Abstracts tagged "macrophage activation syndrome"

Abstract Number: 0562 • ACR Convergence 2022
Large-Scale Targeted Sequencing Study Links Systemic Juvenile Idiopathic Arthritis with Rare Variants of MEFV, LYST, STXBP2, UNC13D
Mariana Correia Marques¹, Danielle Rubin², Emily Shuldiner², Elizabeth Schmitz², Elizabeth Baskin², Andrew Patt³, Alexei Grom⁴, Dirk Foell⁵, Marco Gattorno⁶, John Bohnsack⁷, Rae Yeung⁸, Sampath Prahalad⁹, Elizabeth Mellins¹⁰, Jordi Antón¹¹, Claudio Len¹², Sheila Oliveira¹³, Patricia Woo¹⁴, Seza Ozen¹⁵, INCHARGE Consortium¹⁶ and Michael Ombrello¹⁷, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases / Children`s National Hospital, Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ³National Center for Advancing Translational Sciences, Bethesda, MD, ⁴Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵University Hospital Münster, Münster, Germany, ⁶Pediatric Clinic and Rheumatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy, ⁷University of Utah, Salt Lake City, UT, ⁸The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁹Emory + Children's Pediatric Institute, Atlanta, GA, ¹⁰Stanford University, Stanford, CA, ¹¹Pediatric Rheumatology Department. Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat, Spain, ¹²Universidade Federal de São Paulo, São Paulo, Brazil, ¹³Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁴University College London, London, United Kingdom, ¹⁵Hacettepe University Faculty of Medicine, Ankara, Turkey, ¹⁶International Childhood Arthritis Genetics Consortium, Bethesda, MD, ¹⁷National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a genetically complex inflammatory condition. It can be marked by severe systemic inflammation that resembles the hereditary periodic…
Abstract Number: 0569 • ACR Convergence 2022
Cytokine Storm Susceptibility Factors IL-18 and Cytotoxic Impairment Converge on Pathologic CD8 T-cell Hyperactivation
Emily Landy¹, Scott Canna², Paul Tsoukas³, Vinh Dang² and Jemy Varghese², ¹University of Pittsburgh, Pittsburgh, PA, ²Children's Hospital of Philadelphia, Philadelphia, PA, ³The Hospital for Sick Children, Toronto, ON, Canada
Background/Purpose: Mounting evidence suggests partial defects in cytotoxicity-related genes may promote hyperinflammation in Macrophage Activation Syndrome (MAS) patients, who uniformly have highly-elevated IL-18. In mice,…
Abstract Number: 0799 • ACR Convergence 2022
Serum Laboratory Parameters of Hemophagocytic Lymphohistiocytosis Syndrome in Patients with Autoimmune Rheumatic Diseases Infected with SARS-CoV-2: A Retrospective Comparative Study
Omar Alsaed, Samar Al emadi, Karima Becetti, Eman Satti, Hadil Ashour, Yousef Alrimawi, Miral H.Gharib, Mohamad Alkahlout, Rawan Saleh, Basem Awadh and Mohammed Hammoudeh, Hamad Medical Corporation, Doha, Qatar
Background/Purpose: The serum features of the cytokine storm induced by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection are similar to those of hemophagocytic lymphohistiocytosis…
Abstract Number: 0825 • ACR Convergence 2022
Predicting Outcomes in Macrophage Activation Syndrome (MAS): A Retrospective Study on 43 Consecutive Patients with MAS
Sumanth Madan¹, Suma Balan¹, MITHUN CHALAKKARAYIL BHAGAVALDAS², Spoorthy DR¹, Sudeep Rath¹, Joslyn Thattil¹, Amisha Shahul¹ and Niveditha Kartha¹, ¹Amrita Institute of Medical Sciences, Kochi, India, ²Amrita Institute of Medical Sciences, Muvattupuzha, India
Background/Purpose: Macrophage activation syndrome is a diagnosis along the spectrum of hemophagocytic lymphohistiocytosis (HLH) spectrum disorders. It is a rare, yet potentially fatal disease characterized…
Abstract Number: 0859 • ACR Convergence 2022
Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe
Claudia Bracaglia¹, Francesca Minoia², Christoph Kessel³, Sebastiaan Vastert⁴, Manuela Pardeo¹, Alessia Arduini¹, Ozge Basaran⁵, Nural kiper⁶, Mikhail Kostik⁷, Mia Glerup⁸, Sarka Fingerhutova⁹, Roberta Caorsi¹⁰, AnnaCarin Horne¹¹, Giovanni Filocamo¹², Helmut Wittkowski³, Marija Jelusic¹³, Jordi Anton¹⁴, Samira Khaldi-Plassart¹⁵, Alexandre Belot¹⁶, Gerd Horneff¹⁷, Seraina Palmer Sarott¹⁸, Elvira cannizzaro Schneider¹⁸, Pavla Dolezalova⁹, Angelo Ravelli¹⁹, Seza Ozen²⁰ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy, ³Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Münster, Germany, ⁴Pediatric Rheumatology & Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁵Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, ⁶Department of Pediatrics, Division of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey, ⁷Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ⁸Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, ⁹Centre for Paediatric Rheumatology and Autoinflammatory Diseases , Department of Paediatrics and Inherited Metabolic Disorders, 1st Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic, ¹⁰Department of pediatrics and Rheumatology, IRRCS Istituto G. Gaslini, Genova, Italy, ¹¹Department of pediatric rheumathology Karolinska University Hospital and Department of pediatrics, Karolinska Institute, Stockholm, Sweden, ¹²UOC Pediatria a Media Intensità di Cure, Clinica de Marchi, Milano, Italy, ¹³Department of Pediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia, ¹⁴Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, ¹⁵Pediatric Nephrology, Rheumatology, Dermatology Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France, ¹⁶Hospices Civils de Lyon, Collonges au mont d'or, France, ¹⁷Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ¹⁸Paediatric Rheumatology University Children’s Hospital Zurich, Zürich, Switzerland, ¹⁹Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal Infantile Sciences (DINOGMI) University of Genoa, Italy,Scientific Direction, IRCCS Istituto Giannina Gaslini, Genova, Italy, ²⁰Hacettepe University Faculty of Medicine, Ankara, Turkey
Background/Purpose: Chronic parenchymal lung disease (LD) is a new emerging severe life-threatening complication of sJIA. The number of sJIA patients with LD is apparently increasing…
Abstract Number: 0864 • ACR Convergence 2022
Baseline Clinical and Laboratory Features of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease (SJIA-LD) Cohort
Esraa Eloseily¹, Min-Lee Chang², MaryEllen Riordan³, Alan Russell⁴, Marc Natter², Yukiko Kimura⁵ and Grant Schulert⁶, ¹Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Boston Children's Hospital, Boston, MA, ³Hackensack Meridian Health, Hackensack, NJ, ⁴Duke Clinical Research Institute, Duke, NC, ⁵Hackensack Meridian Health, New York, NY, ⁶Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) associated lung disease (SJIA-LD) is an emerging and life threatening clinical problem, and currently affects as many as 1…
Abstract Number: 0872 • ACR Convergence 2022
Incidence, Risk Factors, and Outcomes of Eosinophilia on IL-1 and IL-6 Inhibitors in Systemic and Non-Systemic Juvenile Idiopathic Arthritis
Holly Wobma¹, Maria Taylor², Ki Pui Lam¹, Helene Brown¹, Siobhan Case¹, Mia Chandler¹, Margaret Chang¹, Ezra Cohen¹, Megan Day-Lewis¹, Olha Halyabar¹, Jonathan Hausmann¹, Melissa Hazen³, Erin Janssen¹, Pui Lee¹, Mindy Lo¹, Esra Meidan¹, Jordan Roberts¹, Mary Beth F. Son¹, Robert Sundel¹, Fatma Dedeoglu¹, Peter Nigrovic¹, Alicia Casey⁴, Joyce Chang¹ and lauren henderson¹, ¹Division of Immunology, Boston Children's Hospital, Boston, MA, ²Division of Immunology, Boston Children's Hospital, Brighton, MA, ³Boston Children's Hospital, Boston, MA, ⁴Division of Pulmonary Medicine, Boston Children's Hospital, Boston, MA
Background/Purpose: Children with systemic juvenile idiopathic arthritis (sJIA) exposed to IL1/6 inhibitors may develop eosinophilia with an atypical rash, reportedly in association with HLA-DRB1*15:XX. There…
Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Kailey Brodeur¹, Liang Chen¹, zhengping huang², Yan Du¹, Holly Wobma³, Maria Taylor⁴, Joyce Chang³, Megan Day-Lewis³, Fatma Dedeoglu³, Olha Halyabar³, Mindy Lo³, Jane W. Newburger⁵, Mary Beth F. Son³, Robert Sundel³, Peter Nigrovic³, lauren henderson³ and Pui Lee³, ¹Boston Children's Hospital, Boston, MA, ²Guangdong Second Provincial Hospital, Guangzhou, China, ³Division of Immunology, Boston Children's Hospital, Boston, MA, ⁴Division of Immunology, Boston Children's Hospital, Brighton, MA, ⁵Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
Abstract Number: L20 • ACR Convergence 2021
Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA) Who Had Failed High-Dose Glucocorticoids (GCs)
Fabrizio De Benedetti¹, Alexei Grom², Paul Brogan³, Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci¹, Despina Eleftheriou³, Charalampia Papadopoulou³, Pierre Quartier⁴, Jordi Antón⁵, Rikke Frederiksen⁶, Veronica Asnaghi⁶ and Cristina De Min⁶, ¹Bambino Gesù Children's Hospital IRCCS, Rome, Italy, ²Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁴Université de Paris, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁵Hospital Sant Joan de Déu, Division of Pediatric Rheumatology, University of Barcelona, Barcelona, Spain, ⁶Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a life-threatening complication of rheumatic diseases, occurring most frequently in sJIA. The mainstay of MAS treatment is high dose GCs; however, GCs…
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: 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…

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