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  • Abstract Number: 241 • 2016 ACR/ARHP Annual Meeting

    Analysis of 84 Patients with IgG4-Related Disease and Malignancy

    Kazunori Yamada1,2, Ichiro Mizushima2, Hideki Nomura3 and Mitsuhiro Kawano2, 1Department of Advanced Research in Community Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan, 2Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan, 3Department of General Medicine, Kanazawa University Hospital, Kanazawa, Japan

    Background/Purpose: IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by an elevated serum level of IgG4, infiltration of IgG4-positive cells in affected organs and…
  • Abstract Number: 242 • 2016 ACR/ARHP Annual Meeting

    Clinical and Laboratory Features of IgG4-Related Retroperitoneal Fibrosis/Periarteritis in Japan: Retrospective Multicenter Study of 99 Cases

    Ichiro Mizushima1, Satomi Kasashima2, Motohisa Yamamoto3, Takako Saeki4, Kazunori Yamada5, Dai Inoue6, Fuminori Kasashima7, Yasushi Matsumoto7, Eisuke Amiya8, Kenji Notohara9, Yasuharu Sato10, Yoh Zen11, Shigeyuki Kawa12, Mitsuhiro Kawano1 and Nobukazu Ishizaka13, 1Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Japan, 2Department of Clinical Laboratory and Pathology, National Hospital Organization, Kanazawa Medical Center, Kanazawa, Japan, 3First Department of Internal Medicine, Sapporo Medical University School of Medicine, Sapporo, Japan, 4Department of Internal Medicine, Nagaoka Red Cross Hospital, Nagaoka, Japan, 5Department of Advanced Research in Community Medicine, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan, 6Department of Radiology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan, 7Department of Cardiovascular Surgery, National Hospital Organization, Kanazawa Medical Center, Kanazawa, Japan, 8Department of Cardiovascular Medicine, University of Tokyo Graduate School of Medicine, Tokyo, Japan, 9Department of Pathology, Kurashiki Central Hospital, Kurashiki, Japan, 10Department of Pathology, Okayama University Graduate School of Medicine, Okayama, Japan, 11Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, Kobe, Japan, 12Center for Health, Safety and Environmental Management, Shinshu University, Matsumoto, Japan, 13Department of Cardiology, Osaka Medical College, Osaka, Japan

    <span">Background/Purpose:  IgG4-related disease (IgG4-RD) is a recently recognized systemic inflammatory disorder that can affect many organs. It frequently causes retroperitoneal/periarterial lesions, which are referred to…
  • Abstract Number: 243 • 2016 ACR/ARHP Annual Meeting

    Eosinophilic Angiocentric Fibrosis : A Mimic of Vasculitis in IgG4 Related Disease Spectrum

    Raphaël Lecomte1, Antoine Néel2, Olivier Malard3, Jérôme Martin4, Michael Hénoux3, Elisabeth Cassagnau5 and Mohamed Hamidou6,7, 1Internal Medicine Department, CHU Nantes, Nantes, France, 2Department of Internal Medicine, Nantes University Hospital, Nantes, France, 3ENT, ENT department, Nantes University Hospital, Nantes, France, 4Immunology laboratory, Immunology laboratory, Nantes University Hospital, Nantes, France, 5Histopathology, Histopathology department, Nantes University Hospital, Nantes, France, 6Hotel Dieu, Service de médecine interne, Hôpital Universitaire de Nantes, Nantes, France, Nantes, France, 7Internal Medicine Department, Internal Medicine Department, Nantes University Hospital, Nantes, France

    Background/Purpose: Eosinophilic angiocentric fibrosis (EAF) is a rare localized fibro-inflammatory lesion involving usually upper respiratory tract and the orbit. It could mimic ENT manifestations of…
  • Abstract Number: 244 • 2016 ACR/ARHP Annual Meeting

    Leflunomide and Glucocorticoids Combination Therapy for the Induction and Maintenance of Remission in Patients with IgG4-Related Disease

    Yiwen Wang1, Dai Gao1, Gui Luo2, Kunpeng Li1, Zheng Zhao1 and Jian Zhu1, 1Rheumatology, Chinese PLA General Hospital, Beijing, China, 2Chinese PLA General Hospital, Beijing, China

    Background/Purpose: Good response could be observed after applying glucocorticoids (GCs) in patients with IgG4-related disease (IgG4-RD), however, the risk of disease relapse was reported relatively…
  • Abstract Number: 245 • 2016 ACR/ARHP Annual Meeting

    Efficacy of Cyclophosphamide Therapy in Idiopathic Retroperitoneal Fibrosis in a Retrospective Monocentric Analysis

    Bimba F. Hoyer1, Caroline Kumpert2, Marcus Makowski3, Birgit Rudolph4, U. Schneider5, Gerd R. Burmester6 and Falk Hiepe7, 1Charité University Medicine, Department of Medicine/Rheumatology and Clinical Immunology and German Rheumatism Research Centre Berlin (DRFZ), Berlin, Germany, 2department of Rheumatology and clinical Immunology, Charité University Medicine Berlin,, Berlin, Germany, 3Department for Radiology, Charité Universityhospital, Berlin, Germany, 4Department for pathology, Charité Universityhospital, Berlin, Germany, 5Rheumatology and Clinical Immunology, Charité, Rheumatology and Clinical Immunology, Berlin, Germany, 6Charité – University Medicine Berlin, Berlin, Germany, 7Charité – Universitätsmedizin, Berlin, Germany

    Background/Purpose: Ormond’s disease (idiopathic retroperitoneal fibrosis ,IRF) is a rare disease. Main clinical symptom is compression of the ureters leading to renal failure. A majority…
  • Abstract Number: 246 • 2016 ACR/ARHP Annual Meeting

    Efficacy of Colchicine and IL-1 Inhibitors in Amyloidosis Associated with Familial Mediterranean Fever: A Retrospective Analysis

    Bahtiyar Toz1, Seher Tecer2, Emin Oğuz1, Murat Erdugan1, Bahar Artim-Esen1, Sevil Kamali3, Murat Inanc4, Lale Ocal3, Burak Erer1 and Ahmet Gul1, 1Department of Internal Medicine, Division of Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey, 2Department of Internal Medicine, Istanbul faculty of medicine Istanbul University, Istanbul, Turkey, 3Department of Internal Medicine, Rheumatology Division, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey, 4Department of Internal Medicine, Division of Rheumatology, Istanbul University, Istanbul Faculty of Medicine, İstanbul, Turkey

    Background/Purpose: Familial Mediterranean fever (FMF), the most common form of hereditary autoinflammatory diseases, is associated with increased risk for secondary (AA) amyloidosis. We herein aimed…
  • Abstract Number: 247 • 2016 ACR/ARHP Annual Meeting

    ANTI-Interleukin 1 Therapy in FMF Amyloidosis: A Single Center Experience

    Serdal Ugurlu, Bilgesu Ergezen and Huri Ozdogan, Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Background/Purpose: Recently there is increasing number of reports pointing out the efficacy of anti-interleukin 1(anti-IL1)therapy to control AA amyloidosis secondary to autoinflammatory diseases. Here we…
  • Abstract Number: 248 • 2016 ACR/ARHP Annual Meeting

    Disease Severity and High Attack Frequency Under Colchicine Treatment Is Associated with Increased Carotid Intima Media Thickness in FMF

    Murat Karabacak1, Ali Ugur Unal2, Gulsen Ozen3, Zeynep Erturk4, Yasemin Yalcinkaya3, Zeynep Komesli1, Nevsun Inanc3, Pamir Atagunduz3 and Haner Direskeneli5, 1Marmara University Faculty of Medicine, Istanbul, Turkey, 2Marmara University, School of Medicine, Rheumatology, Istanbul, Turkey, 3Department of Rheumatology, Marmara University Faculty of Medicine, Istanbul, Turkey, 4Rheumatology, Marmara University Faculty of Medicine, Istanbul, Turkey, 5Rheumatology, Marmara University, School of Medicine, Istanbul, Turkey

    Background/Purpose: Cardiovascular (CV) risk assessment is infrequently performed in Familial Mediterranean Fever (FMF), an autoinflammatory disorder with only acute attacks of inflammation. As a surrogate…
  • Abstract Number: 249 • 2016 ACR/ARHP Annual Meeting

    MiR-204-3p Associates with an Increased Level of IL-6 in Familial Mediterranean Fever By Targeting the PIK3 Signaling Pathway

    Tomohiro Koga1, Kiyoshi Migita2, Akihiro Yachie3, Yukitaka Ueki4, Kazunaga Agematsu5, Junya Masumoto6, Koh-ichiro Yoshiura7, Katsumi Eguchi8 and Atsushi Kawakami1, 1Unit of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, 2Department of Rheumatology and Clinical Research Center, Nagasaki Medical Center, Omura, Japan, 3Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical, and Health Sciences, Kanazawa University, Kanazawa, Japan, 4Rheumatic and Collagen Disease Center, Sasebo Chuo Hospital, Sasebo, Japan, 5Department of Infectious Immunology, Shinshu University, Graduate School of Medicine, Shinshu, Japan, 6Department of Pathology, Division of Analyticalpathology, Ehime University Graduate School of Medicine, Toon, Japan, 7Department of Human Genetics, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan, 8Department of Rheumatology and Metabolism, Sasebo City General Hospital, Sasebo, Japan

    Background/Purpose: Familial Mediterranean fever (FMF) is caused by a number of mutations of the MEFV gene, coding for a protein named pyrin that acts as…
  • Abstract Number: 250 • 2016 ACR/ARHP Annual Meeting

    Application of the 2016 European Leage Against Rheumatism (EULAR) /American College of Rheumatology (ACR)/Paediatric Rheumatology International Trials Organisation (PRINTO) Classification Criteria of Macrophage Activation Syndrome in Patients with Adult Onset Still’s Disease

    Sung Soo Ahn1, Seung Min Jung2, Sang-Won Lee1, Yong-Beom Park1 and Jason Jungsik Song1, 1Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea, 2Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea, The Republic of

    Background/Purpose:  Macrophage activation syndrome (MAS) is acute systemic inflammation arising in the context of various autoimmune and autoinflammatory conditions, which is often life-threatening. In 2016,…
  • Abstract Number: 251 • 2016 ACR/ARHP Annual Meeting

    Persistent Pruritic Skin Lesions with Dyskeratotic Cells in Upper Layer of Epidermis Are Specific and Associated with High Levels of Serum IL-18 in Adult-Onset Still’s Disease

    Natsuki Maeda1,2, Yoshinori Taniguchi3, Kimiko Nakajima4, Yoshiko Shimamura5, Hirofumi Nishikawa6, Shuichi Nakayama4, Shigetoshi Sano4, Shimpei Fujimoto6 and Yoshio Terada5, 1Endocrinology,Metabolism,Nephrology and Rheumatology, Kochi Medical School, Nankoku, Japan, 2Dermatology, Kochi Medical School, Nankoku, Japan, 3Endocrinology, Metabolism,Nephrology and Rheumatology, Kochi University, Kochi, Japan, 4Kochi Medical School, Nankoku, Japan, 5Kochi University, Nankoku, Japan, 6Endocrinology, Metabolism, Nephrology and Rheumatology, Kochi Medical School, Nankoku, Japan

    Background/Purpose:  Adult-onset Still’s disease (AOSD) is an acute and systemic inflammatory disorder that is characterized by high spiking fever, evanescent rash, arthralgia/arthritis and hyperferritinemia. However,…
  • Abstract Number: 252 • 2016 ACR/ARHP Annual Meeting

    Cytokine Profiles of Korean Patients with Adult Onset Still’s Disease Treated with Biologic Agents

    Seung Taek Song1, SuMan Kang2, Sung Won Lee3, Seoung Wan Nam2, Hyukhee Kwon2 and Dae-Hyun Yoo4, 1Division of Rheumatology, Department of Internal Medicine, Cheongju St. Mary's Hospital, Cheongju, Korea, The Republic of, 2Division of Rheumatology, Department of Internal Medicine, Hanyang University Hospital for Rheumatic Diseases, Seoul, Korea, The Republic of, 3Department of Rheumatology, Division of Rheumatology, Department of Internal Medicine, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea, 4Division of Rheumatology, Department of Internal Medicine, Hanyang University Hospital for Rheumatic Diseases, Seoul, South Korea

    Background/Purpose:  Adult onset Still’s disease (AOSD) is a rare inflammatory disorder of unknown etiology. Several studies have reported that pro-inflammatory cytokines including interleukin (IL)-1, IL-6,…
  • Abstract Number: 253 • 2016 ACR/ARHP Annual Meeting

    Corticosteroid-Free Tocilizumab Monotherapy for Adult Onset Still’s Disease: Results in Six Month

    Tsuneo Kondo1, Yusuke Okada2, Akiko Shibata1, Kentaro Chino1, Ayumi Okuyama1, Hirofumi Takei1 and Koichi Amano1, 1Department of Rheumatology and Clinical Immunology, Saitama Medical Center, Saitama Medical University, Saitama, Japan, 2Saitama Medical Center, Saitama Medical University, Kawagoe, Japan

    Background/Purpose: To assess the efficacy and safety of tocilizumab (TCZ) monotherapy for the induction therapy of adult onset Still’s disease (AOSD) in a prospective, single-arm, single-center, cohort,…
  • Abstract Number: 254 • 2016 ACR/ARHP Annual Meeting

    NLRP12 Autoinflammatory Disease: A Chinese Case Series and Literature Review

    Min Shen1, Lin Tang2, Xiaochun Shi3, Xiaofeng Zeng1 and Qingping Yao4, 1Rheumatology, Peking Union Medical College Hospital, Beijing, China, 2Rheumatology, the Second Affiliated Hospital of Chongqing Medical University, Chongqing, China, 3Infectious Disease, Peking Union Medical College Hospital, Beijing, China, 4Rheumatology, Allergy, and Immunology, Stony Brook University School of Medicine, Stony Brook, NY

      Background/Purpose: Systemic autoinflammatory diseases (SAIDs) are a genetically heterogeneous group of rheumatic diseases that are driven by abnormal activation of the innate immune system.…
  • Abstract Number: 255 • 2016 ACR/ARHP Annual Meeting

    Safety and Efficacy of Long-Term Canakinumab Therapy in Patients with CAPS: Final Results from Beta-Confident Registry

    Hal M. Hoffman1, Jasmin B. Kuemmerle-Deschner2, Philip N. Hawkins3, Tom van der Poll4, Ulrich A. Walker5, Antonio Speziale6, Yolandi Joubert6 and Hugh H. Tilson7, 1Division of Rheumatology, Allergy, and Immunology, University of California at San Deigo, La Jolla, CA, 2Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 3University College London Medical School, London, United Kingdom, 4Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands, 5Department of Rheumatology, University Hospital Basel, Basel, Switzerland, 6Novartis Pharma AG, Basel, Switzerland, 7University of North Carolina, Gillings School of Global Public Health, Chapel Hill, NC

    Background/Purpose:  Cryopyrin-associated periodic syndrome (CAPS) is a rare auto-inflammatory disease encompassing a spectrum of 3 phenotypes: familial cold auto-inflammatory syndrome (FCAS), Muckle–Wells syndrome (MWS), and…
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