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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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