Abstracts tagged "interferons and macrophage activation syndrome"

Abstract Number: 1415 • 2018 ACR/ARHP Annual Meeting
Emapalumab, an Anti-Interferon Gamma Monoclonal Antibody in Two Patients with NLRC4-Related Disease and Severe Hemophagocytic Lymphohistiocytosis (HLH)
Claudia Bracaglia¹, Giusi Prencipe¹, Antonella Insalaco¹, Ivan Caiello², Giulia Marucci¹, Raffaele Pecoraro³, Manuela Pardeo¹, Pavla Dolezalova⁴, Sarka Fingerhutova⁴, Maria Ballabio⁵, Cristina de Min⁵ and Fabrizio De Benedetti⁶, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ³Pediatric Department, La Sapienza University of Rome, Rome, Italy, ⁴Paediatric Rheumatology Unit, General University Hospital in Prague and 1st Faculty of Medicine, Charles University, General University Hospital in Prague and 1st Faculty of Medicine, Prague, Czech Republic, ⁵NovImmune S.A., Geneva, Switzerland, ⁶IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Background/Purpose: Interferon gamma (IFNγ) plays a pathogenic role in primary and secondary HLH. An ongoing phase 2/3 trial with emapalumab in primary HLH provides encouraging…
Abstract Number: 2332 • 2017 ACR/ARHP Annual Meeting
Biomarkers for the Diagnosis and the Identification of Risk of Macrophage Activation Syndrome (MAS) in Systemic Juvenile Idiopathic Arthritis (sJIA)
Claudia Bracaglia¹, Denise Pires Marafon², Ivan Caiello³, Kathy de Graaf⁴, Maria Ballabio⁴, Walter Ferlin⁴, Sergio Davì⁵, Grant Schulert⁶, Angelo Ravelli⁷, Alexei A. Grom⁸, Robert Nelson⁴, Cristina de Min⁴ and Fabrizio De Benedetti², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ²Division of Rheumatology, IRCCS Bambino Gesù Children's Hospital, Rome, Rome, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁴NovImmune S.A., Geneva, Switzerland, ⁵University of Genova, IRCCS Istituto Giannina Gaslini, Genoa, Italy, ⁶Pediatric Rheumatology, Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁷University of Genova, IRCCS Istituto Giannina Gaslini, Genova, Italy, ⁸Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States Minor Outlying Islands
Background/Purpose: We have recently reported high levels of IFNγ and of the IFNγ-related chemokines, (CXCL9 and CXCL10) in patients with MAS (1). Methods: Circulating levels…
Abstract Number: 3096 • 2015 ACR/ARHP Annual Meeting
Interferon-Gamma (IFNg) in Macrophage Activation Syndrome (MAS): CXCL9 Levels As a Biomarker for IFNg Production in MAS
Claudia Bracaglia¹, Denise Pires Marafon², Ivan Caiello¹, Kathy De Graaf³, Florence Guilhot⁴, Walter Ferlin⁴, Sergio Davì⁵, Grant Schulert⁶, Angelo Ravelli⁵, Alexei A. Grom⁷, Robert Nelson⁴, Cristina de Min⁴ and Fabrizio De Benedetti¹, ¹Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, ²Pediatric Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ³Novimmune S.A., Geneva, Switzerland, ⁴NovImmune S.A., Geneva, Switzerland, ⁵Istituto Giannina Gaslini and University of Genova, Genova, Italy, ⁶Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁷Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: A vast body of evidence in animals and humans points to a pivotal pathogenic role of IFNγ, in primary HLH. The role of IFNg…
Abstract Number: 1901 • 2014 ACR/ARHP Annual Meeting
Interferon-γ (IFNγ) in Macrophage Activation Syndrome (MAS) Associated with Systemic Juvenile Idiopathic Arthritis (sJIA). High Levels in Patients and a Role in a Murine MAS Model
Claudia Bracaglia¹, Ivan Caiello¹, Kathy De Graaf², Giovanni D'Ario², Florence Guilhot², Walter Ferlin², Lidia Meli¹, Giusi Prencipe¹, Sergio Davì³, Grant Schulert⁴, Angelo Ravelli⁵, Alexei Grom⁶, Cristina De Min² and Fabrizio De Benedetti Sr.¹, ¹Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, ²Novimmmune S.A., Plan-Les-Ouates, Geneva, Switzerland, ³Pediatria II, Istituto Giannina Gaslini, Genova, Italy, ⁴Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵Istituto Giannina Gaslini and University of Genova, Genova, Italy, ⁶Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: IFNγ is the pivotal mediator in murine models of primary HLH. Given the similarities between primary and secondary (sHLH), including MAS, we analyzed IFNγ…

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