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  • Abstract Number: 1781 • 2013 ACR/ARHP Annual Meeting

    Clinical Variation In Children With Mutations In MEFV

    Karyl Barron1, Amanda K. Ombrello2, Ivona Aksentijevich3, Anne Jones3 and Daniel L. Kastner3, 1Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, 2National Human Genome Research Institute,National Institute of Health, Bethesda, MD, 3Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD

    Background/Purpose: As a referral clinic for children with periodic fevers, we see a wide variety of clinical presentations.  While genetic testing is usually helpful in…
  • Abstract Number: 1782 • 2013 ACR/ARHP Annual Meeting

    Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperatures (CANDLE): Clinical Characterization and Initial Response To Janus Kinase Inhibition With Baricitinib

    Gina A. Montealegre Sanchez1, Adam L. Reinhardt2, Paul Brogan3, Yackov Berkun4, Diane Brown5, Peter Chira6, Ling Gao7, Dawn C. Chapelle1, Nicole Plass1, Hanna Kim1, Michael Davis8, Adriana Almeida de Jesus8, Yin Liu1, Yan Huang1, Colleen Hadigan9, Theo Heller10, Zlotogorski Zlotogorski11, John J. O'Shea12, Chyi-chia Lee13, Suvimol C. Hill14, Kristina Rother15, Massimo Gadina16 and Raphaela T. Goldbach-Mansky1, 1Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 2Rheumatology, Children's Hosp of Omaha/UNMC, Omaha, NE, 3Paediatric Rheumatology International Trials Organization (PRINTO), Istituto Giannina Gaslini, Genova, Italy, 4Pediatrics, Hadassah Medical Center, Mount Scopus, Jerusalem, Israel, 5Division Of Rheumatology MS #60, Children's Hospital Los Angeles, Los Angeles, CA, 6Pediatric Rheumatology, Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN, 7Dermatology, University of Arkansas for Medical Sciences, Little Rock, AR, 8Translational Autoinflammatory Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 9National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD, 10National Institute of Diabetes and Digestive Kidney Diseases, NIH, Bethesda, MD, 11Department of Dermatology, Hadassah-Hebrew University Medical Center, Jerusalem, Department of Dermatology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel., Jerusalem, Israel, 12NIAMS, Bethesda, MD, 13National Cancer Institute, National Institutes of Health, Bethesda, MD, 14Radiology and Imaging Sciences, NIH Clinical Center, Bethesda, MD, 15Pediatric Diabetes & Metabolism, National Institute of Diabetes and Digestive Kidney Diseases, NIH, Bethesda, MD, 16National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD

    Background/Purpose: CANDLE, a novel autoinflammatory syndrome presents with attacks of fever, annular cutaneous plaques, persistent periorbital edema, arthritis, lymphopenia and lipodystrophy since early childhood. CANDLE…
  • Abstract Number: 1783 • 2013 ACR/ARHP Annual Meeting

    Canakinumab Treatment In Active Hyper-IgD With Periodic Fever Syndrome

    Juan Ignacio Aróstegui1, Inmaculada Calvo2, Jordi Anton3, Angel Robles4, Jordi Yagüe1, Ruth Viana5, Lillian Tseng6 and Ken Abrams6, 1Immunology Department, Hospital Clinic Barcelona, Barcelona, Spain, 2Hospital La Fe, Valencia, Spain, 3Rheumatology, Hospital Sant Joan de Deu, Barcelona, Spain, 4Hospital La Paz, Madrid, Spain, 5Medical Department, Novartis Farmacéutica, S.A., Barcelona, Spain, 6Novartis Pharmaceuticals Corporation, East Hanover, NJ

    Background/Purpose: Hyper-IgD with periodic fever syndrome (HIDS) is a recessively inherited autoinflammatory disease due to biallelic MVK mutations. It is characterized by 4-6 days-long acute…
  • Abstract Number: 1784 • 2013 ACR/ARHP Annual Meeting

    Effectiveness Of First Line Use Of Recombinant IL-1RA Treatment In Steroid naïve Systemic Juvenile Idiopathic Arthritis: Results Of a Prospective Cohort Study

    S.J. Vastert1, Wilco de Jager2, Bo Jan Noordman2, Dirk Holzinger3, Wietse Kuis4, Berent J. Prakken2 and Nico M. Wulffraat5, 1Pediatric Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands, 2Pediatric Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 3Institute of Immunology, University Muenster, Muenster, Germany, 4Pediatric immunology, University Medical Center Utrecht, Utrecht, Netherlands, 5UMC Utrecht, Utrecht, Netherlands

    Background/Purpose: To perform a prospective cohort study with recombinant IL-1RA (Anakinra, rIL-1RA) as first-line therapy in newly onset sJIA. Methods: Patients fulfilled the ILAR criteria…
  • Abstract Number: 1785 • 2013 ACR/ARHP Annual Meeting

    Structural Determinants Of Low Vertebral Strength In Pediatric Systemic Lupus Erythematosus

    Jon M. Burnham1, Babette S. Zemel1, David C. Lee2, Tony M. Keaveny3 and Mary Beth Leonard1, 1Pediatrics, The Children's Hospital of Philadelphia and the Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, 2O.N. Diagnostics, LLC, Berkeley, CA, 3Founder and Chief Science Officer, University of California, Berkeley, CA and O.N. Diagnostics, LLC, Berkeley, CA

    Background/Purpose: Children with systemic lupus erythematosus (SLE) are at high risk for bone fragility.  Studies in pediatric SLE using two-dimensional lumbar spine (LS) dual X-ray…
  • Abstract Number: 1786 • 2013 ACR/ARHP Annual Meeting

    91% Of Early RA Patients Are Positive For Serum 14-3-3η Or Its Auto-Antibodies

    Walter P. Maksymowych1, Dirkjan van Schaardenburg2, Désirée van der Heijde3, Robert Landewé4, Gilles Boire5, Maarten Boers6, Vivian P. Bykerk7, Edward C. Keystone8, Katherine A. Siminovitch9, Mairead Murphy10 and Anthony Marotta11, 1Medicine, University of Alberta, Edmonton, AB, Canada, 2Jan van Breemen Research Institute | Reade, Jan van Breemen Research Institute | Reade, Amsterdam, Netherlands, 3Department of Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 4Int Med/Rheumatology, Academic Medical Center, Amsterdam, Netherlands, 5Rheumatology Division, Centre Hospitalier Universitaire de Sherbrooke, Université de Sherbrooke, Sherbrooke, QC, Canada, 6Epidemiology & Biostatistics, VU University Medical Center, Amsterdam, Netherlands, 7Rheumatology, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, 8Department of Medicine, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 9Mount Sinai Hospital, Toronto, ON, Canada, 10Augurex Life Sciences Corp., North Vancouver, BC, Canada, 11Augurex Life Sciences Corp, North Vancouver, BC, Canada

    Background/Purpose: Current serological markers such as RF and ACPA are useful for early diagnosis and perhaps for screening people at risk, but there remains a…
  • Abstract Number: 1787 • 2013 ACR/ARHP Annual Meeting

    Does Age At Disease Onset Influence Disease Activity and Function In Rheumatoid Arthritis Patients? Results From The Ontario Best Practices Research Initiative (OBRI) Registry

    Thanu Nadarajah1, Binu Jacob2, Pooneh Akhavan3, Bindee Kuriya4, Edward C. Keystone5 and Claire Bombardier6, 1Department of Medicine, University of Toronto, Toronto, ON, Canada, 2University Health Network, Toronto General Research Institute, Toronto, ON, Canada, 3Medicine, Mount Sinai Hospital/University of Toronto, Toronto, ON, Canada, 4Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 5Department of Medicine, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, 6Rheumatology, University of Toronto, Toronto, ON, Canada

    Background/Purpose: The population of elderly individuals with rheumatoid arthritis (RA) is increasing. The aim of this study was to assess whether age at disease onset…
  • Abstract Number: 1748 • 2013 ACR/ARHP Annual Meeting

    Autoantibodies To RuvBL1 and RuvBL2: A Novel Systemic Sclerosis-Related Antibody Associated With Diffuse Cutaneous and Skeletal Muscle Involvement

    Kenzo Kaji1, Noreen Fertig2, Thomas A. Medsger Jr.3, Takashi Sathoh4, Kana Hoshino4, Yasuhito Hamaguchi1, Minoru Hasegawa5, Mary Lucas6, Andrew Schnure6, Fumihide Ogawa7, Shinichi Sato8, Kazuhiko Takehara9, Manabu Fujimoto5 and Masataka Kuwana4, 1Department of Dermatology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan, 2University of Pittsburgh, Pittsburgh, PA, 3Medicine/Rheumatology, University of Pittsburgh, Pittsburgh, PA, 4Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan, 5Dermatology, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan, 6Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, 7Dermatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, 8Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan, 9Dermatology, Kanazawa University, Kanazawa, Japan

    Background/Purpose: Detection of serum autoantibodies is useful in diagnosis, disease subgrouping, and prediction of subsequent organ involvement and prognosis in patients with systemic sclerosis (SSc).…
  • Abstract Number: 1749 • 2013 ACR/ARHP Annual Meeting

    Treatment Strategies and Outcomes In Patients With Systemic Sclerosis and Acute Myocardial Infarction

    Priya Prakash1, Dhaval Kolte1, Sahil Khera1, Jagadish Khanagavi1, Marjan Mujib2, Chandrasekar Palaniswamy3, Farrah Gutwein4, Sachin Sule1, Wilbert S. Aronow3, William H. Frishman3 and Julia Ash4, 1Medicine, New York Medical College, Valhalla, NY, 2Internal Medicine, New York Medical College, Valhalla, NY, 3Cardiology, New York Medical College, Valhalla, NY, 4Rheumatology, New York Medical College, Valhalla, NY

    Background/Purpose: , Systemic Sclerosis (SSc) is associated with an increased risk of cardiovascular diseases, including acute myocardial infarction (AMI). However, whether SSc influences treatment choice and…
  • Abstract Number: 1750 • 2013 ACR/ARHP Annual Meeting

    Short and Long-Term Biological Therapy In Refractory Uveitis Of Behcet’s Syndrome. Multicenter Study Of 124 Patients

    Francisco Ortiz-Sanjuan1, Vanesa Calvo-Río2, Ricardo Blanco3, Emma Beltrán4, Juan Sánchez-Bursón5, Marina Mesquida6, Alfredo M. Adan6, M Hernandez Grafella7, E Valls Pascual8, L Martínez-Costa9, Agustí Sellas-Fernàndez10, Miguel Cordero-Coma11, Manuel Diaz-llopis12, David Salom12, Jl García Serrano13, Norberto Ortego13, JM Herreras14, Alejandro Fonollosa15, A Aparicio16, O Maíz17, A Blanco18, I Torre19, Cruz Fernández-Espartero20, V Jovani21, D Peitado-Lopez22, Esperanza Pato23, J Cruz24, J. Carlos Fernandez-Cid25, E. Aurrecoechea26, M García27, M Caracuel28, Carlos Montilla29, A Atanes30, F Francisco31, S Insua32, S González-Suárez33, A Sánchez-Andrade34, F Gamero35, Luis Linares36, F Romero-Bueno37, AJ García González38, Raquel Almodovar39, E Minguez40, C Carrasco Cubero41, Alejandro Olive Marques42, J Vázquez43, O Ruiz Moreno44, F Jimenez-Zorzo44, J Manero44, Javier Loricera1 and Miguel Angel González-Gay1, 1Rheumatology, Hospital Universitario Marqués de Valdecilla. IFIMAV. Santander. Spain, Santander, Spain, 2Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Spain, Santander, Spain, 3Hospital Marques de Valdecilla, Santander, Spain, 4Rheumatology, Hospital General Universitario de Valencia. Spain, Valencia, Spain, 5Hospital de Valme. Sevilla, Sevilla, Spain, 6Ophthalmology, Hospital Clínic of Barcelona, Barcelona, Spain, 7Ophthalmology, Hospital General Universitario, Valencia, Valencia, Spain, 8Rheumatology, Hospital Peset Valencia, Valencia, Spain, 9Ophthalmology, Hospital Peset Valencia, Valencia, Spain, 10Rheumatology, Hospital Val d´Hebron. Barcelona, Barcelona, Spain, 11Departament of Ophthalmology, Hospital de León, León, Spain, 12Department of Ophthalmology, Hospital Universitario La Fe de Valencia, Valencia, Spain, 13Hospital San Cecilio. Granada, Granada, Spain, 14Hospital Universitario, IOBA. Valladolid, Valladolid, Spain, 15Ophtalmology Service, Hospital de Cruces. Bilbao, Bilbao, Spain, 16Rheumatology., Hospital de Toledo., Toledo, Spain, 17Rheumatology, Hospital Donosti San Sebastian, San Sebastián, Spain, 18Ophthalmology., Hospital Donosti San Sebastian, San Sebastián, Spain, 19Rheumatology., Hospital Basurto. Bilbao, Bilbao, Spain, 20Servicio de Reumatología, Hospital Universitario de Móstoles, Madrid, Spain, 21Rheumatology., Hospital General de Alicante., Alicante, Spain, 22Rheumatology, Hospital Universitario La Paz Madrid, Madrid, Spain, 23Rheumatology, Hospital Clínico San Carlos. Madrid, Madrid, Spain, 24Rheumatology, Hospital de Pontevedra, Pontevedra, Spain, 25Departament of Ophthalmology, Hospital de Pontevedra, Pontevedra, Spain, 26Hospital Sierrallana. Torrelavega, Torrelavega, Spain, 27Rheumatology., Hospital La Princesa. Madrid, Madrid, Spain, 28Rheumatology., Hospital de Córdoba., Córdoba, Spain, 29Unit Rheumatology, Hospital Universitario de Salamanca, Salamanca, Spain, 30Rheumatology., HUCA La Coruña., A Coruña, Spain, 31Rheumatology., Hospital Doctor Negrín Canarias., Canarias, Spain, 32Rheumatology., Hospital Universitario Santiago de Compostela, Santiago de Compostela, Spain, 33Rheumatology., Hospital Cabueñes, Gijón, Gijón, Spain, 34Rheumatology., Hospital Lucus Augusti Lugo, Lugo, Spain, 35Rheumatology., Hospital San Pedro Alcantara Caceres, Caceres, Spain, 36Rheumatology, Hospital Universitario Virgen de la Arrixaca. Murcia, Murcia, Spain, 37Rheumatology., Fundación Jimenez Díaz. Madrid, Madrid, Spain, 38Rheumatology., Hospital 12 de Octubre. Madrid, Madrid, Spain, 39Rheumatology Unit, Hospital Universitario Fundación Alcorcón. Madrid, Alcorcon. Madrid, Spain, 40Ophthalmology, Hospital Clínico de Zaragoza, Zaragoza, Spain, 41Rheumatology., Hospital de Mérida, Mérida, Spain, 42Hospital Germans Trias i Pujol. Badalona, Barcelona, Spain, 43Rheumatology, Hospital de Ferrol. A Coruña, A Coruña, Spain, 44Hospital Universitario Miguel Servet. Zaragoza, Zaragoza, Spain

    Background/Purpose: To evaluate short and long-term response to biological therapy in uveitis associated to Behçet´s syndrome refractory to standard systemic treatment. Methods: Multicenter study of…
  • Abstract Number: 1751 • 2013 ACR/ARHP Annual Meeting

    Ethnicity-Related Differences In Behçet’s Disease In a French Multiethnic Country

    David Saadoun1, Mathieu Resche Rigon2, Bertrand Wechsler3, Du Le Thi Huong4, Jean-Charles Piette1 and Patrice Cacoub5, 1Groupe Hospitalier Pitié Salpétrière, Service de Médecine Interne, DHU i2B, Paris, France, 2Department of Internal Medicine and Laboratory I3 “Immunology, Immunopathology, Immunotherapy”, UMR CNRS 7211, INSERM U959, Groupe Hospitalier Pitié-Salpetrière, Université Pierre et Marie Curie, Paris 6, Paris, France, Paris, France, 3Department of Internal Medicine 2. Referal center for SLE/APS, CHU Pitié-Salpêtrière, Paris, France, 4Internal Medicine, Groupe Hospitalier Pitié-Salpétrière, Paris, France, 5Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hopital Pitié-Salpétrière, Paris, France

    Background/Purpose: It has been suggested that Behçet’s disease (BD) varies in its phenotypic expression in different ethnies and in different countries indicating that both environmental…
  • Abstract Number: 1752 • 2013 ACR/ARHP Annual Meeting

    Behçet´s Disease Activity: An Important Factor For Immunogenicity Of Unadjuvanted Influenza A/H1N1 Vaccine

    Leandro L. Prado1, Carla G.S. Saad2, Julio C. B. Moraes1, Ana Cristina Medeiros Ribeiro1, Nadia E. Aikawa1, Clovis A. Silva3, Claudia G Schainberg1, Percival D Sampaio-Barros1, Alexander R. Precioso4, Maria A. Ishida5, Eloisa Bonfa6 and Celio Goncalves1, 1Reumatologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil, 2Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, 3Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, 4Instituto Butantan, São Paulo, Brazil, 5Instituto Adolfo Lutz, São Paulo, Brazil, 6University of Sao Paulo, Rheumatology Division, São Paulo, Brazil

    Background/Purpose: Routine annual influenza vaccination is the most effective method for preventing influenza virus infection and its complications. Influenza vaccine is recommended for all persons…
  • Abstract Number: 1753 • 2013 ACR/ARHP Annual Meeting

    An Outcome Survey Of 40 Patients With Budd-Chiari Syndrome Due To BEHÇET’S Syndrome Followed By A Single Center

    Emire Seyahi1, SerdaL Ugurlu2, Erkan Caglar3, Fatih Kantarci4, Abdullah Sonsuz5, Sebahattin Yurdakul2 and Hasan Yazici2, 1Division of Rheumatology,Department of Medicine,Cerrahpasa Medical Faculty, University of Istanbul, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 2Division of Rheumatology,Department of Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 3Division of Gastroenterology, Department of Internal Medicine, Bakirkoy Research and Training Hospital, Istanbul, Turkey, 4Department of Radiology, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 5Division of Gastroenterology,Department of Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Background/Purpose: Budd-Chiari syndrome is a rare complication of BS with a frequency of < 1 %, and carries a high mortality rate. In a previous…
  • Abstract Number: 1754 • 2013 ACR/ARHP Annual Meeting

    Safety and Efficacy Of Peg-IFNa/Ribavirin/Protease Inhibitor Combination In 34 Patients With HCV-Mixed Cryoglobulinemia Vasculitis

    David Saadoun1, Stanislas Pol2, Vincent Thibault3, Gilles Pialoux4, François Blanc5, Lucile Musset6, Alexandre Karras7, Olivier Decaux8, Jean-Marc Ziza9, Olivier Lambotte10 and Patrice Cacoub Sr.11, 1Groupe Hospitalier Pitié Salpétrière, Service de Médecine Interne, DHU i2B, Paris, France, 2Hepatology, Cochin Hospital, Paris, France, 3Virology, Pitié-Salpétrière, Paris, France, 4hôpital Tenon, Paris, France, 5hôpital Montpellier, Montpellier, France, 6Laboratoire d'immunochimie, Pitié- Salpêtrière, Paris, France, 7Nephrology, Hôpital Européen Georges Pompidou, APHP, Paris, France, 8Department of Internal Medicine, Rennes University Hospital, Rennes, France, 9Refferal center for bone and joint infections, Croix Saint Simon Hospital, Paris, France, 10Internal Medicine, Hopital Kremlin Bicêtre, Kremlin Bicêtre, France, 11Department of Internal Medicine 2., CHU Pitié-Salpêtrière, Paris, France

    Background/Purpose: The standard of care treatment of patients presenting a HCV-mixed cryoglobulinemia (MC) vasculitis includes Peg-IFNaplus Ribavirin, w/wo Rituximab. Thirty to 40% of patients are…
  • Abstract Number: 1755 • 2013 ACR/ARHP Annual Meeting

    Genetic Association Of Mitochondrial DNA Polymorphisms With Behçet’s Disease In a Korean Population

    Mi-Hye Kwon1, Chung-Il Joung2 and Jiwon Hwang3, 1Department of Internal medicine, Konyang university hospital, Daejeon, South Korea, 2Department of Internal Medicine, Konyang university hospital, Daejeon, South Korea, 3Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea

    Background/Purpose: Behcet’s disease (BD) is an inflammatory multisystemic disorder. Although precise etiopathogenesis should be elucidated, BD is known to be multifactorial and accumulating data suggest…
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All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

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