Abstracts tagged "macrophage activation syndrome"

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…
Abstract Number: 2199 • 2014 ACR/ARHP Annual Meeting
Intravenous Immunoglobulin Therapy for Secondary Hemophagocytic Lymphohistiocytosis : A Retrospective Study of 46 Patients
Bertrand Dunogué¹, Magdalena Gerin², Claire Larroche³, Catherine Montagnier-Petrissans⁴, Loïc Guillevin⁵ and Luc Mouthon⁵, ¹Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France, ²Internal Medicine, Hôpital Jean Verdier, Bondy, France, ³Internal Medicine, Hôpital Avicenne, Bobigny, France, ⁴Groupe d'Expert AP-HP, Paris, France, ⁵National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France
Background/Purpose Intravenous Immunoglobulins (IVIg) have been reported as giving good results in infectious, but also auto-immune related forms of hemophagocytic lymphohistiocytosis (HLH), but only in…
Abstract Number: 2200 • 2014 ACR/ARHP Annual Meeting
Elevated Serum Ferritin Levels in Adult Inpatients As a Predictor of in-Hospital Mortality and Association with Macrophage Activation Syndrome
Matthew Mullen¹, Marcin Trojanowski², W. Winn Chatham³ and Bita Shakoory³, ¹Rheumatology, University of Alabama at Birmingham, Birmingham, AL, ²Medicine/ Immunology & Rheumatology, University of Alabama at Birmingham, Birmingham, AL, ³University of Alabama at Birmingham, Birmingham, AL
Background/Purpose Macrophage Activation Syndrome (MAS) is a syndrome similar to Familial Hemophagocytic Lymphohistiocytosis (HLH) characterized by increased proliferation and activity of T-cells and macrophages leading…
Abstract Number: 1243 • 2014 ACR/ARHP Annual Meeting
Macrophage Activation Syndrome Complicating Adult Onset Still’s Disease – Single Center Experience and Literature Review
Aleksander Lenert¹ and Qingping Yao², ¹Orthopaedic and Rheumatologic Institute, Dept. of Rheumatic & Immunologic Diseases, Cleveland Clinic, Cleveland, OH, ²Rheumatic and Immunologic Dis, Cleveland Clinic, Cleveland, OH
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening complication typically associated with hematologic malignancies and infections. HLH, also referred to as macrophage activation syndrome (MAS),…
Abstract Number: 2017 • 2013 ACR/ARHP Annual Meeting
Hyper Ferritinemia Suggests a High Frequency Of Macrophage Activation Syndrome In a Large Adult Severe Sepsis Cohort: A Role For Anakinra?
Bita Shakoory¹, Joseph A. Carcillo² and Steven Opal³, ¹Medicine/Rheumatology, Temple University, Philadelphia, PA, ²Pediatrics, University of Pittsburg Medical Center, Pittsburgh, PA, ³Medicine, Brown University, Providence, RI
Background/Purpose: Inreased ferritin levels are protective during infection whereas but very very high level are associated with MAS in rheumatologic disease cTo determine frequency of…

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