Abstracts tagged "Autoinflammatory Disease"

Abstract Number: 1233 • 2014 ACR/ARHP Annual Meeting
Tocilizumab (TCZ) in the Treatment of AA Amyloidosis in Patients with Familial Mediterranean Fever
Huri Ozdogan, Serdal Ugurlu, Aysa Hacioglu, Yasaman Adibnia and Vedat Hamuryudan, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey
Background/Purpose: AA amyloidosis is the major long-term complication of various chronic inflammatory diseases like rheumatoid arthritis, ankylosing spondylitis, FMF and other autoinflammatory syndromes. Treatment of…
Abstract Number: 1232 • 2014 ACR/ARHP Annual Meeting
Canakinumab Therapy in Patients with Familial Mediterranean Fever
Serdal Ugurlu, Emire Seyahi, Gulen Hatemi, Aysa Hacioglu, Fatma Nihan Akkoc and Huri Ozdogan, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey
Background/Purpose: According to a recent pilot study Canakinumab reduced the frequency of attacks in 9 patients with Familial Mediterranean Fever (FMF) resistant to colchicine with no…
Abstract Number: 1231 • 2014 ACR/ARHP Annual Meeting
Evidence Based Recommendations for Genetic Diagnosis of Familial Mediterranean Fever
Gabriella Giancane¹, Nienke ter Haar², Nico Wulffraat³, Bas Vastert⁴, Karyl Barron⁵, Veronique Hentgen⁶, Tilmann Kallinich⁷, Huri Ozdogan⁸, Jordi Anton⁹, Paul Brogan¹⁰, Luca Cantarini¹¹, Joost Frenkel⁴, Caroline Galeotti¹², Marco Gattorno¹³, Gilles Grateau¹⁴, Michael Hofer¹⁵, Isabelle Kone-Paut¹⁶, J B. Kuemmerle-Deschner¹⁷, Helen Lachmann¹⁸, Anna Simon¹⁹, Brian Feldman²⁰, Yosef Uziel²¹ and Seza Ozen²², ¹Pediatric Immunology, UMC, Utrecht, Netherlands, Utrecht, Netherlands, ²Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ³Pediatric rheumatology, Wilhelmina Children's Hospital/ UMC Utrecht, Utrecht, Netherlands, ⁴University Medical Center Utrecht, Utrecht, Netherlands, ⁵NIH, Bethesda, MD, ⁶Versailles Hospital, Le Chesnay Cedex, France, ⁷Charite, University Medicine Berlin, Berlin, Germany, ⁸Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ⁹Pediatric Rheumatology Unit. Hospital Sant Joan de Déu. Universitat de Barcelona, Barcelona, Spain, ¹⁰Rheumatology Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, ¹¹University of Siena, Siena, Italy, ¹²Bicêtre Hospital, University of Paris SUD, Paris, France, ¹³Istituto Giannina Gaslini, Genova, Italy, ¹⁴Service De Médecine Interne, Hopital Tenon, Paris, France, ¹⁵Centre Multisite Romand de Rhumatologie Pediatrique, Lausanne, Switzerland, ¹⁶Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, ¹⁷Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ¹⁸UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom, ¹⁹Radboudumc, Nijmegen, Netherlands, ²⁰Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, ²¹Tel-Aviv University, Sackler School of Medicine, Tel-Aviv, Israel, ²²Deptartment. of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey
Background/Purpose: Familial Mediterranean Fever (FMF) is a disease that starts in childhood and can lead to significant morbidity. In 2013, an initiative called SHARE (Single…
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…
Abstract Number: 2864 • 2013 ACR/ARHP Annual Meeting
Whole Exome Sequencing In Pediatric Patients With Early Onset Rare Immunodysregulatory Diseases That Present With Fever and Systemic Inflammation
Adriana Almeida de Jesus¹, Julie Niemela², Yin Liu³, Steven Boyden⁴, Ivona Aksentijevich⁵, Daniel L. Kastner⁶, Thomas A. Fleisher⁷, Raphaela Goldbach-Mansky³ and Zuoming Deng⁸, ¹Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ²Laboratory Medicine, NIH Clinical Center, Bethesda, MD, ³Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ⁴National Human Genome Research Institute, NIH, Bethesda, MD, ⁵Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, ⁶Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, MD, ⁷Laboratory Medicine, Laboratory Medicine, NIH Clinical Center, Bethesda, MD, ⁸Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD
Background/Purpose: WES (Whole Exome Sequencing) has increasingly become the tool of choice in translational research, providing molecular diagnoses in Mendelian diseases and identifying important genes…
Abstract Number: 1143 • 2013 ACR/ARHP Annual Meeting
Increased Frequency Of Pratroling Monocytes In Experimental Arthritis and Rheumatoid Arthritis Patients In Response To IL6-R Blockade
Julie Quentin¹, Jessy Presumey², Florence Apparailly², Yves-Marie Pers³, Pascale Louis Plence⁴ and Christian Jorgensen³, ¹Inserm U844, Montpellier, France, ²U844, Inserm, Montpellier, France, ³Department of therapy & Immuno-Rhumatology, Inserm U844, CHU saint-Eloi, Université Montpellier 1, CHU Lapeyronie, Montpellier, France, ⁴INSERM U844 Montpellier, Montpellier, France
Background/Purpose: Monocytes represent a heterogeneous circulating population of immune cells that play important roles in the inflammatory response. Two main functional subsets of human monocytes…
Abstract Number: 2747 • 2013 ACR/ARHP Annual Meeting
Canakinumab Treatment In Schnitzler’s Syndrome: A Multi-Center Randomized Placebo-Controlled 4-Month Study
Karoline Krause, Karsten Weller, Martin Metz and Marcus Maurer, Dept. of Dermatology and Allergy, Allergie-Centrum-Charité, Charité – Universitätsmedizin Berlin, Germany, Berlin, Germany
Background/Purpose: Schnitzler’s syndrome (SchS) is an adult-onset autoinflammatory disease characterized by urticarial exanthema and monoclonal gammopathy in combination with episodes of fever, arthralgia, fatigue, and…
Abstract Number: 227 • 2013 ACR/ARHP Annual Meeting
Quantitative Image Analysis Of Articular Involvement In Blau Syndrome By Radiographic Calpal Length and Ultrasound Assessment
Tsuyoshi Yamatou¹, Tomohiro Kubota², Harumi Akaike¹, Yuichi Yamasaki², Yukiko Nonaka¹, Yasuhito Nerome¹, Tomoko Takezaki¹, Hiroyuki Imanaka¹, Kei Ikeda³, Naotomo Kambe⁴, Syuji Takei⁵ and Tomokazu Nagakura⁶, ¹Department of Pediatrics, Kagoshima University Hospital, Kagoshima, Japan, ²Dept of Pediatrics, Faculty of Medicine, Kagoshima University, Kagoshima, Japan, ³Department of Allergy and Clinical Immunology, Chiba University Hospital, Chiba, Japan, ⁴Dermatology, Chiba University Graduate School of Medicine, Chiba, Japan, ⁵School of Health Sciences, Faculty of Medicine, Kagoshima University, Kagoshima, Japan, ⁶Department of Pediatrics, House of Meguminoseibo, Usuki, Japan
Background/Purpose: Blau syndrome (Blau) is a rare auto-inflammatory disease, and it has now been shown to be caused by NOD2/CARD15 gene mutations. Clinical features of…
Abstract Number: 2750 • 2013 ACR/ARHP Annual Meeting
Long-Term Efficacy and Safety Of IL1 Receptor Antagonist In Schnitzler’s Syndrome : A French Multicenter Study
Antoine Néel¹, Agathe Masseau¹, Sebastien Barbarot², Benoit Henry³, Pierre-Jean Weiller⁴, Olivier Decaux⁵, Xavier Kyndt⁶, Xavier Puechal⁷, Arnaud Hot⁸, Pierre Pottier¹, Amar Smail⁹, David Launay¹⁰, Jean-Marie Berthelot¹¹, Eric Hachulla¹², Leonardo Astudillo¹³, Pierre-Yves Hatron¹², Laurent Sailler¹³, Aurelien Lorleac'h¹⁴, Achille Aouba¹⁵, Bérangère Cador¹⁶, Renato Fior¹⁷, Robin Dhote¹⁸, Fabrice Bonnet¹⁹, Jean-Dominique de Korwin²⁰ and Mohamed Hamidou¹, ¹Internal Medicine Department, Nantes University Hospital, Nantes, France, ²Dermatology, Nantes University Hospital, Nantes, France, ³Infectious and Tropical Diseases, Pitié-Salpêtrière University Hospital, Paris, France, ⁴Internal Medicine Department, La Timone University Hospital, Marseille, France, ⁵Department of Internal Medicine, Rennes University Hospital, Rennes, France, ⁶Department of Nephrology and Internal Medicine, CH, Valenciennes, Valenciennes, France, ⁷Internal Medicine, Hôpital Cochin, Paris, France, ⁸Internal Medicine, Edouard Herriot University Hospital, Lyon, France, ⁹Internal Medicine Department, CHU Amiens Nord, Amiens, France, ¹⁰Internal Medicine, Claude Huriez University Hospital, Lille, France, ¹¹Rheumatology Unit, Nantes University Hospital, Nantes, France, ¹²Department of Internal Medicine, Claude Huriez University Hospital, Lille, France, ¹³Department of Internal Medicine, Toulouse University Hospital, Toulouse, France, ¹⁴Internal Medicine Department, Lorient Hospital, Lorient, France, ¹⁵Hematology, Necker University Hospital, Paris, France, ¹⁶Internal Medicine Department, Rennes University Hospital, Rennes, France, ¹⁷Internal Medicine Department, Antoine Béclère University Hospital, Clamart, France, ¹⁸Internal Medicine, Avicenne University Hospital, Bobigny, France, ¹⁹Internal Medicine, Bordeaux University Hospital, Bordeaux, France, ²⁰Internal Medicine Department, Nancy University Hospital, Nancy, France
Background/Purpose: Schnitzler's syndrome is a rare late onset auto-inflammatory disease which associates a chronic/recurrent urticarial skin rash, a monoclonal gammopathy (mostly IgM kappa), and a…
Abstract Number: 2613 • 2013 ACR/ARHP Annual Meeting
NF-κB Pathway Is Depleted In Phagocytes From Behçet´s Disease Patients Secondarily To Constitutive Phosphorylation Of The p65 Subunit
Sandro F. Perazzio^1,2, Paulo Vitor Soeiro Pereira³, Alexandre W.S. Souza^4,5, Antonio Condino-Neto³ and Luis Eduardo C. Andrade⁶, ¹Rheumatology, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ²Fleury Medicine and Health, Sao Paulo, Brazil, ³Immunology, ICB IV - Universidade de São Paulo, São Paulo, Brazil, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Rheumatology Div/Dept of Med, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ⁶Rheumatology Division, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil
Background/Purpose: : Increased neutrophil activation has been previously shown in Behçet’s disease (BD) patients and it is unclear whether neutrophil activation occurs constitutively or if…
Abstract Number: 2614 • 2013 ACR/ARHP Annual Meeting
Plasma Of Active Behçet´s Disease Increases Oxidative Metabolism Profile Of Normal and Patients Phagocytes
Sandro F. Perazzio^1,2, Paulo Vitor Soeiro Pereira³, Alexandre W.S. Souza^4,5, Antonio Condino-Neto³ and Luis Eduardo C. Andrade⁶, ¹Rheumatology, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ²Fleury Medicine and Health, Sao Paulo, Brazil, ³Immunology, ICB IV - Universidade de São Paulo, São Paulo, Brazil, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Rheumatology Div/Dept of Med, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil, ⁶Rheumatology Division, Escola Paulista de Medicina - Universidade Federal de São Paulo, Sao Paulo, Brazil
Background/Purpose: Behçet’s disease (BD) exhibits several features suggestive of neutrophil hiperactivity. It is unclear whether neutrophil activation occurs constitutively or if it is secondary to…
Abstract Number: 2173 • 2013 ACR/ARHP Annual Meeting
The Interleukin-1 Receptor Antagonist Anakinra Is Effective In The Treatment Of Undifferentiated Periodic Fever Syndromes
Daniella M. Schwartz¹, Runsheng Wang², Karyl Barron³, Daniel L. Kastner⁴ and Amanda K. Ombrello⁵, ¹Rheumatology fellowship and training branch, National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ²NIH/NIAMS, Rheumatology fellowship and training branch, Bethesda, MD, ³Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁴Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, MD, ⁵National Human Genome Research Institute,National Institute of Health, Bethesda, MD
Background/Purpose: The autoinflammatory diseases are a group of disorders associated with dysregulation of the innate immune system. They are characterized by recurrent episodes of fever…

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