Abstracts tagged "macrophage activation syndrome"

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…
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…
Abstract Number: 1711 • ACR Convergence 2023
CD14+ Monocytes Demonstrate a Unique Transcriptional Signature in Macrophage Activation Syndrome, Highlighting a Role for Interferons and Identifying Putative Hemophagocytes in Circulation
Susan Canny¹, Hannah DeBerg², Griffin Gessay², Ailing Lu³, Mary Eckert⁴, Andrea La Bella⁵, Susan Shenoi⁶, Joyce Hui-Yuen⁷, Betsy Barnes⁸ and Jessica Hamerman², ¹Seattle Children's Hospital/University of Washington, Seattle, WA, ²Benaroya Research Institute, Seattle, WA, ³Feinstein Institutes for Medical Research, Manhasset, NY, ⁴Seattle Children's Hospital, Seattle, WA, ⁵Cohen Children's Medical Center, Queens, NY, ⁶Seattle Childrens Hospital, Mercer Island, WA, ⁷Division of Pediatric Rheumatology, Cohen Children's Medical Center, Lake Success, NY, ⁸Northwell Health, Manhasset, NY
Background/Purpose: Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic diseases. MAS is characterized by a dysfunctional hyperinflammatory response in which there is…
Abstract Number: 2473 • ACR Convergence 2023
A Consensus Based Algorithm to Screen for Lung Disease in Children with Systemic Juvenile Idiopathic Arthritis
Holly Wobma¹, Ronny Bachrach², Joseph Farrell², Margaret Chang³, Megan Day-Lewis¹, Fatma Dedeoglu¹, Martha Fishman⁴, Olha Halyabar¹, Claudia Harris¹, Daniel Ibanez¹, Liyoung Kim¹, Timothy Klouda⁴, Katie Krone⁴, Pui Lee¹, Mindy Lo¹, Kyle McBrearty¹, Esra Meidan¹, Susan Prockop⁵, Aaida Samad⁴, Mary Beth Son¹, Peter Nigrovic³, Alicia Casey⁴, Joyce Chang³ and Lauren Henderson³, ¹Division of Immunology, Boston Children's Hospital, Boston, MA, ²Community Member, Boston, MA, ³Boston Children's Hospital, Boston, MA, ⁴Division of Pulmonary Medicine, Boston Children’s Hospital, Boston, MA, ⁵Division of Hematology-Oncology, Boston Children's Hospital, Boston, MA
Background/Purpose: Over the last decade, lung disease (LD) has become an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). Children with sJIA-LD may be…
Abstract Number: 079 • 2023 Pediatric Rheumatology Symposium
Dynamics of Neutrophil Activation in Repeated TLR-9-Induced Mouse Model of Macrophage Activation Syndrome
Natsumi Inoue, Richard Chhaing, Sanjeev Dhakal, Thuy Do and Grant Schulert, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases including systemic juvenile idiopathic arthritis (SJIA). SJIA shows prominent neutrophil activation with expansion…
Abstract Number: 095 • 2023 Pediatric Rheumatology Symposium
Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe
Claudia Bracaglia¹, Francesca Minoia², Christoph Kessel³, Sebastiaan Vastert⁴, Manuela Pardeo¹, Alessia Arduini¹, Sarka Fingerhutova⁵, Irina Nikishina⁶, Ozge Basaran⁷, Nural Kiper⁸, Mikhail Kostik⁹, Mia Glerup¹⁰, Roberta Caorsi¹¹, AnnaCarin Horne¹², Giovanni Filocamo², Helmut Wittkowski³, Marija Jelusic¹³, Jordi Anton¹⁴, Samira Khaldi-Plassart¹⁵, Alexandre Belot¹⁵, Gerd Horneff¹⁶, Seraina Palmer Sarrott¹⁷, Elvira Cannizzaro Schneider¹⁸, Lampros Fotis¹⁹, Pavla Dolezalova⁵, Angelo Ravelli²⁰, Seza Ozen⁷ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ²Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milano, Italy, ³Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Muenster, Germany, ⁴Wilhelmina Children’s Hospital, Department of Pediatric Immunology and Rheumatology, Utrecht, The Netherlands, Utrecht, Netherlands, ⁵Centre 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, ⁶V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia, ⁷Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, ⁸Department of Pediatrics, Division of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey, ⁹Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁰Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, ¹¹Department of Pediatrics and Rheumatology, IRRCS Istituto G. Gaslini, Genova, Italy, ¹²Department of pediatric rheumathology Karolinska University Hospital and Department of pediatrics, Karolinska Institute, Stockholm, Sweden, ¹³Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre, 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, ¹⁶Pediatrics, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ¹⁷Paediatric Rheumatology University Children’s Hospital Zurich, Zurich, Zurich, Switzerland, ¹⁸Paediatric Rheumatology University Children’s Hospital Zurich, Zurich, Switzerland, ¹⁹Pediatric Rheumatology Division, 3rd Department of Pediatrics, National and Kapodistrian University of Athens,‘’ATTIKON’’ General University Hospital, Athens, Greece, ²⁰IRRCS Istituto Giannina Gaslini and Università degli Studi di Genova, Genova, Italy
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: 031 • 2023 Pediatric Rheumatology Symposium
Multisystem Inflammatory Syndrome in Children Phenotypes Vary Between SARS-CoV-2 Variants
Greta Mastrangelo¹, Ellen Go², Paul Tsoukas², Hua Lu³, Amy Xu², Arthur Hoi Hin Cheng² and Rae Yeung⁴, ¹The Hospital of Sick Children,Department of Paediatrics, University of Toronto, Division of Rheumatology, Toronto, ON, Canada, ²The Hospital of Sick Children, Department of Paediatrics, University of Toronto, Division of Rheumatology; Cell Biology Program, The Hospital for Sick Children Research Institute, Toronto, ON, Canada, ³The Hospital for Sick Children, Toronto, ON, Canada, ⁴The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Background/Purpose: Multisystem Inflammatory Syndrome in Children (MIS-C) is a serious complication associated with COVID-19, presenting as a hyperinflammatory disorder characterized by fever and multiorgan dysfunction.…
Abstract Number: 037 • 2023 Pediatric Rheumatology Symposium
Transcriptional Analysis of CD14+ Monocytes During Macrophage Activation Syndrome Highlights Role for Interferons and RNA Sensing in Monocytes
Susan Canny¹, Hannah DeBerg², Griffin Gessay², Ailing Lu³, Mary Eckert¹, Andrea La Bella⁴, Susan Shenoi⁵, Joyce Hui-Yuen⁶, Betsy Barnes⁷ and Jessica Hamerman², ¹Seattle Children's Hospital, Seattle, WA, ²Benaroya Research Institute, Seattle, WA, ³Feinstein Institutes for Medical Research, Manhasset, NY, ⁴Northwell Health, Cohen Children's Medical Center, New Hyde Park, NY, ⁵Seattle Children's Hospital, Seattle, WA, ⁶Cohen Children's Medical Center, Northwell Health, Lake Success, New York; Center for Autoimmune, Musculoskeletal, and Hematopoietic Diseases Research, Feinstein Institutes for Medical Research, Northwell Health, Manhasset, NY, ⁷Center for Autoimmune, Musculoskeletal, and Hematopoietic Diseases Research, Feinstein Institutes for Medical Research, Northwell Health, Manhasset, NY
Background/Purpose: Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (HLH), is a potentially fatal complication of rheumatic diseases. MAS is a dysfunctional hyperinflammatory…
Abstract Number: 038 • 2023 Pediatric Rheumatology Symposium
HLA-DRB1*15 Alleles in Systemic Juvenile Idiopathic Arthritis with Lung Disease and Macrophage Activation Syndrome in Italy
Claudia Bracaglia¹, Manuela Pardeo¹, Maria Troiano², Giuseppe Testa², Ivan Caiello¹, Arianna De Matteis³, Matteo Trevisan¹, Franco Locatelli⁴, Marco Andreani² and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ²Laboratory of Transplant Immunogenetics, Department of Haematology/Oncology, Cell and Gene, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Lazio, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Roma, Italy, ⁴Ospedale Pediatrico Bambino Gesù, Department of Hematology/Oncology, Cell and Gene Therapy, Roma, Italy
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is characterized by unique clinical features and it is considered as a polygenic autoinflammatory disease. Macrophage activation syndrome (MAS)…
Abstract Number: 075 • 2023 Pediatric Rheumatology Symposium
Emapalumab Treatment Followed by Hematopoietic Stem Cell Transplantation in Systemic Juvenile Idiopathic Arthritis Complicated by Recurrent Macrophage Activation Syndrome
Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci², Simona Riccio², Francesco Quagliarella³, Ivan Caiello², Giusi Prencipe², Pietro Merli³, Franco Locatelli³ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Roma, Italy, ³Ospedale Pediatrico Bambino Gesù, Department of Hematology/Oncology, Cell and Gene Therapy, Roma, Italy
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of different rheumatic diseases, particularly of systemic juvenile idiopathic arthritis (sJIA).Methods: We report the case of…
Abstract Number: 0004 • ACR Convergence 2022
mTORC1 Drives the Spectrum of Pathology in Systemic JIA and Macrophage Activation Syndrome
zhengping huang¹, xiaomeng You², Liang Chen³, Yan Du³, Kailey Brodeur³, Qiang Wang³, David Sykes⁴, Margaret Chang⁵, Julia Charles², Peter Nigrovic⁵ and Pui Lee⁵, ¹Guangdong Second Provincial Hospital, Guangzhou, China, ²Brigham and Women's Hospital, Boston, MA, ³Boston Children's Hospital, Boston, MA, ⁴Massachusetts General Hospital, Boston, MA, ⁵Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a severe inflammatory syndrome characterized by fever, skin rash and arthritis. A subset of patients with sJIA develop…

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