Abstracts tagged "IL-1/IL-18"

Abstract Number: 925 • 2018 ACR/ARHP Annual Meeting
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: 982 • 2018 ACR/ARHP Annual Meeting
In the Presence of IL-18, IL-10 but Not IL-6 Induces IFN-γ Production and the Surface Expression of TRAIL on NK Cells
Kojiro Sato, Yoshimi Aizaki, Hiroaki Yazawa and Toshihide Mimura, Department of Rheumatology and Applied Immunology, Faculty of Medicine, Saitama Medical University, Saitama, Japan
Background/Purpose: Adult-onset Still’s disease (AOSD) is a systemic inflammatory disease, the cause of which is largely unknown. AOSD has been recently classified as one of…
Abstract Number: 923 • 2018 ACR/ARHP Annual Meeting
Neutrophils from Children with Systemic JIA Exhibit Persistent Proinflammatory Activation Despite Long-Standing Clinically Inactive Disease
Rachel Brown¹, Maggie Henderlight¹, Thuy Do¹, Shima Yasin², Monica DeLay¹, Alexei A. Grom^3,4 and Grant Schulert^2,5, ¹Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Rheumatology, Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ³Pediatrics, University of Cincinnati, CINCINNATI, OH, ⁴Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Pediatrics, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a chronic childhood arthropathy with features of autoinflammation. New-onset SJIA is associated with expansion and activation of neutrophils…
Abstract Number: 938 • 2017 ACR/ARHP Annual Meeting
A Germline Macrophage Activation Syndrome-Associated Nlrc4 Mutation Causes Chronic, Systemic, Non-Hematopoietic IL-18 Elevation and Intestinal MHC-II Upregulation
Eric Weiss¹, Corinne Schneider² and Scott Canna³, ¹RK Mellon Institute, Children’s Hospital of Pittsburgh, Pittsburgh, PA, ²Pediatrics, Children's Hospital Pittsburgh, Pittsburgh, PA, ³NIAMS, National Institutes of Health, Bethesda, MD
Background/Purpose: Patients prone to the development of Macrophage Activation Syndrome (MAS) can have extreme and often chronic elevation in the pro-inflammatory cytokine interleukin-18 (IL-18). In…
Abstract Number: 1895 • 2017 ACR/ARHP Annual Meeting
Serum Interleukin 18 As a Biomarker for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome and Use of Recombinant Human IL-18 BP in a Patient with Refractory Disease
Shima Yasin¹, Rachel Brown¹, Ndate Fall², Krista Solomon¹, Scott Canna³, Charlotte Girard⁴, Cem Gabay⁵, Eduardo Schiffrin⁶, Andrew Sleight⁶, Alexei A. Grom⁷ and Grant Schulert⁸, ¹Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, ³NIAMS, National Institutes of Health, Bethesda, MD, ⁴Division of Rheumatology, Department of Internal Medicine Specialties, University Hospital of Geneva, Geneva, Switzerland, ⁵SCQM, Geneva, Switzerland, Geneva, Switzerland, ⁶AB2 Bio, Lausanne, Switzerland, ⁷Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States Minor Outlying Islands, ⁸Pediatric Rheumatology, Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis is an autoinflammatory childhood arthritis with prominent innate immune activity. Macrophage activation syndrome is a severe and potentially fatal complication…
Abstract Number: 1896 • 2017 ACR/ARHP Annual Meeting
IL-18 As a Diagnostic Biomarker, Differentiating Systemic JIA from Acute Leukaemia, Severe Bacterial Infections and Other Auto-Immune Disorders
Arjen Leek¹, Nienke Ter Haar², Valerie De Haas³, Ayman El Idrissi¹, Judith Wienke¹, Sytze de Roock⁴, Dirk Holzinger⁵, Wilco de Jager⁶, Jorg van Loosdregt⁷ and Sebastiaan Vastert^4,8, ¹Pediatric Rheumatology and Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ²Department of Pediatric Rheumatology and immunology, University Medical Center Utrecht, Utrecht, Netherlands, ³DCOG Laboratory, SKION, Den Haag, Netherlands, ⁴Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁵Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ⁶Dept Immunology, UMC Utrecht, Utrecht, Netherlands, ⁷Laboratory for Translational immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁸Division of Pediatric Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands
Background/Purpose: Systemic onset Juvenile Idiopathic Arthritis (sJIA) is a disease characterized by systemic inflammation in addition to arthritis and it’s diagnosis currently still depends on…
Abstract Number: 2772 • 2017 ACR/ARHP Annual Meeting
Unbound IL-18 Distinguishes Human Macrophage Activation Syndrome from Familial Hemophagocytic Lymphohistiocytosis and Affects Innate Versus Adaptive Murine Lymphocytes Differently
Paul Tsoukas¹, Eric Weiss², Dirk Holzinger³, Charlotte Girard⁴, Dirk Foell⁵, Alexei A. Grom⁶, Sandra Ammann⁷, Stephan Ehl⁷, Eduardo Schiffrin⁸, Adriana Almeida de Jesus⁹, Raphaela Goldbach-Mansky⁹, Cem Gabay¹⁰ and Scott Canna¹¹, ¹Pediatric Rheumatology, Children’s Hospital of Pittsburgh, Pittsburgh, PA, ²RK Mellon Institute, Children’s Hospital of Pittsburgh, Pittsburgh, PA, ³Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ⁴Division of Rheumatology, Department of Internal Medicine Specialties, University Hospital of Geneva, Geneva, Switzerland, ⁵University of Muenster, Muenster, Germany, ⁶Pediatric Rheumatology, Cincinnati Children’s Hospital, Cincinnati, OH, ⁷Center for Chronic Immunodeficiency, Faculty of Medicine, University of Freiburg, Freiburg, Germany, ⁸AB2 Bio, Lausanne, Switzerland, ⁹Translational Autoinflammatory Disease Studies (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, ¹⁰SCQM, Geneva, Switzerland, Geneva, Switzerland, ¹¹Richard King Mellon Foundation Institute for Pediatric Research, Children's Hospital of Pittsburgh, Pittsburrgh, PA
Background/Purpose: Persistently and extremely elevated serum IL-18 has been associated with Macrophage Activation Syndrome (MAS). Chronic IL-18 is hypothesized to contribute to excessive interferon (IFN)-g…
Abstract Number: 142 • 2017 Pediatric Rheumatology Symposium
Neutrophils and monocytes in the early inflammatory cascade of systemic onset Juvenile Idiopathic Arthritis
Nienke M. ter Haar¹, Wilco de Jager², Rianne C. Scholman¹, Jenny Meerding¹, Bas Vastert^3,4 and Sytze de Roock⁴, ¹Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ²Dept Immunology, UMC Utrecht, Utrecht, Netherlands, ³Division of Pediatric Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands, ⁴Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands
Background/Purpose: Systemic onset Juvenile Idiopathic Artritis (sJIA) is an acquired systemic autoinflammatory disease characterized by spiking fever, arthritis and skin rash. Patients display high inflammatory…
Abstract Number: 31 • 2017 Pediatric Rheumatology Symposium
Predicting therapy response to IL-1 blockade in systemic JIA: a biomarker search
Nienke M. ter Haar¹, Rianne C. Scholman¹, Wilco de Jager², Nadia Ryter³, Ariane de Ganck⁴, Dirk Foell⁵, Sytze de Roock⁶ and Bas Vastert⁷, ¹Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ²Dept Immunology, UMC Utrecht, Utrecht, Netherlands, ³BÜHLMANN Laboratories AG, Basel, Switzerland, ⁴Biogazelle NV, Zwijnaarde, Belgium, ⁵Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ⁶Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁷Division of Pediatric Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands
Background/Purpose: Systemic onset juvenile idiopathic arthritis (sJIA) is an autoinflammatory disease, characterized by fever, rash and arthritis. The IL-1 and IL-6 pathway are crucial in…
Abstract Number: 251 • 2016 ACR/ARHP Annual Meeting
Persistent Pruritic Skin Lesions with Dyskeratotic Cells in Upper Layer of Epidermis Are Specific and Associated with High Levels of Serum IL-18 in Adult-Onset Still’s Disease
Natsuki Maeda^1,2, Yoshinori Taniguchi³, Kimiko Nakajima⁴, Yoshiko Shimamura⁵, Hirofumi Nishikawa⁶, Shuichi Nakayama⁴, Shigetoshi Sano⁴, Shimpei Fujimoto⁶ and Yoshio Terada⁵, ¹Endocrinology,Metabolism,Nephrology and Rheumatology, Kochi Medical School, Nankoku, Japan, ²Dermatology, Kochi Medical School, Nankoku, Japan, ³Endocrinology, Metabolism,Nephrology and Rheumatology, Kochi University, Kochi, Japan, ⁴Kochi Medical School, Nankoku, Japan, ⁵Kochi University, Nankoku, Japan, ⁶Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School, Nankoku, Japan
Background/Purpose: Adult-onset Still’s disease (AOSD) is an acute and systemic inflammatory disorder that is characterized by high spiking fever, evanescent rash, arthralgia/arthritis and hyperferritinemia. However,…
Abstract Number: 663 • 2016 ACR/ARHP Annual Meeting
Cytokine and Chemokine Levels in Serum and Tears As Disease Activity Biomarkers in Patients with Primary Sjögren´s Syndrome
Angel Alejandro Castillo-Ortiz¹, Rosa Elda Barbosa-Cobos², Lizbeth Becerril-Mendoza³, Gustavo Lugo-Zamudio⁴, Virgilio Lima-Gómez⁴, Adan Moreno-Eutimio⁵, Nayeli Nieto-Velázquez⁵ and Angel Tzec-Pérez⁵, ¹Rheumatology, Hospital Juárez de México, Mérida, Mexico, ²Rheumatology, Hospital Juárez de México, Mexico, Mexico, ³Hospital Juárez de México, Mexico, Mexico, ⁴Hospital Juárez de México, México city, Mexico, ⁵Hospital Juárez de México, Mexico city, Mexico
Background/Purpose: Evidence suggests that levels of different cytokines in primary Sjögren´s syndrome (pSS) are associated with cell infiltration degree within lacrimal and salivary glands and…
Abstract Number: 1985 • 2016 ACR/ARHP Annual Meeting
IL-18 Elevation in Macrophage Activation Syndrome: Human Evidence for a Chronic Set-Point and Murine Evidence for a Non-Hematopoietic Source
Zeshan Tariq¹, Eric Weiss², Wendy Goodspeed³, Raphaela Goldbach-Mansky⁴ and Scott Canna², ¹Autoinflammatory Pathogenesis Unit, NIAMS/NIH, Bethesd, MD, ²Autoinflammatory Pathogenesis Unit, NIAMS/NIH, Bethesda, MD, ³Office of the Clinical Director, NIAMS/NIH, Bethesda, MD, ⁴Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD
Background/Purpose: Macrophage Activation Syndrome (MAS) is a life-threatening sepsis-like condition complicating many systemic JIA (sJIA) and Adult-Onset Stills Disease (AOSD) patients. We recently identified that…
Abstract Number: 253 • 2015 ACR/ARHP Annual Meeting
Multiple Serum Cytokine Profiling to Identify Specific Molecular Networks in Attacks of Familial Mediterranean Fever
Tomohiro Koga¹, Kiyoshi Migita², Shuntaro Sato³, Masataka Umeda⁴, Shoichi Fukui⁵, Ayako Nishino⁴, Shinya Kawashiri⁶, Naoki Iwamoto⁵, Kunihiro Ichinose⁵, Mami Tamai⁵, Hideki Nakamura⁵, Tomoki Origuchi⁷, Yukitaka Ueki⁸, Kazunaga Agematsu⁹, Katsumi Eguchi¹⁰ and Atsushi Kawakami⁴, ¹Departments of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ²Department of Rheumatology and Clinical Research Center, Nagasaki Medical Center, Omura, Japan, ³Clinical Research Center, Nagasaki University Hospital, Nagasaki, Japan, ⁴Department of Immunology and Rheumatology, Nagasaki University, Nagasaki, Japan, ⁵Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ⁶Department of Public Health, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, ⁷Department of Rehabilitation Sciences, Nagasaki University, Nagasaki, Japan, ⁸Rheumatic and Collagen Disease Center, Sasebo Chuo Hospital, Sasebo, Japan, ⁹Department of Infectious Immunology, Shinshu University, Graduate School of Medicine, Shinshu, Japan, ¹⁰Department of Rheumatology, Sasebo Chuo Hospital, Sasebo, Japan
Background/Purpose: Familial mediterranean fever (FMF) is caused by a number of mutations of the MEFV gene, coding for a protein named pyrin that acts as…
Abstract Number: 1458 • 2015 ACR/ARHP Annual Meeting
Association of Chronic, Extreme Elevation of Serum IL-18 with the Development of Macrophage Activation Syndrome in a Cohort of Autoinflammatory Disease Patients: A Potential Diagnostic Biomarker?
Scott Canna¹, Adriana Almeida de Jesus², Yan Huang³, Sushanth Gouni¹, Guangpu Shi⁴, Igal Gery⁴ and Raphaela Goldbach-Mansky³, ¹Molecular Immunology and Inflammation Branch, NIAMS/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ³Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ⁴National Eye Institute/NIH, Bethesda, MD
Background/Purpose: Macrophage Activation Syndrome (MAS) is a life-threatening systemic inflammatory syndrome that complicates several rheumatic diseases. Current MAS-related serum biomarkers (ferritin, neopterin, CD163, and CD25)…
Abstract Number: 1815 • 2014 ACR/ARHP Annual Meeting
STAT3-Mediated Regulation of Mitochondrial Membrane Potential Is Critical for NLRP3 Inflammasome Activation
Jehad H. Edwan¹, Raphaela Goldbach-Mansky² and Robert A. Colbert³, ¹NIAMS NIH, Bethesda, MD, ²NIAMS, NIH Building 10 Room 6D47B, Bethesda, MD, ³NIAMS/NIH, Bethesda, MD
Background/Purpose: Self-activating mutations in NLRP3 cause a spectrum of autoinflammatory diseases known as cryopyrin-associated periodic syndromes (CAPS). NLRP3 is a key component of a multiprotein…

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