Abstracts tagged "macrophage activation syndrome"

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: 0196 • ACR Convergence 2021
A Single Center, Double Blind, Randomized, Placebo-Controlled Trial of Anakinra in Adult Patients with Features of Cytokine Storm Syndrome in COVID-19
Lesley Jackson¹, Randy Cron¹, Nitasha Khullar², Christopher Chapleau³, Dongmei Sun⁴ and Winn Chatham¹, ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Alabama Birmingham, Birmingham, AL, ³UAB Pharmacy, University of Alabama at Birmingham, Birmingham, AL, ⁴Division of Clinical Immunology and Rheumatology, The University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Some patients with COVID-19 develop respiratory distress and cytokine storm syndrome (CSS) which is characterized by hyperinflammation and may progress to multi-organ failure. Anakinra…
Abstract Number: 0246 • ACR Convergence 2021
Macrophage Activating Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis Treated with Biological Drugs
Betul Sozeri¹, Kenan Barut², Erdal Atalay³, Aysenur Pac Kisaarslan⁴, Semanur Ozdel⁵, Ozge Altug⁶, Ferhat Demir¹, Balahan Makay⁷, Nuray Aktay Ayaz⁸, Ersen Acar⁹, Fatih Haslak², Erdal Sag¹⁰, Mehmet YILDIZ², Ummusen Kaya Akca¹⁰, Amra Adrovic², Yelda Bilginer¹⁰, Hakan Poyrazoglu¹¹, Erbil Unsal⁷, Ozgur Kasapcopur¹² and Seza Ozen³, ¹University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology, Istanbul, Turkey, ²Istanbul University-Cerrahpasa, Faculty of Medicine, Pediatric Rheumatology, İstanbul, Turkey, ³Hacettepe University Faculty of Medicine, Pediatric Romatology, Ankara, Turkey, ⁴Erciyes University Medical Faculty, Pediatric Rheumatology, Kayeri, Turkey, ⁵Ankara Dr.Sami Ulus Gynecology and Pediatrics Research and Training Hospital, Pediatric Rheumatology, Ankara, Turkey, ⁶Dr Behcet Uz Children's Hospital, Pediatric Rheumatology, Izmir, Turkey, ⁷Dokuz Eylul University Faculty of Medicine, Pediatric Rheumatology, Izmir, Turkey, ⁸Istanbul University, Faculty of Medicine, Pediatric Rheumatology, İstanbul, Turkey, ⁹Novartis, Istanbul, Turkey, ¹⁰Hacettepe University, Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Ankara, Turkey, ¹¹Erciyes University Faculty of Medicine, Pediatric Rheumatology, Kayseri, Turkey, ¹²Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) is the most acute and severe form of JIA. Despite the substantial evidence supporting the effectiveness of biologic drugs…
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: 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…

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