Abstracts tagged "macrophage activation syndrome"

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: 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: 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)…
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.…

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