Abstracts tagged "Systemic JIA and macrophage activation syndrome"

Abstract Number: 919 • ACR/ARP Annual Meeting 2019
Free Interlukin-18: A New Promising Biomarker for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome
Shima Yasin¹, Emiliana Rodriguez ², Thuy Do ³, Elizabeth Baker ¹, Sanjeev Dhakal ³, Scott Canna ⁴, Cem Gabay ⁵, Alexei A. Grom ³ and Grant Schulert ³, ¹Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, ²Rheumatology, Geneva University Hospitals and University of Geneva, Switzerland, Geneva, Swaziland, ³Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁵Rheumatology, Geneva University Hospitals and University of Geneva, Switzerland, Geneva, Switzerland
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with features of autoinflammation, and is associated with high risk of macrophage activation syndrome (MAS).…
Abstract Number: 925 • ACR/ARHP Annual Meeting 2018
Abortive Viral Infection Becomes Macrophage Activation Syndrome in Mice with Chronically Elevated Interleukin-18: Evidence for Synergy with Cytotoxic Impairment
Paul Tsoukas¹, Corinne Schneider², Lauren Van Der Kraak² and Scott Canna³, ¹Pediatric Rheumatology, Children’s Hospital of Pittsburgh, Pittsburgh, PA, ²RK Mellon Institute for Pediatric Research, University of Pittsburgh/Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, ³RK Mellon Institute for Pediatric Research, University of Pittsburgh/Children's Hospital of Pittsburgh of UPMC, Pittsburrgh, PA
Background/Purpose: Macrophage Activation Syndrome (MAS) and Hemophagocytic lymphohistiocytosis (HLH) are clinically similar life-threatening hyperinflammatory syndromes, often triggered by viral infection. HLH is associated with cytotoxic…
Abstract Number: 2919 • ACR/ARHP Annual Meeting 2018
An Epidemic: Severe Lung Disease in Patients with Systemic Juvenile Idiopathic Arthritis, Risk Factors and Predictors
Shima Yasin¹, Christopher Towe^2,3, Ndate Fall⁴, Alexei Grom^3,4 and Grant Schulert^1,5, ¹Rheumatology, Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²Pulmonology, Division of Pulmonology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ³Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, ⁴Division of Rheumatology, Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Pediatrics, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH
Background/Purpose: There is growing awareness of severe and often fatal chronic lung disease in patients with systemic juvenile idiopathic arthritis (sJIA). However, clinical features and…
Abstract Number: 1897 • ACR/ARHP Annual Meeting 2017
the Ferritin to ESR Ratio: A Simple Measure to Distinguish Macrophage Activation Syndrome from Systemic Arthritis Flare
Esraa M. A. Eloseily^1,2, Francesca Minoia³, Timothy Beukelman², Angelo Ravelli⁴ and Randy Q. Cron², ¹Pediatrics, Assiut University, Assiut, Egypt, ²Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL, ³Istituto Giannina Gaslini, Genoa, Italy, ⁴University of Genova, Genova, Italy
Background/Purpose: Macrophage activation syndrome (MAS) is a life threatening complication of systemic juvenile idiopathic arthritis (sJIA). As MAS often shares clinical and laboratory features with…
Abstract Number: 75 • Pediatric Rheumatology Symposium 2017
Ferritin:ESR, A Predictor of MAS?
Miriah Gillispie¹, Marietta DeGuzman², Mark Gorelik³ and Tiphanie Vogel^4,5, ¹Pediatrics, Department of IAR, Baylor College of Medicine, Houston, TX, ²Pediatric Immunology, Allergy and Rheumatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ³Baylor College of Medicine, San Antonio, TX, ⁴Department of Pediatrics, Division of Immunology, Allergy and Rheumatology and the Center for Human Immunology at Texas Children's Hospital., Baylor College of Medicine, Houston, TX, ⁵Center for Human Immunobiology, Texas Children's Hospital, Houston, TX
Background/Purpose: Systemic onset juvenile idiopathic arthritis (SoJIA) is a rare autoinflammatory disorder comprising only 10% of JIA. Macrophage activation syndrome (MAS), an excessive and uncontrolled…
Abstract Number: 2375 • ACR/ARHP Annual Meeting 2016
Biologic Therapy Modifies Clinical and Laboratory Features of Macrophage Activation Syndrome Associated with Systemic Juvenile Idiopathic Arthritis
Grant Schulert¹, Francesca Minoia², John F. Bohnsack³, Randy Q. Cron⁴, Soah Hashad⁵, Isabelle Koné-Paut⁶, Mikhail Kostik⁷, Daniel J Lovell⁸, Despoina Maritsi⁹, Peter A. Nigrovic¹⁰, Priyankar Pal¹¹, Angelo Ravelli², Masaki Shimizu¹², Valda Stanevicha¹³, Bas Vastert¹⁴, Fabrizio De Benedetti¹⁵ and Alexei Grom¹⁶, ¹Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Istituto Giannina Gaslini, Genoa, Italy, ³Division of Allergy, Immunology and Pediatric Rheumatology, University of Utah, Salt Lake City, UT, ⁴Pediatric Rheumatology, Children's Hospital of Alabama, Birmingham, AL, ⁵Tripoli Children's Hospital, Tripoli, Libya, ⁶Hopital Kremlin Bicetre, University of Paris SUD, Paris, France, ⁷Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, ⁸PRCSG Cincinnati Children's Hospital Medical Center, Cinncinnati, OH, ⁹2nd Department of Academic Pediatrics, Athens Medical School, university of Athens, Athens, Greece, ¹⁰Rheumatology, Immunology, and Allergy, Brigham and Women’s Hospital, Boston, MA, ¹¹Institute of Child Health, Kolkata, India, ¹²Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University, Kanazawa, Japan, ¹³Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, ¹⁴Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Netherlands, ¹⁵Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy, Rome, Italy, ¹⁶Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening episode of hyperinflammation and a substantial cause of morbidity and mortality in pediatric rheumatology. It occurs most…
Abstract Number: 2415 • ACR/ARHP Annual Meeting 2016
Next Generation Sequencing Analysis of Familial Haemophagocytic Lymphohistiocytosis (HLH) Related Genes in Macrophage Activation Syndrome (MAS) and Secondary HLH (secHLH)
Chiara Passarelli¹, Manuela Pardeo², Elisa Pisaneschi¹, Antonio Novelli¹, Fabrizio De Benedetti² and Claudia Bracaglia², ¹Ospedale Pediatrico Bambino Gesù IRCCS, Unit of Medical Genetics, Laboratory of Cytogenetics and Molecular Genetics, Rome, Italy, ²Division of Rheumatology, Ospedale Pediatrico Bambino Gesù IRCCS, Roma, Italy, Rome, Italy
Background/Purpose: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease, particularly of systemic JIA (sJIA). It is currently classified among the secondary forms…
Abstract Number: 1456 • ACR/ARHP Annual Meeting 2015
Macrophage Activation Syndrome and Familial Hemophagocytic Lymphohistiocytosis: Is Their Clinical Phenotype Really Similar?
Francesca Minoia¹, AnnaCarin Horne², Sergio Davì¹, Francesca Bovis¹, Silvia Rosina¹, Kai Lehmberg³, Sheila Weitzman⁴, Antonella Insalaco⁵, Carine Wouters⁶, Susan Shenoi⁷, Graciela Espada⁸, Seza Ozen⁹, Jordi Anton¹⁰, Raju Khubchandani¹¹, Ricardo Russo¹², Nicolino Ruperto¹³, Alberto Martini¹, Randy Q. Cron¹⁴ and Angelo Ravelli¹, ¹Istituto Giannina Gaslini, Genoa, Italy, ²Karolinska University Hospital Solna, Stockholm, Sweden, ³University Medical Center, Hamburg, Germany, ⁴The Hospital for Sick Children, Toronto, ON, Canada, ⁵Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁶University Hospital Gasthuisberg, Leuven, Belgium, ⁷Seattle Children's Hospital, Seattle, WA, ⁸Hospital de Ninos Ricardo Gutierrez, Buenos Aires, Argentina, ⁹Hacettepe University, Ankara, Turkey, ¹⁰Unitat de Reumatologia Pediàtrica, Hospital Sant Joan de Déu, Barcelona, Spain, ¹¹Jaslok Hospital and Research Center, Mumbai, India, ¹²Hospital de Pediatria Garrahan, Buenos Aires, Argentina, ¹³Istituto G. Gaslini, Pediatria II, PRINTO, Genoa, Italy, ¹⁴University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Macrophage activations syndrome (MAS) is a potentially life-threatening complication of systemic juvenile idiopathic arthritis (sJIA). It is common view that sJIA associated MAS bears…
Abstract Number: 2281 • ACR/ARHP Annual Meeting 2014
Dissecting the Heterogeneity of Macrophage Activation Syndrome
Francesca Minoia¹, Sergio Davì¹, AnnaCarin Horne², Francesca Bovis³, Erkan Demirkaya⁴, Alessandro Consolaro⁵, Jonathan Akikusa⁶, Nuray Aktay Ayaz⁷, Patrizia Barone⁸, Bianca Bica⁹, Isabel Bolt¹⁰, Luciana Breda¹¹, Zane Davidsone¹², Carmen De Cunto¹³, Jaime De Inocencio¹⁴, Sandra Enciso¹⁵, Romina Gallizzi¹⁶, Thomas Griffin¹⁷, Teresa Hennon¹⁸, Gerd Horneff¹⁹, Maka Ioseliani²⁰, Michael Jeng²¹, Agneza Marija Kapovic²², Bianca Lattanzi⁵, Jeffrey M Lipton²³, Silvia Magni-Manzoni²⁴, Clarissa Nassif²⁵, Ingrida Rumba-Rozenfelde²⁶, Claudia Saad-Magalhães²⁷, Sulaiman Almayouf²⁸, Wafaa Al-Suwairi²⁹, Kimo C Stine³⁰, Olga Vougiouka³¹, Lehn K. Weaver³², Nicolino Ruperto³³, Alberto Martini^5,34, Randy Q. Cron³⁵ and Angelo Ravelli³⁴, ¹Pediatria II, Istituto Giannina Gaslini, Genova, Italy, ²Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden, ³Pediatria II, PRINTO, PRINTO - Istituto Giannina Gaslini, Genoa, Italy, ⁴Gulhane Military Medical Faculty, Ankara, Turkey, ⁵Istituto Giannina Gaslini, Genova, Italy, ⁶3 West Clinical Offices, Royal, Royal Children's Hospital, Melbourne, Australia, ⁷Bakırkoy Maternity and Childrens Education and Research Hospital, Istanbul, Turkey, ⁸Azienda Policlinico Università di Catania, Catania, Italy, ⁹Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, Brazil, ¹⁰Kinderspital Zuerich, Universitaetskinderklinik, Zurich, Switzerland, ¹¹Department of Paediatrics, University of Chieti G. D'Annunzio, Chieti, Italy, ¹²Children's University Hospital, Riga, Latvia, ¹³Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, ¹⁴Hospital 12 de Octubre, Madrid, Spain, ¹⁵Hospital Infantil de Mexico Federico Gomez in Mexico City, Mexico City, Mexico, ¹⁶University of Messina, Messina, Italy, ¹⁷Pediatrics, Division of Rheumatology, Levine Children's Hospital, Carolinas Medical Ctr, Charlotte, NC, ¹⁸Division of Pediatric Allergy/Immunology/Rheumatol, Women & Children's Hospital of Buffalo, Buffalo, NY, ¹⁹Asklepios Klinik Sankt Augustin, Sankt Augustin, Germany, ²⁰M. Iashvili Children's Central Clinic, Tbilisi, Georgia, ²¹Stanford University, Stanford, CA, ²²Children's Hospital Zagreb, Zagreb, Croatia, ²³Steven and Alexandra Cohen Children's Hospital of New York, New York, NY, ²⁴Ospedale Pediatrico Bambino Gesù, Roma, Italy, ²⁵Hospital das Clínicas da Universidade Federal de Minas Gerais, Belo Horizonte, Brazil, ²⁶University of Latvia, Riga, Latvia, ²⁷Alameda Das Hortensias 868, Botucatu Medical School, Botucatu, Brazil, ²⁸King Faisal Specialist H.,Riyadh, Riyadh, Saudi Arabia, ²⁹Pediatric, King AbdulAziz Medical City, Riyadh, Saudi Arabia, ³⁰Arkansas Children's Hospital, Little Rock, AR, ³¹P. A. Kyriakou Childrens Hospital of Athens University, Athens, Greece, ³²Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, ³³Pediatria II,, Istituto Giannina Gaslini, Genoa, Italy, ³⁴University of Genova, Genova, Italy, ³⁵Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose Macrophage activations syndrome (MAS) occurring in the context of systemic juvenile idiopathic arthritis (sJIA) can pursue a rapidly fatal course. However, the diagnosis of…
Abstract Number: 321 • ACR/ARHP Annual Meeting 2014
Mutations of Familial Hemophagocytic Lymphohistiocytosis (FHL) Related Genes and Abnormalities of Cytotoxicity function tests in Patients with Macrophage Activation Syndrome (MAS) Occurring in Systemic Juvenile Idiopathic Arthritis (sJIA)
Claudia Bracaglia¹, Elena Sieni², Martina Da Ros², Carmela De Fusco³, Concetta Micalizzi⁴, Valentina Cetica², Benedetta Ciambotti², Maria Luisa Coniglio², Antonella Insalaco⁵, Fabrizio De Benedetti Sr.¹ and Maurizio Arico' Sr.⁶, ¹Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, ²Department of Pediatric Hematology-Oncology, Meyer Children’s Hospital, Florence, Italy, ³Department of Pediatric Hematology-Oncology, Pausillipon Children’s Hospital, Naple, Italy, ⁴Department of Pediatric Hematology-Oncology, G. Gaslini Children' s Hospital, Genoa, Italy, ⁵Department of Pediatric Medicine,, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, ⁶Istituto Toscano Tumori (I.T.T.), Florence, Italy
Background/Purpose: MAS is a severe complication of rheumatic diseases, mostly sJIA. Clinical and laboratory features are similar to those of FHL resulting from mutations in…
Abstract Number: 2171 • ACR/ARHP Annual Meeting 2013
Identification Of Best Cutoff Points and Clinical Signs Specific For Early Recognition Of Macrophage Activation Syndrome In Active Systemic Juvenile Idiopathic Arthritis
Mikhail Kostik¹, Margarita Dubko², Ludmila Snegireva¹, Vera Masalova¹, Tatyana Kornishina², Natalya Abramova³, Irina Chikova², Natalya Glebova³, Ekaterina Kuchinskaya³, Eugenia Balbotkina³, Olga Kalshnikova³ and Vyacheslav Chasnyk⁴, ¹Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, ²State Pediatric Medical University, Saint-Petersburg, Russia, ³Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ⁴Hospital Pediatry, State Pediatric Medical University, Saint-Petersburg, Russia
Background/Purpose: Macrophage activation syndrome (MAS) – is a severe life-threatening hematological condition, mostly complicated systemic juvenile idiopathic arthritis (SJIA). Early detection of MAS can lead…