Abstracts tagged "Autoinflammatory Disease"

Abstract Number: 1297 • 2013 ACR/ARHP Annual Meeting
Human IL-1alpha Conditional Transgenic Mice Mimic Autoinflammatory Syndromes In Human
Hiroya Kanagawa¹, Yasuo Niki², Yoshiaki Toyama³ and Takeshi Miyamoto⁴, ¹Department of Orthopaedic Surgery, Keio University, Shinjuku-ku, Tokyo, Japan, ²Orthopaedic Surgery, Department of Orthopaedic Surgery, Keio University, Tokyo, Japan, ³Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, Japan, ⁴Department of Orthopaedic Surgery, Keio University, Shinjuku-ku,Tokyo, Japan
Background/Purpose: Recently, IL-1 is implicated in the pathogenesis of autoinflammatory syndrome, which characterized by rash, fever, systemic polyarthritis and neurological comorbidity. Recently, IL-1 becomes noticed…
Abstract Number: 1203 • 2013 ACR/ARHP Annual Meeting
An Escalating Dose Of Anakinra In Patients With Autoinflammatory Disease Is a Safe and Reasonable Therapeutic Option
Amanda K. Ombrello¹, Karyl Barron², Patrycja M. Hoffmann³, Anne Jones³, Deborah Stone⁴ and Daniel L. Kastner⁵, ¹National Human Genome Research Institute,National Institute of Health, Bethesda, MD, ²Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ³Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁴National Human Genome Research Institute, Bethesda, MD, ⁵Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, MD
Background/Purpose: The autoinflammatory diseases are diseases in which there is seemingly unprovoked stimulation of the innate immune system. The enhanced release of cytokines in…
Abstract Number: 1205 • 2013 ACR/ARHP Annual Meeting
Effectiveness Of Canakinumab In a Cryopyrin-Associated Periodic Syndrome Cohort.A Single Center Experience
Virginia Moreira-Navarrete¹, Francisco Javier Toyos Saenz de Miera², Carmen Vargas Lebrón³ and F. Navarro Sarabia⁴, ¹Rheumatology, University Hospital Virgen Macarena, Sevilla, Spain, ²Hospital Virgen de la Macarena, Sevilla, Spain, ³Hospital Virgen Macarena, SEVILLA, Spain, ⁴Rheumatologist Service, Hospital Universitario Virgen Macarena, Sevilla, Spain
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) comprises a group of rare, but severe, autoinflammatory diseases, characterized by urticaria, periodic fever, central nervous system inflammation, arthropathy, and…
Abstract Number: 1206 • 2013 ACR/ARHP Annual Meeting
Canakinumab Treatment Regimens In CAPS Patients
Ferdinand Hofer¹, Theresa Endres¹, Birgit Kortus-Goetze², Norbert Blank³, Elisabeth Weissbarth-Riedel⁴, Catharina Schuetz⁵, Tilmann Kallinich⁶, Karoline Krause⁷, Christoph Rietschel⁸, Gerd Horneff⁹ and Jasmin B. Kuemmerle-Deschner¹⁰, ¹Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, ²Klinik für Innere Medizin, Klinikum der Philipps-Universität Marburg, Marburg, Germany, ³University of Heidelberg, Heidelberg, Germany, ⁴Kinderrheumatologische Ambulanz, Universitätsklinikum Eppendorf, Hamburg, Germany, ⁵Klinik für Kinder und Jugendmedizin, Universitätsklinikum Ulm, Ulm, Germany, ⁶Charite, University Medicine Berlin, Berlin, Germany, ⁷Dept. of Dermatology and Allergy, Allergie-Centrum-Charité, Charité – Universitätsmedizin Berlin, Germany, Berlin, Germany, ⁸Kinder- und Jugendrheumatologie, Clementine-Kinderhospital, Frankfurt, Germany, ⁹Department of Pediatrics, Asklepios Klinik Sankt Augustin, Sankt Augustin, Germany, ¹⁰University Hospital Tuebingen, Tuebingen, Germany
Background/Purpose: Canakinumab is a recombinant monoclonal fully human antibody against Interleukin-1β and approved for the treatment of CAPS in many countries including Europe and the…
Abstract Number: 1213 • 2013 ACR/ARHP Annual Meeting
Low-Penetrance NLRP3-Variants
Theresa Endres¹, Ferdinand Hofer¹, Raphaela T. Goldbach-Mansky², Hal M. Hoffman³, Norbert Blank⁴, Karoline Krause⁵, Christoph Rietschel⁶, Gerd Horneff⁷, Peter Lohse⁸ and Jasmin B. Kuemmerle-Deschner⁹, ¹Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, ²Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ³Division of Allergy, Immunology and Rheumatology, University of California at San Diego, La Jolla, CA, ⁴University of Heidelberg, Heidelberg, Germany, ⁵Dept. of Dermatology and Allergy, Allergie-Centrum-Charité, Charité – Universitätsmedizin Berlin, Germany, Berlin, Germany, ⁶Kinder- und Jugendrheumatologie, Clementine-Kinderhospital, Frankfurt, Germany, ⁷Department of Pediatrics, Asklepios Klinik Sankt Augustin, Sankt Augustin, Germany, ⁸Institut für Laboratoriumsmedizin und Humangenetik, Singen, Germany, ⁹University Hospital Tuebingen, Tuebingen, Germany
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) presents as rare, autosomal dominant disease spectrum, due to mutations in the NLRP3-gene which lead to excessive interleukin-1 (IL-1) release.…
Abstract Number: 1167 • 2013 ACR/ARHP Annual Meeting
A Possible Mechanism Of NLRP3 Inflammasome Hypersensitivity In Cryopyrin-Associated Periodic Syndrome
Sayaka Ito, Chie Tagami, Yukichi Hara and Tetsuo Kubota, Tokyo Medical and Dental University, Tokyo, Japan
Background/Purpose: NLRP3 in monocytes and other cell types plays a role in innate immunity as one of the intracellular pathogen recognition receptors, and its mutation…

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