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: 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: 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…

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