Abstracts tagged "macrophage activation syndrome"

Abstract Number: 811 • 2019 ACR/ARP Annual Meeting
Monomethyl Fumarate as a Novel Therapy for Macrophage Activation Syndrome: Mechanism of Action in an Animal Model
Chhanda Biswas ¹, Thomas Burn ¹, Niansheng Chu ¹ and Edward Behrens¹, ¹The Children's Hospital of Philadelphia, Philadelphia
Background/Purpose: Macrophage activation syndrome (MAS) is a deadly systemic inflammatory condition marked by an increase in enzymes of iron metabolism including ferritin and heme-oxygenase 1…
Abstract Number: 813 • 2019 ACR/ARP Annual Meeting
Monocyte and Macrophage Transcriptional Phenotypes in Systemic Juvenile Idiopathic Arthritis Reveal TRIM8 as a Mediator of IFNγ Hyperresponsiveness and Risk for Macrophage Activation Syndrome
Grant Schulert¹, Thuy Do ¹, Sanjeev Dhakal ¹, Ndate Fall ², Mario Medvedovich ³, Sherry Thornton ¹, Nathan salomonis ² and Alexei A. Grom ¹, ¹Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³University of Cincinnati College of Medicine, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a severe and distinct subtype of childhood arthritis. Children with SJIA are at risk for macrophage activation syndrome…
Abstract Number: 916 • 2019 ACR/ARP Annual Meeting
Development and Initial Validation of the MS Score for Diagnosis of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis
Francesca Minoia¹, Francesca Bovis ², Sergio Davì ³, AnnaCarin Horne ⁴, Michel Fischbach ⁵, Michael Frosch ⁶, Adam Huber ⁷, Marija Jelusic ⁸, Sujata Sawhney ⁹, Deborah McCurdy ¹⁰, Clovis Artur Silva ¹¹, Donato Rigante ¹², Erbil Unsal ¹³, Nicolino Ruperto ¹⁴, Alberto Martini ¹⁵, Randy Cron ¹⁶ and Angelo Ravelli ¹⁵, ¹Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Lombardia, Italy, ²Università degli Studi di Genova, Genoa, Italy, ³IRCCS Istituto Giannina Gaslini, Genova, Italy, ⁴Karolinska University Hospital, Stockholm, Sweden, ⁵Hopital Universitaire Hautepierre, Strasbourg, France, ⁶School of Medicine Witten/Herdecke University, Datteln, Germany, ⁷IWK Health Centre, Halifax, NS, Canada, ⁸University of Zagreb, Zagreb, Croatia, ⁹Sir Ganga Ram Hospital, New Delhi, India, ¹⁰University of California, MDCC 12-430, 10833 Le Conte Ave, Los Angeles, 90095, CA, USA, Los Angeles, ¹¹Childrens’ Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil, ¹²Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ¹³Dokuz Eylul University, School of Medicie, Izmir, Turkey, ¹⁴Paediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy, ¹⁵IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy, ¹⁶University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Macrophage activation syndrome (MAS) is the most severe complication of systemic juvenile idiopathic arthritis (sJIA) and its adult equivalent, adult-onset Still’s disease (AOSD). Because…
Abstract Number: 917 • 2019 ACR/ARP Annual Meeting
Systemic Juvenile Idiopathic Arthritis-Lung Disease: Characterization and Risk Factors
Grant Schulert¹, Shima Yasin ², Brenna Carey ³, Claudia Chalk ³, Thuy Do ¹, Andrew Schapiro ³, Ammar Husami ³, Allen Watts ³, Hermine Brunner ⁴, Jennifer Huggins ³, Elizabeth Mellins ⁵, Esi Morgan ³, Tracy Ting ³, Bruce Trapnell ⁶, Katheryn Wikenheiser-Brokamp ³, Christopher towe ³ and Alexei A. Grom ¹, ¹Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Pediatric Rheumatology Collaborative Study Group (PRCSG), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Stanford University, Palo Alto, ⁶Cincinnati Children's Hospital, Cincinnati
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) is associated with a recently recognized albeit poorly defined and characterized lung disease (LD). Our objective is to describe…
Abstract Number: 918 • 2019 ACR/ARP Annual Meeting
Multiplex Serum Analysis Identifies Potential Biomarkers of Systemic Juvenile Idiopathic Arthritis, Macrophage Activation Syndrome, and Associated Pulmonary Alveolar Proteinosis: Evidence for Independently-regulated Hyperinflammatory and Eosinophilic Inflammation
Guangbo Chen ¹, Grant Schulert ², Adriana De Jesus ³, Vivian Saper ⁴, Corinne Schneider ⁵, Bruce Trapnell ⁶, Alexei A. Grom ², Raphaela Goldbach-Mansky ⁷, Elizabeth Mellins ⁴, Purvesh Khatri ⁸ and Scott Canna⁹, ¹Stanford University, Palo Alto, CA, ²Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³NIH/NIAID/TADS, Bethesda, MD, ⁴Stanford University, Palo Alto, ⁵University of Pittsburgh, Pittsburgh, ⁶Cincinnati Children's Hospital, Cincinnati, ⁷Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD, ⁸Institute for Immunity, Transplantation and Infection, Stanford University, Palo Alto, ⁹UPMC Children's Hospital of Pittsburgh, Pittsburgh
Background/Purpose: Recent experiences suggest a disturbing epidemic of digital clubbing and insidious, Pulmonary Alveolar Proteinosis (PAP)-like lung disease occurring in patients with Systemic Juvenile Idiopathic…
Abstract Number: 1926 • 2019 ACR/ARP Annual Meeting
Characterization of DOCK8 as a Novel Gene Associated with Macrophage Activation Syndrome
Mingce Zhang ¹, Remy Cron ², Devin Absher ³, Courtney Crayne ², Prescott Atkinson ², W. Winn Chatham ² and Randy Cron¹, ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Alabama at Birmingham, Birmingham, ³HudsonAlpha Institute for Biotechnology, Huntsville, AL
Background/Purpose: Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis (HLH), is a life threatening condition that commonly presents with unremitting fever and shock…
Abstract Number: 2697 • 2019 ACR/ARP Annual Meeting
Risk Score of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Simone Carbogno ¹, Denise Pires Marafon ², Giulia Marucci ³, Manuela Pardeo ³, Antonella Insalaco ³, Virginia Messia ³, Emanuela Sacco ⁴, Ferhat Demir ⁵, Betul Sozeri ⁵, Natasia Čekada ⁶, Marija Jelusic ⁷, Olga Vougiouka ⁸, Mikhail Kostik ⁹, Alenka Gagro ¹⁰, Christoph Kessel ¹¹, Francesca Minoia ¹², Fabrizio De Benedetti ¹³ and Claudia Bracaglia¹⁴, ¹Pediatric Area, University of Milan, Milan, Italy, Milan, Italy, ²Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁴Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, ⁵University of Health Sciences , Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology, Istanbul, Turkey, Istanbul, Turkey, ⁶Division of Paediatric Rheumatology and Immunology, University of Zagreb, Zagreb, Croatia, Zagreb, Croatia, ⁷University of Zagreb, Zagreb, Croatia, ⁸P. & A. Kyriakou Children’s Hospital, Athens, Greece, Athens, Greece, ⁹Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, Saint Petersburg, Russia, ¹⁰Children's Hospital Zagreb, Zagreb, Croatia, Zagreb, Croatia, ¹¹University Hospital Muenster, Muenster, Germany, ¹²Clinica De Marchi Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, ¹³Bambino Gesù Children’s Hospital, Rome, Italy, ¹⁴Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of rheumatic diseases in childhood, particularly of systemic Juvenile Idiopathic Arthritis (sJIA), occurring in approximately…
Abstract Number: 1327 • 2018 ACR/ARHP Annual Meeting
The Effect of Etoposide on Inducing Remission in Refractory Adult-Onset Still’s Disease: A Retrospective, Single-Center Study
Haiting Wang¹, Xiaodong Wang¹, Ting Li¹ and Shuang Ye², ¹Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiao Tong University School of Medicine, Shanghai, China, ²Rheumatology, Ren Ji Hospital, South Campus, School of Medicine, Shanghai Jiaotong University, Shanghai, China
Background/Purpose: Adult onset Still¡¯s disease (AOSD) is a rare inflammatory disorder of unknown etiology. Macrophage activation syndrome (MAS) is a life-threatening complication and has been…
Abstract Number: 2900 • 2017 ACR/ARHP Annual Meeting
Osteoarthritis-Associated Calcium-Containing Crystals and Biomaterial Microparticles Both Drive M1 Macrophage Polarization in a Syk and MAP Kinase-Dependent Manner
Geraldine M. McCarthy¹, Olwyn Mahon², Sarah O'Hanlon³, Daniel Kelly⁴ and Aisling Dunne⁵, ¹Div of Rheumatology, Mater Misericordiae University Hospital, Dublin, Ireland, ²School of Biochemistry and Immunology,, ,Trinity College Dublin, Dublin, Ireland, ³School of Biochemistry and Immunology, Trinity College Dublin, Dublin, Ireland, ⁴Trinity Center for Bioengineering, ,Trinity College Dublin, Dublin, Ireland, ⁵School of Biochemistry and Immunology, Trinity College Dublin, Dublin 2, Ireland
Background/Purpose: Intra-articular basic calcium phosphate (BCP) crystals are associated with advanced osteoarthritis (OA) andsynovitis. OA-associated synovium exhibits increased numbers of macrophages compared with non-OA joints.…
Abstract Number: 4 • 2017 Pediatric Rheumatology Symposium
Microbiota-Dependent Signals Regulate Inflammatory Myelopoiesis in a Murine Model of Macrophage Activation Syndrome
Lehn K. Weaver¹, Chhanda Biswas¹, Niansheng Chu¹ and Edward M. Behrens², ¹Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, ²Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA
Background/Purpose: Targeting host-microbiota interactions to limit production of pathogenic myeloid cells that fuel chronic inflammatory responses is of therapeutic interest. Recent evidence suggests that this may…
Abstract Number: 187 • 2016 ACR/ARHP Annual Meeting
Commensal Microbiota Tune Systemic Toll-like Receptor-Mediated Inflammatory Responses
Lehn K. Weaver¹, Chhanda Biswas¹ and Edward M. Behrens², ¹Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, ²Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA
Background/Purpose: Although commensal microbiota are thought to contribute to the development of autoimmunity, the cellular and molecular mechanisms connecting changes in gut microbiota to the…
Abstract Number: 2377 • 2016 ACR/ARHP Annual Meeting
Innovative Use of PK and PD to Guide Dose Selection for a Monoclonal Antibody Aimed at Neutralizing the High IFNγ Activity Present in Patients with Macrophage Activation Syndrome (MAS)
Philippe Jacqmin¹, Kathy de Graaf², Maria Ballabio², Robert Nelson², Zoë Johnson², Walter Ferlin², Geneviève Lapeyre², Fabrizio De Benedetti³ and Cristina de Min², ¹MnS, Dinant, Belgium, ²NovImmune S.A., Geneva, Switzerland, ³Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy, Rome, Italy
Background/Purpose: Data from an animal model of MAS and the observed high IFNγ and IFNγ-related chemokines (CXCL9, CXCL10) levels in MAS/sJIA patients have prompted the…
Abstract Number: 3005 • 2016 ACR/ARHP Annual Meeting
Development and Initial Validation of the “MH Score”, a New Diagnostic Tool That Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome
Francesca Minoia¹, AnnaCarin Horne², Francesca Bovis¹, Sergio Davì¹, Laura Pagani¹, Graciela Espada³, Gao Yi-Jin⁴, Antonella Insalaco⁵, Kai Lehmberg⁶, Helga Sanner⁷, Susan Shenoi⁸, Sheila Weitzman⁹, Nicolino Ruperto¹⁰, Alberto Martini¹, Randy Q. Cron¹¹ and Angelo Ravelli¹, ¹Istituto Giannina Gaslini, Genoa, Italy, ²Karolinska University Hospital Solna, Stockholm, Sweden, ³Hospital de Ninos Ricardo Gutierrez, Buenos Aires, Argentina, ⁴Children's Hospital of Fudan, Shanghai, China, ⁵Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁶University Medical Center, Hamburg, Germany, ⁷Norwegian National Advisory Unit on Rheumatic Diseases in Children and Adolescents, Oslo University Hospital, Rikshospitalet, Oslo, Norway, Oslo, Norway, ⁸Seattle Children's Hospital, Seattle, WA, ⁹The Hospital for Sick Children, Toronto, ON, Canada, ¹⁰Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy, ¹¹University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: It is common view that macrophage activation syndrome (MAS) bears close similarities with primary hemophagocytic lymphohistiocytosis (pHLH). The resemblance of their clinical and laboratory…
Abstract Number: 404 • 2015 ACR/ARHP Annual Meeting
Long-Term Outcomes of Macrophage Activation Syndrome in Childhood-Onset Systemic Lupus Erythematosus
Roberto Ezequiel Borgia¹, Maya Gerstein¹, Deborah M. Levy², Earl Silverman² and Linda T Hiraki², ¹Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ²The Hospital for Sick Children, Toronto, ON, Canada
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening inflammatory complication of childhood-onset systemic lupus erythematosus (cSLE). There are few reports of long-term outcomes of MAS complicating cSLE.…
Abstract Number: 2799 • 2014 ACR/ARHP Annual Meeting
The Differential Impact of Obesity on the Pathogenesis of RA or Preclinical Models Is Contingent on the Disease Status
Zhenlong Chen¹, Seung-jae Kim¹, Abdul Essani², Michael V. Volin³, Suncica Volkov¹, William Swedler⁴, Shiva Arami², Giamila Fantuzzi², Nadera J. Sweiss⁵ and Shiva Shahrara¹, ¹Medicine/Rheumatology, University of Illinois at Chicago, Chicago, IL, ²University of Illinois at Chicago, Chicago, IL, ³Department of Microbiology and Immunology, Chicago College of Osteopathic Medicine Midwestern University, Downers Grove, IL, ⁴Section of Rheumatology, University of Illinois at Chicago, Chicago, IL, ⁵internal medicine section of rheumatology, University of Illinois at Chicago, Chicago, IL
Background/Purpose: Studies were performed to determine the significance of obesity in the pathogenesis of rheumatoid arthritis (RA) and experimental arthritis models. Methods: Chronic and acute…

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