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: 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: 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: 0363 • ACR Convergence 2023
Allogenic Hematopoietic Stem Cell Transplant for Children with Refractory Systemic Juvenile Idiopathic Arthritis and sJIA-Associated Lung Disease: 6-month Post-Transplant Outcomes from an International Cohort
Michael Matt¹, Daniel Drozdov¹, Rolla Abu-Arja², Shanmuganathan Chandrakasan³, Kyla Driest², Elvira Cannizzaro Schneider⁴, Despina Moshous⁵, Benedicte Neven⁵, Karen Onel⁶, Sampath Prahalad⁷, Susan Prockop⁸, Pierre Quartier⁵, Johannes Roth⁹, Donna Wall¹⁰, Ulrike Zeilhofer¹¹, Scott Canna¹², Alexei Grom¹, Grant Schulert¹ and Rebecca Marsh¹, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Nationwide Children's Hospital, Columbus, OH, ³Emory University School of Medicine, Atlanta, GA, ⁴Universitaetskinderspital Zurich, Zurich, Switzerland, ⁵Paris Cité Université and Necker Hospital, Paris, France, ⁶Hospital for Special Surgery, New York, NY, ⁷Emory University School of Medicine, Children's Pediatric Institute, Atlanta, GA, ⁸Division of Hematology-Oncology, Boston Children's Hospital, Boston, MA, ⁹University of Ottawa, Ottawa, ON, Canada, ¹⁰The Hospital for Sick Children, Toronto, ON, Canada, ¹¹Children's Hospital of Zurich, Zurich, Switzerland, ¹²Children's Hospital of Philadelphia, Philadelphia, PA
Background/Purpose: Refractory systemic juvenile idiopathic arthritis (sJIA) in children can be complicated by repeated episodes of macrophage activation syndrome (MAS) or sJIA-related lung disease (sJIA-LD).…
Abstract Number: 0378 • ACR Convergence 2023
Characteristics of Macrophage Activation Syndrome in Systemic JIA Patients Receiving Anakinra as First-line Treatment
Remco Erkens¹, Laura Huber¹, Anouk Verwoerd¹, Greta Rogani¹, Dieneke Schonenberg-Meinema², Merlijn van den Berg², Wineke Armbrust³, Elizabeth Legger⁴, Sylvia Kamphuis⁵, Ellen Schatorjé⁶, Esther Hoppenreijs⁷, Joost Swart⁸, Marc Jansen¹, Jorg van Loosdregt¹ and Sebastiaan Vastert¹, ¹University Medical Center Utrecht, Utrecht, Netherlands, ²Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, Netherlands, ³University Medical Hospital Groningen, Groningen, Netherlands, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Erasmus University Medical Center, Department of Pediatric Rheumatology, Rotterdam, Netherlands, ⁶University Medical Center Radboud, Nijmegen, Netherlands, ⁷Radboud University Medical Center, Nijmegen, Netherlands, ⁸Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Netherlands
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) is a severe inflammatory disease with auto-inflammatory characteristics. The introduction of targeted biologic therapies has revolutionized the treatment and…
Abstract Number: 0758 • ACR Convergence 2023
Clinical and Biological Characteristics of Children and Adults Affected with Still’s Disease: A Systematic Review and Meta-analysis Informing the 2023 EULAR/PReS Recommendations for the Diagnosis and Management of Systemic Juvenile Idiopathic Arthritis and Adult-onset Still’s Disease
STEPHANE MITROVIC¹, Arianna De Matteis², Sara Bindoli³, Fabrizio De Benedetti⁴, Bruno Fautrel⁵, Loreto Carmona⁶ and On Behalf Of The Eular/PreS QoC011 Task Force Member⁷, ¹Pitie Salpetriere Hospital, Sorbonne University, APHP, Paris, France, ²IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ³Rheumatology Unit, Dept of Medicine, University of Padova, Padova, Italy, ⁴Bambino Gesu Children's Hospital, Division of Rheumatology, Rome, Italy, ⁵Sorbonne Université APHP, Paris, France, ⁶Instituto de Salud Musculoesquelética (InMusc), Madrid, Spain, ⁷EULAR/PReS QoC011 Task Force members are: Jordi Anton, Alexandre Belot, Claudia Bracaglia, Tamas Constantin, Lorenzo Dagna, Alessandro De Bartolo, Eugen Feist, Dirk Foell, Marco Gattorno, Sophie Georgin-Lavialle, Roberto Giacomelli, Alexei Grom, Yvan Jamilloux, Katarina Laskari, Calin Lazar, Francesca Minoia, Peter Nigrovic, Filipa Oliveira Ramos, Seza Ozen, Pierre Quartier-dit-Maire, Piero Ruscitti, Erdal Sag, Sinisa Savic, Marie-Elise Truchetet, Bas Vastert, Tanita Wilhelmer, Carine Wouters., Paris, France
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are part of the non-familial (sporadic) systemic inflammatory disorders and are frequently mentioned as…
Abstract Number: 0759 • ACR Convergence 2023
Derivation and Validation of Four Patient Clusters in Still’s Disease, Results from GIRRCS AOSD-study Group and AIDA Network Still Disease Registry
Piero Ruscitti¹, Antonio Vitale², Ilenia Di Cola¹, Roberto giacomelli³ and Luca Cantarini⁴, ¹University of L'Aquila, L'Aquila, Italy, ²University of Siena, Siena, Italy, ³University of Rome Campus Biomedico, Rome, Italy, ⁴Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy
Background/Purpose: Still's disease is a rare inflammatory disorder, these patients have a highly heterogeneous disease according to age of onset, clinical presentation, presence of life-threatening…

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