Abstracts tagged "macrophage activation syndrome"

Abstract Number: 0252 • ACR Convergence 2021
Role of Clinical and Laboratory Parameters in Differentiating Infection from Disease Flare in Febrile Patients of Systemic Juvenile Idiopathic Arthritis
Rajesh Kanumuri¹, Suma Balan², Vishal Marwaha², Sajitha Krishnan² and Pranav Chickermane², ¹Amrita Institute of Medical Sciences, Guntur, India, ²Amrita Institute of Medical Sciences, Kochi, India
Background/Purpose: Fever is the most common presentation of systemic juvenile idiopathic arthritis (SJIA) and it is difficult to predict whether the fever is due to…
Abstract Number: 0254 • ACR Convergence 2021
Patient and Disease-Level Factors Associated with Sustained Cessation of Medication for Disease Remission in Systemic Juvenile Idiopathic Arthritis
Elaine Flanagan¹, Rosemary Peterson², Susan Shenoi³, Helena Chang⁴, Kelly Wang⁴, Rebecca Trachtman⁴ and Karen Onel⁵, ¹Emory/CHOA, Atlanta, GA, ²Dell Children's Medical Center, Austin, TX, ³Seattle Children's Hospital, Seattle, WA, ⁴Icahn School of Medicine at Mount Sinai, New York, NY, ⁵Hospital for Special Surgery, New York, NY
Background/Purpose: The emergence of IL-1 and IL-6 inhibitors (biologics) for the treatment of systemic juvenile idiopathic arthritis (SJIA) has dramatically improved patient outcomes. With higher…
Abstract Number: 1541 • ACR Convergence 2021
Preliminary Criteria for Macrophage Activation Syndrome Associated with Coronavirus Disease-19
Shirkhan Amikishiyev¹, Mehmet Guven Gunver², Murat Bektas¹, Sarvan Aghamuradov¹, Burak Ince¹, Nevzat Koca¹, Ege Sinan Torun¹, Numune Aliyeva¹, Selma Sari¹, Cigdem Cetin³, Banu Cicek Yalcin Dulundu¹, Rabia Deniz⁴, Fatih Kemik⁴, Besim Fazil Agargun⁴, Ubeyde Ayse Gulseren⁴, Beliz Besisik⁴, Onur Alkan⁴, Ceren Bağrıaçık⁴, Yavuz Burak Tor⁴, Naci Senkal⁴, Yunus Catma⁴, Gorkem Durak⁵, Sevim Mese⁶, Ali Agacfidan⁶, Murat Kose⁴, Mustafa Erelel⁷, Arif Atahan Çağatay⁸, Sevgi Kalayoglu Besısık⁹, Figen Esen⁷ and Ahmet Gül¹⁰, ¹Istanbul Faculty of Medicine, Istanbul University, Division of Rheumatology, İstanbul, Turkey, ²Department of Biostatistics, Istanbul Medical Faculty, İstanbul, Turkey,, Istanbul, Turkey, ³Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey, ⁴Istanbul Faculty of Medicine, Istanbul University, Department of Internal Medicine, İstanbul, Turkey, ⁵Istanbul Faculty of Medicine, Istanbul University, Department of Radiology, İstanbul, Turkey, ⁶Istanbul Faculty of Medicine, Istanbul University, Department of Medical Microbiology, Division of Virology and Fundamental Immunology, İstanbul, Turkey, ⁷Istanbul Faculty of Medicine, Istanbul University, Department of Anesthesiology, İstanbul, Turkey, ⁸Istanbul Faculty of Medicine, Istanbul University, Department of Infectious Diseases and Clinical Microbiology, İstanbul, Turkey, ⁹Istanbul Faculty of Medicine, Istanbul University, Division of Hematology and Therapeutic Apheresis Unit, İstanbul, Turkey, ¹⁰Division of Rheumatology, Istanbul University Istanbul School of Medicine, Istanbul, Turkey
Background/Purpose: COVID-19 runs a severe disease associated with acute respiratory distress syndrome in a subset of patients, and a hyperinflammatory response developing in the second…
Abstract Number: 1547 • ACR Convergence 2021
Potential Predictors of Outcome for Anakinra Treatment in COVID-19 Patients with Macrophage Activation Syndrome
Shirkhan Amikishiyev¹, Rabia Deniz², Mehmet Guven Gunver³, Sarvan Aghamuradov¹, Nevzat Koca¹, Burak Ince¹, Murat Bektas¹, Gorkem Durak⁴, Murat Kose², Mustafa Erelel⁵, Arif Atahan Çağatay⁶, Sevgi Kalayoglu Besısık⁷, Figen Esen⁵ and Ahmet Gül⁸, ¹Istanbul Faculty of Medicine, Istanbul University, Division of Rheumatology, İstanbul, Turkey, ²Istanbul Faculty of Medicine, Istanbul University, Department of Internal Medicine, İstanbul, Turkey, ³Department of Biostatistics, Istanbul Medical Faculty, İstanbul, Turkey,, Istanbul, Turkey, ⁴Istanbul Faculty of Medicine, Istanbul University, Department of Radiology, İstanbul, Turkey, ⁵Istanbul Faculty of Medicine, Istanbul University, Department of Anesthesiology, İstanbul, Turkey, ⁶Istanbul Faculty of Medicine, Istanbul University, Department of Infectious Diseases and Clinical Microbiology, İstanbul, Turkey, ⁷Istanbul Faculty of Medicine, Istanbul University, Division of Hematology and Therapeutic Apheresis Unit, İstanbul, Turkey, ⁸Division of Rheumatology, Istanbul University Istanbul School of Medicine, Istanbul, Turkey
Background/Purpose: Coronavirus Disease 2019 (COVID-19) runs a severe course in a subset of patients with acute respiratory distress syndrome and multiorgan failure, and a hyperinflammatory…
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…

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