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  • Abstract Number: 1326 • 2018 ACR/ARHP Annual Meeting

    Does Testing for SAA Is More Beneficial Than CRP for the Follow-up of Patients with Familial Mediterranean Fever ?

    Serdal Ugurlu1, Oguzhan Selvi2, Bilgesu Ergezen1 and Huri Ozdogan1, 1Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 2Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Background/Purpose: In order to follow subclinical inflammation and adjust the therapy for an optimal disease control, clinicians seek for readily accessible, affordable and reproducible markers.…
  • Abstract Number: 1327 • 2018 ACR/ARHP Annual Meeting

    The Effect of Etoposide on Inducing Remission in Refractory Adult-Onset Still’s Disease: A Retrospective, Single-Center Study

    Haiting Wang1, Xiaodong Wang1, Ting Li1 and Shuang Ye2, 1Department of Rheumatology, Renji Hospital South Campus, Shanghai Jiao Tong University School of Medicine, Shanghai, China, 2Rheumatology, Ren Ji Hospital, South Campus, School of Medicine, Shanghai Jiaotong University, Shanghai, China

    Background/Purpose: Adult onset Still¡¯s disease (AOSD) is a rare inflammatory disorder of unknown etiology. Macrophage activation syndrome (MAS) is a life-threatening complication and has been…
  • Abstract Number: 1328 • 2018 ACR/ARHP Annual Meeting

    Recurrent Pericarditis: A Challenge in Autoinflammatory Disease Clinic and the Role of Anakinra

    Blas Betancourt1, Amanda Ombrello2, Ananta Subedi3, Patrycja M. Hoffmann2 and Daniel L. Kastner4, 1National Institute of Arthritis, Musculoskeletal and Skin Disease (NIAMS), Bethesda, MD, 2NHGRI, National Institutes of Health, Bethesda, MD, 3National Institutes of Health, NIAMS, Bethesda, MD, 4Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD

    Background/Purpose: Recurrent pericarditis is a common complication following an acute episode of pericarditis affecting about 15-30% of the cases. The etiology of this condition is…
  • Abstract Number: 1329 • 2018 ACR/ARHP Annual Meeting

    Combination of Methotrexate and Leflunomide in Refractory Chronic Adult Onset Still’s Disease: Case Series and Literature Review

    Eunyoung Emily Lee and Jin Kyun Park, Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea, Republic of (South)

    Background/Purpose: To demonstrate the efficacy of methotrexate (MTX) and leflunomide (LEF) combination therapy in refractory chronic adult onset Still¡¯s disease (AOSD). Methods: This is a…
  • Abstract Number: 1330 • 2018 ACR/ARHP Annual Meeting

    Dyskeratotic Cells in Persistent Pruritic Skin Lesions Are Apoptotic and Associated with High Levels of Serum IL-18, and Possibly Predict the Outcomes in Adult-Onset Still’s Disease

    Hirofumi Nishikawa1, Yoshinori Taniguchi1, Natsuki Maeda1, Satoshi Inotani1, Kimiko Nakajima2, Kaoru Arii3 and Yoshio Terada1, 1Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School Hospital, Nankoku, Japan, 2Dermatology, Kochi Medical School, Nankoku, Japan, 3Internal Medicine, Kochi Red Cross Hospital, Kochi, Japan

    Background/Purpose: To assess the clinical significance of dyskeratotic cells (DCs) in skin lesions of adult-onset Still’s disease (AOSD). Methods: We assessed clinical characteristics, serum markers,…
  • Abstract Number: 1331 • 2018 ACR/ARHP Annual Meeting

    Moving from Dermatomyositis Associated with Anti-Melanoma Differentiation-Associated Gene 5 Antibody to Anti-Melanoma Differentiation-Associated Gene 5 Syndrome

    Yves Allenbach1, Yurdagul Uzunhan2, Ségolène Toquet3, Gaëlle Leroux4, Laure Gallay5, Aude Rigolet6, Baptiste Hervier6, Nicolas Champtiaux6, Mathieu Vautier6, Perrine Guillaume6, Nicolas Limal7, Alain Meyer8, Christophe Deligny9, Bernard Bonnotte10, Hervé Devilliers11, Sylvain Audia12, Maxime Samson13, Amélie Servettaz14, Nathalie Costedoat15, David Saadoun16, elisabeth diot17, Alice Berezne18, Arsène Mékinian19, Kuberaka Mariampillai20, Hilario Nunes2 and Olivier Benveniste6, 1Internal Medicine and Clinical Immunology, Sorbonne University - Assistance Public - Hopitaux de Paris, Pitié-Salpêtrière University Hospital, Paris, France, 2Pulmonary diseases department, Avicenne Hospital (AP-HP), Bobigny, France, 3Sorbonne Universités, UPMC Univ Paris 06, INSERM UMRS_974, CNRS FRE 3617, Center of Research in Myology., Paris, France, 4Internal Medicine, Pitié-Salpêtrière University Hospital, Paris, France, 5Internal Medicine, Université de Lyon - CHU Lyon, Lyon, France, 6Department of Internal Medicine and Clinical Immunology and Inflammation-Immunopathology-Biotherapy Department (I2B), Pitié-Salpêtrière University Hospital, Assistance Publique-Hôpitaux de Paris, East Paris Neuromuscular Diseases Reference Center, Paris, France, 7Internal Medicine, APHP - Henri Mondor Hospital, Paris, France, 815Département de Rhumatologie, Centre de Référence des Maladies Auto-immunes Rares, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, 9Zobda Quitman Hospital, Rheumatology and Internal Medicine, Fort de France, Martinique, 10Dijon, Dijon, France, 11Service de Médecine Interne et Maladies Systémiques, CHU de Dijon, Dijon, France, 12Department of Internal Medicine and Clinical Immunology, Hôpital François Mitterrand, CHU de Dijon; INSERM, UMR1098, University of Bourgogne Franche-Comté, FHU INCREASE, Dijon, France, 13Dijon University Hospital, Dijon, France, 14Internal Medicine, CHU - Reims University, Reims, France, 15Internal Medicine, Paris Descartes Université - APHP, paris, France, 16Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), F-75005, Paris, France; INSERM, UMR_S 959, F-75013, Paris, France; CNRS, FRE3632, F-75005, Paris, France; AP-HP, Groupe Hospitalier, Paris, France, 17Internal Medicine, Université Tours - CHU, Tours, France, 18Internal Medicine, CH Annecy, Annecy, France, 19Service de médecine interne. Hôpital Saint-Antoine., Paris, France, 20Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Neuro-Musculaires Paris Est, Assistance Publique – Hôpitaux de Paris (AP-HP), Hôpital Universitaire Pitié-Salpêtrière, Paris, France

    Background/Purpose: Dermatomyositis (DM) is a heterogenous group of diseases ranging from skin limited disorders to non-specific auto-immune diseases with patients suffering from additional extra-cutaneous manifestations.…
  • Abstract Number: 1332 • 2018 ACR/ARHP Annual Meeting

    Proteomic Discovery Analysis Identifies Unique Proteins and Pathways Correlating with Different Clinical Activity and Damage Measures in Juvenile Dermatomyositis (JDM)

    Hanna Kim1, Angelique Biancotto2, Foo Cheung2, Terrance P. O'Hanlon3, Yan Huang4, Frederick W. Miller3, Raphaela Goldbach-Mansky4 and Lisa G. Rider5, 1Pediatric Translational Research Branch, NIAMS, NIH, Bethesda, MD, 2Center for Human Immunology Autoimmunity and Inflammation (CHI), NIAID, NIH, Bethesda, MD, 3Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, 4Translational Autoinflammatory Disease Section (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, 5Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD

    Background/Purpose: JDM is a complex heterogeneous autoimmune disease.  To define biomarkers and better understand JDM pathogenesis, aptamer-based proteomic technology was used to mine the serum…
  • Abstract Number: 1333 • 2018 ACR/ARHP Annual Meeting

    Abnormal Function of High Density Lipoproteins in Idiopathic Inflammatory Myopathies

    Sangmee Bae1, Jennifer Wang1, Ani Shahbazian1, Buzand Oganesian2, Ilana Golub1, Srinivasa T. Reddy1 and Christina Charles-Schoeman3, 1University of California, Los Angeles, Los Angeles, CA, 2University of California, Los Angeles, Los angeles, CA, 3Rheumatology, UCLA David Geffen School of Medicine, Los Angeles, CA

    Background/Purpose: Damage to the vascular endothelium is strongly implicated in the pathogenesis of dermatomyositis (DM) and to a lesser degree other idiopathic inflammatory myopathies (IIM).…
  • Abstract Number: 1334 • 2018 ACR/ARHP Annual Meeting

    Low Density Granulocytes As Biomarkers of Disease Activity and Damage in Patients with Idiopathic Inflammatory Myopathies

    Jiram Torres-Ruíz1, Araceli Leal-Alanis2, Ricardo Vazquez-Rodriguez3, Mario René Alvarado-Lara3, Edgar Rafael Carazo-Vargas3, José Luis Maravillas-Montero4, Jorge Alcocer-Varela5 and Diana Gómez-Martín5, 1Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición, Salvador Zubirán, Mexico City, Mexico, 2Internal Medicine, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico, Mexico, 3Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico, Mexico, 4Red de Apoyo a la Investigación, UNAM, Mexico, Mexico, 5Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico

    Background/Purpose: Low density granulocytes (LDGs) are a subset of neutrophils that spontaneously produce neutrophil extracellular traps (NETs) and type I IFN. The latter correlates with…
  • Abstract Number: 1335 • 2018 ACR/ARHP Annual Meeting

    Extracellular Vesicles Induce Pro-Inflammatory Cytokines in Dermatomyositis

    Krisha Desai1,2, Majid Zeidi3,4, Ming-Lin Liu1,2 and Victoria P. Werth1,2, 1Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, 2Department of Dermatology, Corporal Michael J. Crescenz VAMC, Philadelphia, PA, 3University of Pennsylvania, Philadelphia, PA, 4Philadelphia Veterans Affairs Medical Center, Philadelphia, PA

    Background/Purpose: Extracellular vesicles (EVs) are micron-scale bilayer membrane vesicles released from almost all cell types under activation or apoptosis. They have been detected in various…
  • Abstract Number: 1336 • 2018 ACR/ARHP Annual Meeting

    Functional, Radiographic and Serologic Correlates of Anti-SSA52 Kd – Associated Interstitial Lung Disease

    Estefania Calle Botero1, Benjamin Wang2, Juan J Maya2, Isabel Mira-Avendano3 and Andy Abril2, 1Rheumatology, Universidad de Antioquia, Medellin, Colombia, 2Rheumatology, Mayo Clinic Florida, Jacksonville, FL, 3Pulmonary and Critical Care, Mayo Clinic Florida, Jacksonville, FL

    Background/Purpose: Autoantibodies against the SSA52 Kd protein (anti-SSA52) have been cataloged as myositis-associated antibodies, occurring in up to 30% of cases idiopathic inflammatory myopathies (IIM),…
  • Abstract Number: 1337 • 2018 ACR/ARHP Annual Meeting

    Myocardial Fatty Acid Metabolism and Perfusion Mismatch in Scintigraphy Predicts Worse Prognosis in Clinically Amyopathic Dermatomyositis

    Harunobu Iida1, Hironari Hanaoka1, Kana Ishimori2, Tomofumi Kiyokawa1, Yukiko Takakuwa1 and Kimito Kawahata1, 1Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan, 2Division of Rheumatology and Allergy, Department of Internal Medicine, St. Marianna University School of Medicine, Kawasaki, Japan

    Background/Purpose: Clinically amyopathic dermatomyositis (CADM) patients are frequently manifested by rapidly progressive interstitial lung disease (ILD) and associated with a poor prognosis. Recently cardiac involvement…
  • Abstract Number: 1338 • 2018 ACR/ARHP Annual Meeting

    Validation of the Diagnostic Accuracy of Myositis-Related Antibodies in a Large Patient-Cohort

    Angelika Lackner1, Viktoria Tiefenthaler2, Jalia Mirzayeva2, Winfried Graninger3 and Martin Stradner4, 1Rheumatology and Immunology, Medical University of Graz, Graz, Austria, 2Department of Rheumatology & Immunology, Medical University of Graz, Graz, Austria, 3Division of Rheumatology and Immunology, Medical University of Graz, Graz, Austria, 4Department of Rheumatology and Immunology, Medical University of Graz, Graz, Austria

    Validation of the diagnostic accuracy of myositis-related antibodies in a large patient-cohortLackner, A; Tiefenthaler, V; Mirzayeva, J; Graninger, W; Stradner, MH Background/Purpose: Myositis-specific antibodies (MSA) and…
  • Abstract Number: 1339 • 2018 ACR/ARHP Annual Meeting

    Myositis Specific Anti-Histidyl tRNA Synthetase (HisRS) Autoantibodies Display High Reactivity Against Hisrs Conformational Epitopes and Associate with Lung and Joint Involvement

    Catia Fernandes-Cerqueira1,2, Nuria Renard1,2, Antonella Notarnicola1,2, Edvard Wigren1,2,3, Per-Johan Jakobsson2,4, Susanne Graslund1,2,3 and Ingrid E. Lundberg1,2, 1Department of Medicine, Division of Rheumatology, Karolinska Institutet, Stockholm, Sweden, 2Center for Molecular Medicine, Stockholm, Sweden, 3Structural Genomics Consortium, Stockholm, Sweden, 4Rheumatology Unit, Dept. of Medicine, Karolinska Institutet, Stockholm, Sweden

    Background/Purpose: Autoimmune myositis (rheumatic muscle inflammation) associated with interstitial lung disease (ILD) and arthritis is strongly correlated with the presence of anti-histidyl tRNA synthetase (HisRS)…
  • Abstract Number: 1340 • 2018 ACR/ARHP Annual Meeting

    Anti-Aminoacyl-tRNA-Synthetase Antibodies Which Are Positive By ELISA but Negative By RNA-Immunoprecipitation Suggest Different Antigen Recognition and Clinical Relevance Different from Typical Anti-Synthetase Syndrome

    Tsuneo Sasai1, Ran Nakashima2, Yuki Ishikawa3, Takuya Isayama4 and Tsuneyo Mimori2, 1Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Japan, Kyoto, Japan, 2Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan, 3One Joslin Place, Joslin Diabetes Center, Harvard Medical School, Boston, MA, 4Medical & Biological Laboratories CO.,LTD., Nagoya, Japan

    Background/Purpose: Anti-aminoacyl-tRNA-synthetase (ARS) antibodies are myositis specific autoantibodies and associated with common clinical characteristics called anti-synthetase syndrome (ASS). Recently anti-ARS detecting enzyme-linked immunosorbent assay (ELISA)…
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