ACR Meeting Abstracts

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  • Abstract Number: 329 • 2012 ACR/ARHP Annual Meeting

    The SYK Inhibitor, Fostamatinib, Administered Alone or in Combination with Methotrexate in Rat Collagen-Induced Arthritis, Reduces Bone Erosions, Biomarkers of Cartilage/Bone Destruction, and Synovial Osteoclastogenic Cytokines

    Polly Pine1, Ayodele Apatira1, Betty Y. Chang1, Nathan Schoettler2, Elliott B. Grossbard3 and Ernest Brahn2, 1Pharmacology, Rigel Pharmaceuticals, So San Francisco, CA, 2Rheumatology, UCLA School of Medicine, Los Angeles, CA, 3Rigel Pharmaceuticals, So San Francisco, CA

    Background/Purpose: Spleen tyrosine kinase (SYK) is expressed in hematopoietic cells and is a major downstream regulator of signaling through Fcγ and immunoglobulin receptors as well…
  • Abstract Number: 289 • 2012 ACR/ARHP Annual Meeting

    Mycophenolate Mofetil and Abatacept Combination Therapy in Refractory Pediatric Systemic Lupus Erythematosus Nephritis

    Rhina Castillo1, Suhas M. Radhakrishna2, Andreas O. Reiff3 and Katherine AB Marzan1, 1Division of Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA, 2Pediatric Rheumatology, Kaiser Permananete Medical Group, Oakland, CA, 3Rheumatology, Children's Hospital Los Angeles, Los Angeles, CA

    Background/Purpose: Nephritis (LN) in pediatric systemic lupus erythematosus (pSLE) requires treatment (tx) with corticosteroids (CS) and other agents such as mycophenolate mofetil (MMF) or cyclophosphamide…
  • Abstract Number: 290 • 2012 ACR/ARHP Annual Meeting

    Long-Term Outcomes in Neonatal Lupus

    Amit Saxena1, Peter M. Izmirly2, Deborah Friedman3 and Jill P. Buyon4, 1Medicine, New York University School of Medicine, New York, NY, 2Medicine, Division of Rheumatology, NYU School of Medicine, New York, NY, 3Division of Pediatric Cardiology, New York Medical College, Valhalla, NY, 4Department of Medicine, Division of Rheumatology, New York University School of Medicine, New York, NY

    Background/Purpose: Several studies have evaluated mortality and short-term morbidity in neonatal lupus (NL), however there have been no substantive descriptions of the long term cardiac,…
  • Abstract Number: 291 • 2012 ACR/ARHP Annual Meeting

    Illness Features Associated with an Increased Risk of Mortality in Children with Juvenile Idiopathic Inflammatory Myopathies

    Adam M. Huber1, Gulnara Mamyrova2, Julia A. Lee3, Peter A. Lachenbruch4, Ira N. Targoff5, Frederick W. Miller4, Lisa G. Rider4 and Childhood Myositis Heterogeneity Study Group6, 1Dalhousie University, Halifax, NS, Canada, 2Rheumatology, George Washington University, Washington, DC, 3Environmental Autoimmunity Grp, NIEHS, Bethesda, MD, 4Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, 5Arthritis and Immunology, Oklahoma Medical Research Foun, Oklahoma City, OK, 6Bethesda, MD

    Background/Purpose: Juvenile idiopathic inflammatory myopathies (JIIM) are potentially life-threatening systemic autoimmune diseases but little in known regarding factors associated with mortality. Methods: Patients enrolled in…
  • Abstract Number: 292 • 2012 ACR/ARHP Annual Meeting

    Clinical Utility of Anti-CADM-140/Melanoma Differentiation-Associated Gene 5 Autoantibody Titers in Patients with Juvenile Dermatomyositis and Rapidly Progressive Interstitial Lung Disease

    Shinji Sato1, Norimoto Kobayashi2, Kazuko Yamazaki3 and Yasuo Suzuki4, 1Rheumatology, Tokai University School of Medicine, Isehara, Japan, 2Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan, 3Department of Pediatrics, Yokohama City University School of Medicine, Yokohama, Japan, 4Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Japan

    Background/Purpose: The presence of anti-CADM-140/ (Melanoma Differentiation-Associated Gene 5: MDA5) autoantibody is specific for adult dermatomyositis (DM), especially in patients with little or no muscle…
  • Abstract Number: 293 • 2012 ACR/ARHP Annual Meeting

    Usefulness of Cardiac Magnetic Resonance in the Assessment of Myocardial Inflammation and Fibrosis in Children Born to Mothers with Anti-SSA/Ro Antibodies: A Prospective Study of 23 Cases and 6 Controls

    Nathalie Costedoat-Chalumeau1, Alice Maltret2, Kateri Levesque1, Shelby Kutty3, Elisabeth Villain2, Phalla Ou4 and Gaëlle Guettrot-Imbert5, 1Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hopital Pitié-Salpétrière, Paris, France, 2Cardiology, Groupe Hospitalier Necker - Enfants Malades, Paris, France, 3Division of Pediatric Cardiology, university of Nebraska Medical Center and Children's Hospital ans Medical Center, Omaha, NE, USA, Omaha, 4Radiology,, Groupe Hospitalier Necker - Enfants Malades, Paris, France, 5Internal Medicine, Hopital Gabriel Montpied, Clermont-Ferrand, France

    Background/Purpose: Besides congenital heart blocks (CHB), others manifestations of cardiac neonatal lupus erythematosus syndrome (NLES) include endocardial fibroelastosis (EFE), and dilated cardiomyopathy. Recently, autopsy of…
  • Abstract Number: 294 • 2012 ACR/ARHP Annual Meeting

    Effects of Obesity On Health-Related Quality of Life in Childhood-Onset Systemic Lupus Erythematosus

    Rina Mina1, Marisa S. Klein-Gitelman2, Shannen Nelson3, Lori B. Tucker4, B. Anne Eberhard5, Nora G. Singer6, Deborah M. Levy7, Kathleen A. Haines8, Karen Onel9, Marilynn G. Punaro10, Kathleen M. O'Neil11, Michael Henrickson12, Jun Ying13 and Hermine Brunner14, 1Rheumatology, Cincinnati Children's Hospital Medical Center/ University of Cincinnati, Cincinnati, OH, 2Division of Rheumatology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 3Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, 4Pediatric Rheum/Rm K4-120, BC Childrens Hospital, Vancouver, BC, Canada, 5Cohen Children's Hospital Medical Center, New Hyde Park, NY, 6Medicine, Division of Rheumatology, MetroHealth Medical Center, Cleveland, OH, 7Rheumatology, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada, 8Department of Pediatrics, Joseph M Sanzari Children’s Hospital, Hackensack University Medical Center, Hackensack, NJ, 9Pediatric Rheumatology, University of Chicago, Chicago, IL, 10Pediatric Rheumatology, Texas Scottish Rite Hospital, Dallas, TX, 11Pediatric Rheumatology, Riley Hospital for Children, Indianapolis, IN, 12MLC 4010, Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 13Medicine-Internal Medicine-General Medicine, University of Cincinnati, Cincinnati, OH, 14Cincinnati Children's Hospital, Cincinnati, OH

    Background/Purpose: Obesity in adults with Systemic Lupus Erythematosus (SLE) is associated with an added risk of cardiovascular disease, decreased health-related quality of life (HRQOL), and…
  • Abstract Number: 295 • 2012 ACR/ARHP Annual Meeting

    Brain Biopsy Diagnosis in Magnetic Resonance Imaging Negative Childhood Primary Angiitis of the Central Nervous System

    Senq-J Lee1, Cynthia Hawkins2, Suzanne Laughlin3, Shehla Sheikh4 and Susanne M. Benseler4, 1Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 2Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada, 3Diagnostic Imaging, The Hospital for Sick Children, Toronto, ON, Canada, 4Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada

    Background/Purpose: Primary CNS vasculitis in childhood (cPACNS) is a devastating inflammatory brain disease mandating rapid and accurate diagnostic evaluation, in order to optimize neurologic outcomes. …
  • Abstract Number: 296 • 2012 ACR/ARHP Annual Meeting

    A Brazilian Multicenter Study of 71 Children and Adolescents with Takayasu’s Arteritis

    Maria Teresa Terreri1, Gleice Clemente2, Clovis Silva3, Silvana Sacchetti4, Adriana M. Sallum5, Lucia M. A. Campos6, Maria Carolina Santos4, Flavio Sztajnbok7, Rozana Gasparello de Almeida8, Virginia P. Ferriani9, Blanca E. Bica10, Teresa Robazzi11, Marcia Bandeira12, Andre Cavalcanti13, Marise Lessa14, Sheila K. Feitosa de Oliveira15 and Maria Odete Hilario16, 1Pediatrics, Universidade Federal de São Paulo / UNIFESP, Sao Paulo, Brazil, 2Pediatrics, Universidade Federal de São Paulo/UNIFESP, São Paulo, Brazil, 3Pediatric Rheumatology, MD; PhD, São Paulo-SP, Brazil, 4Pediatrics, Santa Casa de Misericórdia de São Paulo, São Paulo, Brazil, 5Pediatric Rheumatology Unit, Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo-SP, Brazil, 6Pediatric Rheumatology, University of São Paulo Medical School, São Paulo, Brazil, 7PRINTO, Genoa, Italy, 8Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, 9Pediatrics, FMUSP-Ribeirao Preto, Ribeirao Preto, Brazil, 10International Investigator Consortium for MAS Diagnostic Criteria, Rio de Janeiro, Brazil, 11Pediatrics, Universidade Federal da Bahia, Salvador, Brazil, 12Pediatrics, Hospital Infantil Pequeno Príncipe, Curitiba, Brazil, 13Universidade Federal de Pernambuco, Recife, Brazil, 14Pediatrics, Instituto de Pediatria e Puericultura Martagão Gesteira (IPPMG) da Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil, 15Pediatric Rheumatology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, 16Pediatrics, Universidade Federal de Sao Paulo / UNIFESP, Sao Paulo, Brazil

    Background/Purpose: Takayasu’s arteritis is a chronic granulomatous disease that affects the vascular wall of the large arteries and can result in end organ damage. It…
  • Abstract Number: 297 • 2012 ACR/ARHP Annual Meeting

    Effectiveness of Intravenous Cyclophosphamide in Severe or Refractory Juvenile Dermatomyositis – A National Cohort Study: UK and Ireland

    Elena Moraitis1, Katie Arnold2, Clarissa Pilkington3 and Juvenile Dermatomyositis Research Group4, 1Rheumatology, Great Ormond Street Hospital, London, United Kingdom, 2Infection, Inflammation and Rheumatology Section, UCL Institute for Child Health, London, United Kingdom, 3Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 4London, United Kingdom

    Background/Purpose: Juvenile dermatomyositis (JDM) is a rare autoimmune vasculopathy affecting primarily the muscle and skin, but can also involve other organs. Early and aggressive treatment improves outcome and prevents complications. Cyclophosphamide has been used as a second-line agent in the treatment of severe or refractory JDM. The published literature on the effectiveness of cyclophosphamide in JDM is limited to a small case series and case reports.The objective of the study is to describe the response to cyclophosphamide in the patients with severe or refractory JDM. Methods:  56 patients treated with cyclophosphamide between years 2000-2011 were identified in the JDM National (UK and Ireland) Cohort Biomarker Study and Repository for Idiopathic Inflammatory Myopathies. 8 patients were excluded due to incomplete data or too short follow up. For the 48 patients included, demographics, myositis core outcome variables, skin measures, laboratory measures, steroid dose and other treatments were recorded at baseline, time 6, 12, 18, 24 months and last follow up post commencement of the drug.Results:  Indications for starting cyclophosphamide were ulcerative or severe skin disease, profound muscle weakness, lung disease, gastro-intestinal vasculopathy or refractory disease. Previous medications were steroids and Methotrexate for 47 patients and steroids and Cyclosporin for 1 patient. All patients starting with muscle weakness (n=44) significantly improved at time12, and the gains were maintained at follow up (see table1). Physician VAS was available for 32 patients and these all improved by 12 and 24 months, and for 31 remained stable at follow up.  At last follow up 26/46(56%) had no rash, 32/46(69%) had normal nailfolds, 37/45(82%) had no Gottron’s, and calcinosis improved in 9/14(64%). Conclusion: This study, the largest to date, demonstrated significant improvement in both muscle and skin domains in patients with JDM treated with intravenous cyclophosphamide.Cyclophosphamide appears effective  in the treatment of severe or refractory JDM.Table 1Table 2
  • Abstract Number: 298 • 2012 ACR/ARHP Annual Meeting

    Childhood-Onset Predicts Increased Disease Damage and Steroid Toxicity in a Cohort of Adults with Systemic Lupus Erythematosus

    Erica F. Lawson1, Laura Trupin2, Jinoos Yazdany3, Aimee Hersh4, Emily von Scheven5 and Edward H. Yelin6, 1Pediatrics, University of California, San Francisco, San Francisco, CA, 2Medicine, University of California San Francisco, San Francisco, CA, 3Medicine, University of California, San Francisco, San Francisco, CA, 4Pediatrics, University of Utah, Salt Lake City, UT, 5Pediatric Rheumatology, University of California San Francisco, San Francisco, CA, 6Arthritis Research Group, University of California, San Francisco, San Francisco, CA

    Background/Purpose: While previous work has shown that adults with childhood-onset systemic lupus erythematosus (cSLE) have increased risk of mortality, renal disease and myocardial infarction as…
  • Abstract Number: 299 • 2012 ACR/ARHP Annual Meeting

    Reduction of Cerebral and Corpus Callosum Volumes in Childhood-Onset Systemic Lupus Erythematosus. A Volumetric Magnetic Resonance Imaging Analysis

    Aline T. Lapa1, Wesley G. Ferreira1, Mariana Postal1, Nailu A. Sinicato1, Roberto Marini2, Fernando Cendes3 and Simone Appenzeller4, 1Medicine, State University of Campinas, Campinas, Brazil, 2State University of Campinas, Campinas, Brazil, 3Neurology, State University of Campinas, Campinas, Brazil, 4Medicine, Faculty of Medical Science, State University of Campinas Unicamp, São Paulo, Brazil

    Background/Purpose: Cerebral atrophy has been described to occur in SLE with variable frequency. Aging, systemic diseases, corticosteroid use and central nervous system (CNS) involvement may…
  • Abstract Number: 300 • 2012 ACR/ARHP Annual Meeting

    Prevalence and Clinical Significance of Hippocampal Atrophy in Childhood-Onset Systemic Lupus Erythematosus

    Aline T. Lapa1, Renata Brabosa2, Mariana Postal1, Nailu A. Sinicato1, Roberto Marini3, Fernando Cendes4 and Simone Appenzeller5, 1Medicine, State University of Campinas, Campinas, Brazil, 2Medicine, Faculdade de Ciencias Medicas, Universidade Estadual de Campinas, Campinas, Germany, 3State University of Campinas, Campinas, Brazil, 4Neurology, State University of Campinas, Campinas, Brazil, 5Medicine, Faculty of Medical Science, State University of Campinas Unicamp, São Paulo, Brazil

    Background/Purpose: Hippocampal atrophy is associated with corticosteroid use and may be related to cognitive impairment in systemic lupus erythematosus (SLE).Objectives: To determine the prevalence of…
  • Abstract Number: 301 • 2012 ACR/ARHP Annual Meeting

    Decreased Frequency of Dystrophic Calcifications in Children with Juvenile Dermatomyositis: A 10-Year Study

    Lauren M. Pachman1, Gabrielle A. Morgan2, Megan L. Curran1, Lori J. Ferguson3 and Chiang-Ching Huang4, 1Division of Pediatric Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 2Cure JM Myositis Center, Ann & Robert H. Lurie Children's Hospital of Chicago Research Center, Chicago, IL, 3Children's Hospital of Chicago Research Center, Cure JM Myositis Center, Chicago, IL, 4Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL

    Background/Purpose: In Juvenile Dermatomyositis (JDM), dystrophic calcifications, associated with increased morbidity and mortality, have been reported for 20-30% of patients. There are few laboratory indicators…
  • Abstract Number: 302 • 2012 ACR/ARHP Annual Meeting

    Accuracy of Systemic Lupus International Collaborating Clinics Classiffication Criteria Applied to Juvenile Systemic Lupus Erythematosus Patients

    Marìa M. Katsicas1, Ezequiel Borgia2, Ileana Villarroel2 and Ricardo Russo3, 1Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, 2Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Caba, Argentina, 3Paediatric Rheumatology International Trials Organization (PRINTO), Istituto Giannina Gaslini, Genova, Italy

    Background/Purpose: Systemic lupus erythematosus (SLE) is a prototype autoimmune disease. The most widely used classification criteria for SLE were those developed by the American College…
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