ACR Meeting Abstracts

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Abstracts tagged "macrophage activation syndrome"

  • Abstract Number: L19 • ACR Convergence 2024

    Efficacy and Safety of Emapalumab in Children and Adults with Macrophage Activation Syndrome (MAS) in Still’s Disease: Results from a Phase 3 Study and a Pooled Analysis of Two Prospective Trials

    Alexei Grom1, Uwe Ullman2, Adnan Mahmood3, Josefin Blomkvist3, Brian Jamieson4 and Fabrizio De Benedetti5, 1Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, 2Sobi, Basel, Switzerland, 3Sobi, Stockholm, Sweden, 4Sobi, Inc., Morrisville, NC, 5Bambino Gesu Children's Hospital, Rome, Italy

    Background/Purpose: MAS is a life-threatening complication of Still’s disease, characterized by interferon-gamma (IFNg)-driven macrophage activation and systemic hyperinflammation. Emapalumab, an anti-IFNg antibody, binds free and…
  • Abstract Number: 0389 • ACR Convergence 2024

    Histopathological Features of Liver Tissue Biopsies in SJIA Patients with and Without Clinical Macrophage Activation Syndrome

    Esraa Eloseily1, Lara Berklite2, Jennifer Picarsic1, grant schulert1, Rachel Sheridan1 and Alexei Grom1, 1Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Cincinnati Children's Hospital Medical Center, Cincinnti, OH

    Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) can present with or without Macrophage Activation Syndrome (MAS), a severe, potentially life-threatening complication. Liver tissue injury is commonly…
  • Abstract Number: 0829 • ACR Convergence 2024

    Epstein-Barr Virus Infection Triggers Hyperinflammation and Cytokine Storm in Healthy Children

    Kailey Brodeur1, Meng Liu2, Rachel Weng2, Evan Hsu2, Lauren Henderson3, Fatma Dedeoglu2, Jane Newburger3, Peter Nigrovic4, Mary Beth Son4 and Pui Lee5, 1Boston Children's Hospital, Cumberland, RI, 2Boston Children's Hospital, Boston, MA, 3Boston Children's Hospital, Watertown, MA, 4Boston Children's Hospital, Brookline, MA, 5Boston Children's Hospital, Newton, MA

    Background/Purpose: Epstein-Barr virus (EBV) is common pathogen responsible for infectious mononucleosis but also triggers hemophagocytic lymphohistiocytosis (HLH). This variation in the immune response to EBV…
  • Abstract Number: 0834 • ACR Convergence 2024

    Evolution of Lung Inflammation in a Mouse Model of Chronic/Recurrent Macrophage Activation Syndrome

    Richard Chhaing, Natsumi Inoue, Jana Latayan and grant schulert, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening complication and associated in >80% of cases with macrophage activation syndrome (MAS); however, the…
  • Abstract Number: 1765 • ACR Convergence 2024

    CD8+ T Cells and Monocytes from Children with Macrophage Activation Syndrome Demonstrate Specific Transcriptional Changes Consistent with T Cell Activation and Expansion of Monocytes Shaped by Interferon and TLR Signaling

    Susan Canny1, Hannah DeBerg2, Griffin Gessay2, Ailing Lu3, Mary Eckert4, Andrea La Bella5, Hayley Waterman2, Danish Nadeem2, Susan Shenoi6, Joyce Hui-Yuen7, Daniel Campbell2, Betsy Barnes8 and Jessica Hamerman2, 1University of Washington, Seattle, WA, 2Benaroya Research Institute, Seattle, WA, 3Northwell Health, Manhasset, NY, 4Seattle Children's Hospital, Seattle, WA, 5Cohen Children's Medical Center, Queens, NY, 6Seattle Children's Hospital and Research Center, Mercer Island, WA, WA, 7North Shore LIJ Health System, Great Neck, NY, 8Feinstein Institutes for Medical Science, Manhasset, NY

    Background/Purpose: Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (sHLH), is a potentially fatal complication of rheumatic diseases. MAS is characterized by a…
  • Abstract Number: 1768 • ACR Convergence 2024

    A Potential Role of Longstanding IL-18 Stimulation in the Susceptibility for Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis

    Greta Rogani1, Remco Erkens1, Marein Putmans1, Rianne Scholman1, Jorg van Loosdregt2 and Sebastiaan Vastert1, 1University Medical Center Utrecht, Utrecht, Netherlands, 2University Medical Center Utrecht, La Jolla, CA

    Background/Purpose: Macrophage Activation Syndrome (MAS) is a pathologic condition of immune hyperactivation, which occurs in 10-30% of cases of Still’s Disease (SD), the spectrum of…
  • Abstract Number: 1772 • ACR Convergence 2024

    Modeling and Predicting HLH Through Measurement of Immune Synapse Duration, Cytokine Production, and Target Cell Death

    Anastasia Frank-Kamenetskii1, Jemy Varghese2, Jeremy Morrissette3, Hannah Klinghoffer4, Caroline Diorio5, Janis Burkhardt6 and Scott Canna2, 1CHOP/UPENN, Philadelphia, PA, 2Children's Hospital of Philadelphia, Philadelphia, PA, 3University of Pennsylvania - Perelman School of Medicine, Philadelphia, PA, 4The Children's Hospital of Philadelphia, Philadelphia, PA, 5Division of Pediatric Oncology, The Children’s Hospital of Philadelphia, Philadelphia, PA, 6The Children's Hospital of Philadelphia, Philadelphia

    Background/Purpose: Hyperinflammation is a life-threatening systemic inflammatory state most commonly associated with Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS), but observed in nearly all inflammatory…
  • Abstract Number: 1782 • ACR Convergence 2024

    DOCK2 Mutations and Hyper-Inflammatory Syndromes

    Randy Cron, Mingce Zhang and Prescott Atkinson, University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Cytokine storm syndromes (CSS) are frequently fatal hyper-inflammatory complications of a variety of oncologic, rheumatic, and infectious diseases. Many patients with CSS possess heterozygous…
  • Abstract Number: 1997 • ACR Convergence 2024

    Features of interleukin-1 and interleukin-6 Inhibitor (IL-1i/IL-6i) Related Severe Delayed Adverse Reactions in Subjects with or Without Reaction-risk Associated HLA-DRB1*15

    Vivian E Saper1, Kazutoyo Osoegawa1, Ruud Verstegen2, Marcelo A Fernandez Vina1 and Lu Tian1, and Drug Hypersensitivity Consortium, 1Stanford University, Stanford, CA, 2The Hospital for Sick Children, Toronto, ON, Canada

    Background/Purpose: A very serious drug-related adverse event is reported with inhibitors of IL-1 and IL-6 (IL-1i/IL6i). This reaction scores as drug reaction with eosinophilia and…
  • Abstract Number: 2174 • ACR Convergence 2024

    Current Treatment of Macrophage Activation Syndrome Worldwide: The METAPHOR Project, a PReS/PRINTO Real-life International Survey

    Francesca Minoia1, Francesco Baldo2, Remco Erkens3, Greta Rogani3, Claudia Bracaglia4, Dirk Foell5, Marco Gattorno6, Marija Jelusic7, Jordi Anton8, Paul Brogan9, Scott Canna10, Shanmuganathan Chandrakasan11, Randy Cron12, Fabrizio De Benedetti4, Alexei Grom13, Merav Heshin Bekenstein14, AnnaCarin Horne15, Raju Khubchandani16, Mao Mizuta17, Seza Ozen18, PIERRE QUARTIER19, Angelo Ravelli6, Masaki Shimizu20, grant schulert13, Christiaan Scott21, Rashmi Sinha22, Nicolino Ruperto23, Joost Swart3 and Sebastiaan Vastert3, and the PReS MAS/sJIA Working Party and Pediatric Rheumatology International Trial Organization., 1Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy, 2Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, 3University Medical Center Utrecht, Utrecht, Netherlands, 4IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, 5University Hospital Muenster, Muenster, Germany, 6IRCCS Giannina Gaslini, Genoa, Italy, 7University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, 8Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, 9UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 10Children's Hospital of Philadelphia, Philadelphia, PA, 11Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta Emory University School of Medicine, Atlanta, GA, 12University of Alabama at Birmingham, Birmingham, AL, 13Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 14Dana Dwek Children's Hospital, Tel Aviv Medical Center, Tel Aviv University, Binyamina, Israel, 15Karolinska Institute, Sollentuna, Sweden, 16SRCC Childrens Hospital, Mumbai, India, 17Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kanazawa, Hyogo, Japan, 18Department of Pediatrics, Hacettepe University, Ankara, Turkey, 19Université Paris-Cite, IMAGINE Institute, Necker Children's Hospital, Paris Cedex 15, France, 20Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan, 21University of Cape Town, Cape Town, South Africa, 22Systemic JIA Foundation, Cincinnati, OH, 23IRCCS Giannina Gaslini, Genova, Italy

    Background/Purpose: Despite significant improvement in its management, macrophage activation syndrome (MAS) treatment is still not standardized, due to lack of robust evidence and differences in…
  • Abstract Number: 2569 • ACR Convergence 2024

    A Novel Hyperferritinemia Screen to Aid Differentiation of Hyperinflammatory Disorders

    Hallie Carol1, Adam Mayer2, Jemy Varghese3, Zachary Martinez4, Caroline Diorio4, Paul Tsoukas5, Kate Kernan6 and Scott Canna7, 1Children's Hospital of Philadelphia, Philadelphia, 2University of Pennsylvania/Children's Hospital of Philadelphia, Philadelphia, PA, 3Division of Pediatric Rheumatology, The Children’s Hospital of Philadelphia, Philadelphia, PA, 4Division of Pediatric Oncology, The Children’s Hospital of Philadelphia, Philadelphia, PA, 5Hospital for Sick Children/University of Toronto, Toronto, ON, Canada, 6Department of Critical Care Medicine, University of Pittsburgh School of Medicine, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, 7Children's Hospital of Philadelphia, Philadelphia, PA

    Background/Purpose: High ferritin is an important and sensitive biomarker for the diverse and deadly group of cytokine storm syndromes grouped together under the term hemophagocytic…
  • Abstract Number: 0760 • ACR Convergence 2023

    Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still’s Disease Treated with Emapalumab: The REAL-HLH Study

    Carl Allen1, Shanmuganathan Chandrakasan2, Michael Jordan3, Jennifer Leiding4, Abiola Oladapo5, Priti Pednekar6, Kelly Walkovich7 and John Yee5, 1Baylor College of Medicine, Houston, TX, 2Emory University School of Medicine, Atlanta, GA, 3Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, 4Division of Allergy and Immunology, Department of Pediatrics, Johns Hopkins University; bluebird bio, Cambridge, MA, 5Sobi, Inc., Waltham, MA, 6PRECISIONheor, Bethesda, MD, 7Division of Pediatric Hematology Oncology, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI

    Background/Purpose: Macrophage activating syndrome (MAS) is a rare, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD). MAS (a form…
  • Abstract Number: 0803 • ACR Convergence 2023

    IL10 Inhibits Toll-like Receptor-9-induced T Cell Receptor-mediated T Cell Activation in Macrophage Activation Syndrome

    Matthew Eremita1, Omar Geier2, Joyce Hui-Yuen1 and Matthew Taylor3, 1Division of Pediatric Rheumatology, Cohen Children's Medical Center, Lake Success, NY, 2Sepsis Research Laboratory, Feinstein Institutes for Medical Research, Manhasset, NY, 3Division of Pediatric Critical Care, Cohen Children's Medical Center, Manhasset, NY

    Background/Purpose: Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA). Gene expression pathway analyses have identified altered Toll-like receptor…
  • Abstract Number: 0865 • ACR Convergence 2023

    Reactive Oxygen Species-scavenging Nanoparticles Target Macrophage Polarization for Osteoarthritis Therapy

    Caifeng Deng1, Yongbing Xiao2, Xuan Zhao1, Hui Li2, Yuxiao Chen1, Chao Zeng2 and Guanghua Lei2, 1Xiangya Hospital, Changsha, China, 2Department of Orthopaedics, Xiangya Hospital, Central South University, Changsha, China

    Background/Purpose: Unbalanced M1/M2 polarization of synovial macrophages plays vital roles in the symptomatic and structural progression of osteoarthritis (OA). The accumulation of intracellular reactive oxygen…
  • Abstract Number: 1662 • ACR Convergence 2023

    Chronic Excess IL-18 Induces NK Deficiency, but Drives Hyperinflammation via CD8 T-cell Cytokine Overproduction and Selective Immunodeficiency

    Jemy Varghese1, Scott Canna1, Emily Landy2, Laurence Eisenlohr1, Elise Peauroi3, Vinh Dang1, Anastasia Frank-Kamenetskii4 and Jeremy Morrissette5, 1Children's Hospital of Philadelphia, Philadelphia, PA, 2University of Pittsburgh, Pittsburgh, PA, 3University of Pennsylvania, Philadelphia, PA, 4CHOP/UPENN, Philadelphia, PA, 5University of Pennsylvania - Perelman School of Medicine, Philadelphia, PA

    Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) and Macrophage Activation Syndrome (MAS) are associated with highly elevated peripheral blood levels of the inflammasome-activated cytokine IL-18 and…
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All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

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