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  • Abstract Number: 2278 • 2014 ACR/ARHP Annual Meeting

    Orbital Pseudotumor As the Presenting Symptom of Pediatric ANCA-Associated Vasculitis

    Amanda Schlefman1, Maureen Leffler2, AnneMarie C. Brescia3 and Carlos D. Rose4, 1A.I. DuPont Hospital for Children, Wilmington, DE, 2Rheumatology, AI duPont Hospital for Children, Wilmington, DE, 3Pediatric Rheumatology, Thomas Jefferson University/ AI duPont Hospital for Children, Wilmington, DE, 4Pediatrics, Division of Rheumatology, Nemours A.I. duPont Hospital for Children, Thomas Jefferson University, Wilmington, DE

    Background/Purpose: Ocular involvement, particularly orbital pseudotumor, has been reported as the initial manifestation of ANCA-associated vasculitis (AAV) in the adult population, predominantly in granulomatosis with…
  • Abstract Number: 2279 • 2014 ACR/ARHP Annual Meeting

    Evidence Based Recommendations for Diagnosis and Management of Tumor Necrosis Factor Receptor-1 Associated Periodic Syndrome (TRAPS)

    Nienke ter Haar1, Paul Brogan2, Gilles Grateau3, Jordi Anton4, Karyl Barron5, Luca Cantarini6, Joost Frenkel7, Caroline Galeotti8, Veronique Hentgen9, Michael Hofer10, Tilmann Kallinich11, Isabelle Kone-Paut12, Jasmin Kuemmerle-Deschner13, Huri Ozdogan14, Seza Ozen15, Ricardo Russo16, Anna Simon17, Yosef Uziel18, Carine Wouters19, Brian Feldman20, Bas Vastert7, Nico Wulffraat21, Helen Lachmann22 and Marco Gattorno23, 1Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 2Rheumatology Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 3Service De Médecine Interne, Hopital Tenon, Paris, France, 4Pediatric Rheumatology Unit. Hospital Sant Joan de Déu. Universitat de Barcelona, Barcelona, Spain, 5NIH, Bethesda, MD, 6University of Siena, Siena, Italy, 7University Medical Center Utrecht, Utrecht, Netherlands, 8Bicêtre Hospital, University of Paris SUD, Paris, France, 9Versailles Hospital, Le Chesnay Cedex, France, 10Centre Multisite Romand de Rhumatologie Pediatrique, Lausanne, Switzerland, 11Charite, University Medicine Berlin, Berlin, Germany, 12Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, 13Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 14Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 15Deptartment. of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, 16Immunology & Rheumatology, Hospital De Pediatria, Buenos Aires, Argentina, 17Radboudumc, Nijmegen, Netherlands, 18Tel-Aviv University, Sackler School of Medicine, Tel-Aviv, Israel, 19University of Leuven, Laboratory of Pediatric Immunology, University Hospital Leuven, Leuven, Belgium, 20Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 21Pediatric rheumatology, Wilhelmina Children's Hospital/ UMC Utrecht, Utrecht, Netherlands, 22UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom, 23Istituto Giannina Gaslini, Genova, Italy

    Background/Purpose Tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS) is a rare hereditary autoinflammatory syndrome that can lead to significant morbidity. Evidence-based guidelines are lacking…
  • Abstract Number: 2280 • 2014 ACR/ARHP Annual Meeting

    Evidence Based Recommendations for the Management of Cryopyrin Associated Periodic Syndromes (CAPS)

    Nienke ter Haar1, Marlen Oswald2, Luca Cantarini3, Marco Gattorno4, Michael Hofer5, Isabelle Kone-Paut6, Jordi Anton Lopez7, Karyl Barron8, Paul Brogan9, Joost Frenkel10, Caroline Galeotti11, Gilles Grateau12, Veronique Hentgen13, Tilmann Kallinich14, Helen Lachmann15, Huri Ozdogan16, Seza Ozen17, Ricardo Russo18, Anna Simon19, Yosef Uziel20, Carine Wouters21, Brian Feldman22, Bas Vastert10, Nico Wulffraat23, Susanne Benseler24 and Jasmin Kuemmerle-Deschner25, 1Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 2University Hospital Tuebingen, Tuebingen, Germany, 3University of Siena, Siena, Italy, 4Pediatric Rheumatology, Instituto Giannina Gaslini, Genoa, Italy, 5Centre Multisite Romand de Rhumatologie Pediatrique, Lausanne, Switzerland, 6Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, 7pediatric Rheumatology, University Childrenxs Hospital, Barcelona, Spain, 8NIH, Bethesda, MD, 9Rheumatology Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 10University Medical Center Utrecht, Utrecht, Netherlands, 11Bicêtre Hospital, University of Paris SUD, Paris, France, 12Service De Médecine Interne, Hopital Tenon, Paris, France, 13Versailles Hospital, Le Chesnay Cedex, France, 14Charite, University Medicine Berlin, Berlin, Germany, 15UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom, 16Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 17Hacettepe University, Ankara, Turkey, 18Immunology & Rheumatology, Hospital De Pediatria, Buenos Aires, Argentina, 19Radboudumc, Nijmegen, Netherlands, 20Tel-Aviv University, Sackler School of Medicine, Tel-Aviv, Israel, 21University of Leuven, Laboratory of Pediatric Immunology, University Hospital Leuven, Leuven, Belgium, 22Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 23Paediatric Immunology and Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands, 24The Hospital for Sick Children, Toronto, ON, Canada, 25Pediatrics, University Hospital Tuebingen, Tuebingen, Germany

    Background/Purpose Cryopyrin-associated periodic syndromes (CAPS) is a group of rare monogenetic autoinflammatory disorders. Evidence-based guidelines are lacking and management is mostly based on physician’s experience. Consequently,…
  • Abstract Number: 2281 • 2014 ACR/ARHP Annual Meeting

    Dissecting the Heterogeneity of  Macrophage Activation Syndrome

    Francesca Minoia1, Sergio Davì1, AnnaCarin Horne2, Francesca Bovis3, Erkan Demirkaya4, Alessandro Consolaro5, Jonathan Akikusa6, Nuray Aktay Ayaz7, Patrizia Barone8, Bianca Bica9, Isabel Bolt10, Luciana Breda11, Zane Davidsone12, Carmen De Cunto13, Jaime De Inocencio14, Sandra Enciso15, Romina Gallizzi16, Thomas Griffin17, Teresa Hennon18, Gerd Horneff19, Maka Ioseliani20, Michael Jeng21, Agneza Marija Kapovic22, Bianca Lattanzi5, Jeffrey M Lipton23, Silvia Magni-Manzoni24, Clarissa Nassif25, Ingrida Rumba-Rozenfelde26, Claudia Saad-Magalhães27, Sulaiman Almayouf28, Wafaa Al-Suwairi29, Kimo C Stine30, Olga Vougiouka31, Lehn K. Weaver32, Nicolino Ruperto33, Alberto Martini5,34, Randy Q. Cron35 and Angelo Ravelli34, 1Pediatria II, Istituto Giannina Gaslini, Genova, Italy, 2Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden, 3Pediatria II, PRINTO, PRINTO - Istituto Giannina Gaslini, Genoa, Italy, 4Gulhane Military Medical Faculty, Ankara, Turkey, 5Istituto Giannina Gaslini, Genova, Italy, 63 West Clinical Offices, Royal, Royal Children's Hospital, Melbourne, Australia, 7Bakırkoy Maternity and Childrens Education and Research Hospital, Istanbul, Turkey, 8Azienda Policlinico Università di Catania, Catania, Italy, 9Hospital Universitário Clementino Fraga Filho, Rio de Janeiro, Brazil, 10Kinderspital Zuerich, Universitaetskinderklinik, Zurich, Switzerland, 11Department of Paediatrics, University of Chieti G. D'Annunzio, Chieti, Italy, 12Children's University Hospital, Riga, Latvia, 13Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 14Hospital 12 de Octubre, Madrid, Spain, 15Hospital Infantil de Mexico Federico Gomez in Mexico City, Mexico City, Mexico, 16University of Messina, Messina, Italy, 17Pediatrics, Division of Rheumatology, Levine Children's Hospital, Carolinas Medical Ctr, Charlotte, NC, 18Division of Pediatric Allergy/Immunology/Rheumatol, Women & Children's Hospital of Buffalo, Buffalo, NY, 19Asklepios Klinik Sankt Augustin, Sankt Augustin, Germany, 20M. Iashvili Children's Central Clinic, Tbilisi, Georgia, 21Stanford University, Stanford, CA, 22Children's Hospital Zagreb, Zagreb, Croatia, 23Steven and Alexandra Cohen Children's Hospital of New York, New York, NY, 24Ospedale Pediatrico Bambino Gesù, Roma, Italy, 25Hospital das Clínicas da Universidade Federal de Minas Gerais, Belo Horizonte, Brazil, 26University of Latvia, Riga, Latvia, 27Alameda Das Hortensias 868, Botucatu Medical School, Botucatu, Brazil, 28King Faisal Specialist H.,Riyadh, Riyadh, Saudi Arabia, 29Pediatric, King AbdulAziz Medical City, Riyadh, Saudi Arabia, 30Arkansas Children's Hospital, Little Rock, AR, 31P. A. Kyriakou Childrens Hospital of Athens University, Athens, Greece, 32Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, 33Pediatria II,, Istituto Giannina Gaslini, Genoa, Italy, 34University of Genova, Genova, Italy, 35Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose Macrophage activations syndrome (MAS) occurring in the context of systemic juvenile idiopathic arthritis (sJIA) can pursue a rapidly fatal course. However, the diagnosis of…
  • Abstract Number: 2282 • 2014 ACR/ARHP Annual Meeting

    Evidence Based Recommendatinos for Diagnosis and Management of Mevalonate Kinase Deficiency (MKD)

    Nienke ter Haar1, Jerold Jeyaratnam2, Jordi Anton3, Caroline Galeotti4, Karyl Barron5, Paul Brogan6, Luca Cantarini7, Marco Gattorno8, Gilles Grateau9, Veronique Hentgen10, Michael Hofer11, Tilmann Kallinich12, Isabelle Kone-Paut13, Jasmin Kuemmerle-Deschner14, Helen Lachmann15, Huri Ozdogan16, Seza Ozen17, Ricardo Russo18, Yosef Uziel19, Carine Wouters20, Brian Feldman21, Bas Vastert2, Nico Wulffraat22, Anna Simon23 and Joost Frenkel2, 1Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 2University Medical Center Utrecht, Utrecht, Netherlands, 3Pediatric Rheumatology Unit. Hospital Sant Joan de Déu. Universitat de Barcelona, Barcelona, Spain, 4Bicêtre Hospital, University of Paris SUD, Paris, France, 5NIH, Bethesda, MD, 6Rheumatology Unit, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 7University of Siena, Siena, Italy, 8Istituto Giannina Gaslini, Genova, Italy, 9Service De Médecine Interne, Hopital Tenon, Paris, France, 10Versailles Hospital, Le Chesnay Cedex, France, 11Centre Multisite Romand de Rhumatologie Pediatrique, Lausanne, Switzerland, 12Charite, University Medicine Berlin, Berlin, Germany, 13Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, 14Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 15UK National Amyloidosis Centre, University College London Medical School, London, United Kingdom, 16Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 17Deptartment. of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, 18Immunology & Rheumatology, Hospital De Pediatria, Buenos Aires, Argentina, 19Tel-Aviv University, Sackler School of Medicine, Tel-Aviv, Israel, 20University of Leuven, Laboratory of Pediatric Immunology, University Hospital Leuven, Leuven, Belgium, 21Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 22Pediatric rheumatology, Wilhelmina Children's Hospital/ UMC Utrecht, Utrecht, Netherlands, 23Radboudumc, Nijmegen, Netherlands

    Background/Purpose Mevalonate kinase deficiency (MKD) is a rare hereditary autoinflammatory syndrome that can lead to significant morbidity. Evidence-based guidelines are lacking and management is mostly…
  • Abstract Number: 2283 • 2014 ACR/ARHP Annual Meeting

    Steroid-Sparing Effect of Anakinra (Kineret®) in the Treatment of Patients with Severe Cryopyrin-Associated Periodic Syndrome

    Bengt Hallén, Mika Leinonen, Torbjörn Kullenberg and Hans Olivecrona, Swedish Orphan Biovitrum, Stockholm, Sweden

    Background/Purpose Cryopyrin-Associated Periodic Syndromes (CAPS) include a group of rare inherited autoinflammatory diseases consisting of FCAS, Muckle-Wells Syndrome and the most severe form, NOMID. Reduction…
  • Abstract Number: 2284 • 2014 ACR/ARHP Annual Meeting

    Joint Involvement in Pediatric Crohn’s Disease Is Related to Higher Disease Activity and Worse Quality of Life

    Beata Derfalvi1,2, Gabor Bozsaki3, Doloresz Szabo3, Aron Cseh3, Katalin Eszter Muller3, Andras Arato3 and Gabor Veres3, 1Pediatrics, Dalhousie University, Halifax, NS, Canada, 22nd. Dept. of Pediatrics, Semmelweis University, Budapest, Hungary, 31st. Dept. of Pediatrics, Semmelweis University, Budapest, Hungary

    Background/Purpose: The incidence of arthritis and arthralgia in pediatric patients with Crohn's disease (CD) is reported to be 2–15% and 22%, respectively. The aim of our…
  • Abstract Number: 2285 • 2014 ACR/ARHP Annual Meeting

    Safety and Efficacy of Rilonacept in Patients with Deficiency of Interleukin-1 Receptor Antagonist (DIRA)

    Dawn C. Chapelle Neal1, Adriana Almeida de Jesus2, Yan Huang3, Yin Liu3, Raphaela Goldbach-Mansky3 and Gina Montealegre4, 1National Institutes of Health, NIAMS/NIH, Bethesda, MD, 2Translational Autoinflammatory Disease Section, NIAMS/NIH, Bethesda, MD, 3Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, 4Office of the Clinical Director, NIAMS/NIH, Bethesda, MD

    Background/Purpose: Deficiency of interleukin-1 receptor antagonist (DIRA) is a neonatal-onset autoinflammatory syndrome caused by recessive mutations in IL1RN gene, the gene encoding the interleukin-1-receptor antagonist.…
  • Abstract Number: 2286 • 2014 ACR/ARHP Annual Meeting

    Food Allergy and Celiac Disease in Children with Juvenile Idiopathic Arthritis

    Trevor E. Davis1,2, Mei-Sing Ong3, Diana Milojevic2, Jyoti Ramakrishna4 and Marc D. Natter5, 1Medicine, Division of Rheumatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, 2Pediatrics, Division of Pediatric Rheumatology, Floating Hospital for Children at Tufts Medical Center, Boston, MA, 3Australian Institute of Health Innovation, University of New South Wales, Sydney, Australia, 4Pediatrics, Division of Pediatric Gastroenterology and Nutrition, Floating Hospital for Children at Tufts Medical Center, Boston, MA, 5Intelligent Health Labs, Children's Hospital Boston, Boston, MA

    Background/Purpose: There are multiple strong associations between gut pathology and rheumatologic diseases. This connection, between primary GI disease and rheumatologic diseases, is manifest in both…
  • Abstract Number: 2287 • 2014 ACR/ARHP Annual Meeting

    Cartilage Thickness and Bone Health in Children with Juvenile Idiopathic Arthritis

    Marinka Twilt1, Dan Pradsgaard2, Anne Helene Spannow2, Arne Horlyck3, Carsten Heuck2 and Troels Herlin4, 1Paediatrics, Aarhus University Hospital, Aarhus, Denmark, 2paediatrics, Aarhus University Hospital, Aarhus, Denmark, 3radiology, Aarhus University Hospital, Aarhus, Denmark, 4Pediatric Rheumatology Clinic, Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark

    Background/Purpose Although treatment options have increased and morbidity has decreased in the last decade Juvenile Idiopathic Arthritis (JIA) may still result in disability. Increasingly ultrasonography…
  • Abstract Number: 2288 • 2014 ACR/ARHP Annual Meeting

    The Research in Arthritis in Canadian Children Emphasizing Outcomes (ReACCh Out) Cohort:  Independent Risk Factors and Medication Use in New Onset Uveitis in Juvenile Idiopathic Arthritis

    Jennifer JY Lee1, Ciarán M. Duffy2, Jaime Guzman3, Nick Barrowman4, Deepti Reddy4, Kimberly Morishita5, Lynn R. Spiegel6, Elizabeth Stringer7, Michele Gibbon2, Rae S.M. Yeung8, Lori B. Tucker3, Kiem Oen9, Karen N Watanabe Duffy2 and ReACChOut Investigators2, 1Pediatrics, Children's Hospital of Eastern Ontario and University of Ottawa, Ottawa, ON, Canada, 2Children's Hospital of Eastern Ontario and University of Ottawa, Ottawa, ON, Canada, 3Rheumatology, BC Children's Hospital and University of British Columbia, Vancouver, BC, Canada, 4Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada, 5BC Children's Hospital and University of British Columbia, Vancouver, BC, Canada, 6The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada, 7Department of Rheumatology, IWK Health Centre, Halifax, NS, Canada, 8Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada, 9University of Manitoba, Winnipeg, MB, Canada

    Background/Purpose: The Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh Out) cohort is a multi-centre prospective inception cohort of newly diagnosed Juvenile Idiopathic Arthritis…
  • Abstract Number: 2289 • 2014 ACR/ARHP Annual Meeting

    Accuracy of the Use of Administrative Diagnostic Codes to Identify Pediatric in-Patient Musculoskeletal Conditions in an African Tertiary Hospital

    Rosie Scuccimarri1, Carol Hitchon2, Sasha Bernatsky3, Eugene Were4, Thomas Ngwiri5 and Ines Colmegna6, 1Rheumatology, Montreal Children's Hospital, Montreal, QC, Canada, 2Rheumatology, University of Manitoba, Winnipeg, MB, Canada, 3Divisions of Rheumatology and Clinical Epidemiology, McGill University Health Centre, Montreal, QC, Canada, 4Gertrude's Children's Hospital, Nairobi, Kenya, 5Pediatrics, Gertrude's Children's Hospital, Nairobi, Kenya, 6Rheumatology, McGill University Health Centre, Montreal, QC, Canada

    Background/Purpose:  The spectrum and frequency of pediatric rheumatic conditions in East Africa are unknown. Administrative data that is systematically collected using International Classification of Disease…
  • Abstract Number: 2290 • 2014 ACR/ARHP Annual Meeting

    Race and Other Risk Markers of Uveitis in a Prospective Cohort of Children with Juvenile Idiopathic Arthritis

    Sheila T. Angeles-Han1, Courtney McCracken1, Steven Yeh2, Kirsten Jenkins3, Erica Myoung4, Daneka Stryker4, Kelly A. Rouster-Stevens5, Larry B. Vogler1, Christine Kennedy6, Sampath Prahalad1 and Carolyn Drews-Botsch7, 1Emory University School of Medicine, Atlanta, GA, 2Ophthalmology, Emory University School of Medicine, Atlanta, GA, 3Children's Healthcare of Atlanta, Atlanta, GA, 4Emory University, Atlanta, GA, 5Pediatrics, Emory University School of Medicine, Atlanta, GA, 6Rheumatology Immunology, Emory Children's Center, Atlanta, GA, 7Epidemiology, Emory University School of Public Health, Atlanta, GA

    Background/Purpose Juvenile idiopathic arthritis-associated uveitis (JIA-U) can lead to poor visual outcomes.  American Academy of Pediatric guidelines recommend screening every 3 months in children with…
  • Abstract Number: 2291 • 2014 ACR/ARHP Annual Meeting

    Canakinumab in Biologic-naïve Versus Previously Biologic-Exposed Systemic Juvenile Idiopathic Arthritis Patients: Efficacy Results from a 12 Week Pooled Post Hoc Analysis

    A Grom1, P Quartier2, N Ruperto3, H.I Brunner1, K Schikler1, M Erguven3, L Goffin3, M Hofer3, T Kallinich3, K Marzan1, C Gaillez4, K Lheritier4, K Abrams5, A Martini3 and D.J Lovell1, 1PRCSG, Cincinnati, OH, 2Hôpital Necker-Enfants Malades, Paris, France, 3PRINTO-Istituto Gaslini, Genova, Italy, 4Novartis Pharma AG, Basel, Switzerland, 5Novartis Pharmaceutical Corporation, East Hanover, NJ

    Background/Purpose: Efficacy and safety of canakinumab (CAN), a selective, human,  anti-IL-1β monoclonal antibody, was previously demonstrated in 2 phase III trials.1 Out of these trials…
  • Abstract Number: 2251 • 2014 ACR/ARHP Annual Meeting

    Kneeling Disability Associated with the Treatment of Osteoarthritis: Analysis of a Copcord Study in Mexico

    Alexia Hernández-Cáceres1, Jacqueline Rodríguez-Amado1, Ingris Peláez-Ballestas2, David Vega-Morales3, Mario Garza-Elizondo1, Roberto Negrete-López1, Lorena Pérez-Barbosa1 and Janett Riega-Torres1, 1Departamento de Medicina Interna del Hospital Universitario “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Servicio de Reumatología, Departamento de Medicina Interna del Hospital Universitario “Dr. José Eleuterio González”, Universidad Autónoma de Nuevo León, Monterrey, Mexico, 2Departamento de Reumatología, Departamento de Reumatología Hospital General de México “Dr. Eduardo Liceaga”, Distrito Federal, Mexico, 3Rheumatology, Hospital Universitario UANL, Monterrey, Mexico

    Background/Purpose Osteoarthritis (OA) is the most prevalent rheumatic disease in Mexico. The core treatment, a combination of pharmacological and non-pharmacological modalities, is mainly performed in…
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