati⁹, ¹Loma Linda University Medical Center, Redlands, CA, ²UC Riverside School of Medicine, Riverside, CA, ³Loma Linda University Health System, Loma Linda, CA, ⁴Loma Linda University Medical Center, Loma Linda, CA, ⁵Loma Linda University Health, Department of Rheumatology, Loma Linda, CA, ⁶Loma Linda University, Loma Linda, CA, ⁷Loma Linda University School of Medicine, Redlands, CA, ⁸Loma Linda University Medical Center, Loma Linda, ⁹Loma Linda University, Loma Linda
Background/Purpose: Cytokine release syndrome (CRS) is a condition characterized by a sepsis-like condition and laboratory abnormalities such as high ferritin, low ESR, and low fibrinogen…
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: 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)…

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