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  • Abstract Number: 367 • 2017 ACR/ARHP Annual Meeting

    Interleukin-1  Receptor Antagonist Is a Potential Treatment for Undifferentiated Autoinflammatory Syndromes

    Ananta Subedi1, Daniella Schwartz2, Karyl Barron3, Daniel L. Kastner4 and Amanda Ombrello5, 1National Institute of Arthritis, Musculoskeletal and Skin Disease (NIAMS), Bethesda, MD, 2NIAMS - Rheumatology, National Institutes of Health, Bethesda, MD, 3National Institutes of Health, Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, MD, 4Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 5Inflammatory Disease Section, NHGRI/NIH, Bethesda, MD

    Background/Purpose: The autoinflammatory diseases (AIDs) are a group of disorders of the innate immune system characterized by seemingly unprovoked inflammation1. A variety of genetic alterations…
  • Abstract Number: 368 • 2017 ACR/ARHP Annual Meeting

    Evaluation of Efficacy and Safety of Opocalcium Colchicine, and Anti-IL1 Treatment in Childhood Colchicine-Resistant Familial Mediterranean Fever

    Kenan Barut, Amra Adrovic, Sezgin Sahin, Asli Kaplan and Ozgur Kasapcopur, Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey

    Background/Purpose: The colchicine-resistant FMF (crFMF) is defined as 6 or more polyserositis attacks in the last year despite the regular usage of colchicine in the…
  • Abstract Number: 369 • 2017 ACR/ARHP Annual Meeting

    Hepatitis A Virus Vaccination in Juvenile-Onset Systemic Lupus Erythematosus

    Sevinc Mertoglu1, Sezgin Sahin1, Omer Faruk Beser2, Amra Adrovic1, Kenan Barut1, Pelin Yuksel3, Soner Sazak4, Bekir Kocazeybek5 and Ozgur Kasapcopur1, 1Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, 2Pediatric Gastroenterology, Department of Pediatrics, Okmeydani Education and Training Hospital, Istanbul, Turkey, 3Microbiology, Istanbul University, Cerrahpasa Medical School, Department of Microbiology, Istanbul, Turkey, 4Pediatrics, Department of Pediatrics, Okmeydani Education and Training Hospital, Istanbul, Turkey, 5Istanbul University, Cerrahpasa Medical School, Department of Microbiology, Istanbul, Turkey

    Background/Purpose: Various infections play significant roles in flares of systemic lupus erythematosus (SLE). Hepatitis A virus is one of these infectious agents that has high…
  • Abstract Number: 370 • 2017 ACR/ARHP Annual Meeting

    Validation of 2016 ACR/EULAR Classification Criteria of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis in Japanese Patients

    Masaki Shimizu1, Mao Mizuta1, Takahiro Yasumi2, Naomi Iwata3, Yuka Okura4, Noriko Kinjo5, Hiroaki Umebayashi6, Tomohiro Kubota7, Yasuo Nakagishi8, Kenichi Nishimura9, Masato Yashiro10, Junko Yasumura11, Kazuko Yamazaki12, Hiroyuki Wakiguchi13, Nami Okamoto14 and Masaaki Mori15, 1Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan, 2Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan, 3Department of Immunology and Infectious Diseases, Aichi Children’s Health and Medical Center, Obu, Japan, 4Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan, 5Department of Pediatrics, Faculty of medicine, University of the Ryukyus, Nakagami-gun, Japan, 6Department of Rheumatics, Miyagi Children’s Hospital, Sendai, Japan, 7Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshima, Japan, 8Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan, 9Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan, 10Department of Pediatrics, Okayama University Hospital, Okayama, Japan, 11Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan, 12Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan, 13Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan, 14Department of Pediatrics, Osaka Medical College, Takatsuki, Japan, 15Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan

    Background/Purpose: To validate the 2016 ACR/EULAR classification criteria of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) in Japanese patients. Methods: A combination…
  • Abstract Number: 371 • 2017 ACR/ARHP Annual Meeting

    Longer Term Outcomes of Chronic Relapsing Multifocal Osteomyelitis in a UK Tertiary Adolescent and Young Adult Rheumatology Centre

    Kristina E.N. Clark1, Francesca Josephs2, Nicola Daly3, Claire Louise Murphy3 and Debajit Sen4, 1Rheumatology, University college London Hospitals, London, United Kingdom, 2rhuematology, UCL, London, United Kingdom, 3Rheumatology, University College London Hospital, London, United Kingdom, 4Adolescent Rheumatology Department, University College London Hospital NHS Trust, London, United Kingdom

    Background/Purpose: Chronic relapsing multifocal osteomyelitis (CRMO) is a rare autoinflammatory bone condition presenting primarily in children & adolescents. It characteristically affects the epiphysis & metaphysis…
  • Abstract Number: 372 • 2017 ACR/ARHP Annual Meeting

    Phenotype of Chronic Recurrent Multifocal Osteomyelitis in a Tertiary Referral Centre: The Great Ormond Street Hospital Experience

    Kulsoom Riaz1 and Sandrine Lacassagne2, 1Paediatric Rheumatology/Gastroenterology, Great Ormond Street Hospital, London, United Kingdom, 2Paediatric Rheumatology, Great Ormond Street Hospital, London, United Kingdom

    Background/Purpose: Recurrent Multifocal Osteomyelitis (CRMO) is an aseptic inflammatory bone disease that typically affects the metaphases of the long bones. It affects children, adolescents and…
  • Abstract Number: 373 • 2017 ACR/ARHP Annual Meeting

    a Retrospective Study of Clinical Factors Influencing the Development of Overlapping Disease Features in Pediatric Patients with Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Spondyloarthropathies (SpA)

    Lillian Lim1, Jyoti Panwar2, Jennifer Stimec3, Shirley M.L. Tse4, Brian M. Feldman5 and Ronald M. Laxer6, 1Paediatrics, The Hospital for Sick Children, Toronto, ON, Canada, 2Christian Medical College, Vellore, India, Vellore, India, 3The Hospital for Sick Children, Toronto, ON, Canada, 4Rheumatology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, 5Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 6Div of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada

    Background/Purpose: Some studies have suggested that chronic recurrent multifocal osteomyelitis (CRMO) and spondyloarthropathies (SpA) fall on a spectrum of disease, as they have been noted…
  • Abstract Number: 374 • 2017 ACR/ARHP Annual Meeting

    A Pilot Study of Infrared Thermal Imaging to Detect Active Bone Lesions in Children with Chronic Nonbacterial Osteomyelitis

    Yongdong Zhao1, Ramesh Iyer2, Lucas Reichley1, Assaf Oron3, Averi Kitsch4, Seth Friedman5, Savannah Partridge4 and Carol A Wallace1, 1University of Washington, Department of Pediatrics, Seattle, WA, 2Division of Radiology, University of Washington, Seattle Children’s Hospital, Seattle, WA, 3Center for Clinical and Translational Research, Seattle Children’s Research Institute, Seattle, WA, 4Department of Radiology, University of Washington, Seattle, WA, 5Division of Radiology, University of Washington, Seattle Children's Hospital, Seattle, WA

    Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disease. For detection of active bone lesions, bone scintigraphy and magnetic resonance imaging (MRI) are much…
  • Abstract Number: 375 • 2017 ACR/ARHP Annual Meeting

    Controlled Discontinuation of  Colchicine Therapy in  Familial  Mediterranean Fever Patients with Single MEFV Mutation

    Yonatan Butbul Aviel1, Shafe Fahoum2 and Riva Brik3, 1Department of Pediatrics B Pediatric Rheumatology Service, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel, Haifa, Israel, 2Department of Pediatrics B,, , Ruth Children's Hospital, Rambam Medical Center, Haifa, Israel, Pediatric Rheumatology Service, .Rappaport Faculty of Medicine, Technion-lsrael Institute of Technology, Haifa, Israel, 3Pediatrics, Rambam Medical Center, Haifa, Israel

    Background/Purpose: Familial Mediterranean fever (FMF) traditionally has been considered an autosomal recessive disease; however, the diagnosis remains predominantly clinical, since mutations cannot always be identified…
  • Abstract Number: 376 • 2017 ACR/ARHP Annual Meeting

    Improvement of Disease Activity in Patients with Colchicine-Resistant FMF, Hids/Mkd and TRAPS Assessed By Autoinflammatory Disease Activity Index (AIDAI): Results from a Randomized Phase III Trial

    Isabelle Koné-Paut1, Michaël Hofer2, Susanne Benseler3, Jasmin B. Kuemmerle-Deschner4, Annette Jansson5, Itzhak Rosner6, Raffaele Manna7, Sara Murias8, Omer Karadag9, Lori Tucker10, Ilonka Orban11, Vincent Tormey12, Maria Alessio13, Huri Ozdogan14 and Fabrizio De Benedetti15, 1Bicêtre Hospital, APHP, Univeristy Paris Sud, Paris, France, 2Unité romande d’immuno-rhumatologie pédiatrique, CHUV, University of Lausanne, Genova, Italy, 3Alberta Children's Hospital, Calgary, AB, Canada, 4University Hospital Tuebingen, Tuebingen, Germany, 5Ludwig Maximilian University, Munich, Germany, 6Bnai-Zion Medical Center, Haifa, Israel, 7Department of Internal Medicine, Università Cattolica Sacro Cuore, Rome, Italy, 8Hospital Infantil La Paz, Madrid, Spain, 9Hacettepe University Faculty of Medicine, Ankara, Turkey, 10BC Children's Hospital, Vancouver, BC, Canada, 11Orszagos Reumatologial es Fizioterepias, Budapest, Hungary, 12University College Hospital Galway, Galway, Ireland, 13A Osped-Universit Policlinico Federico II, Univ degli Studi di Napoli, Napoli, Italy, 14Rheumatology, Istanbul University, Cerrahpasa Medical Faculty, Division of Rheumatology, Istanbul, Turkey, 15Istituto Giannina Gaslini - Pediatria II, Reumatologia - PRINTO, Genoa, Italy

    Background/Purpose: AIDAI is a novel, validated tool for the assessment of disease activity across a wide spectrum of autoinflammatory diseases including recurrent fever syndromes such…
  • Abstract Number: 377 • 2017 ACR/ARHP Annual Meeting

    Novel Insights into Periodic Fever Syndromes

    Tiffany Hoang1, Shreya Shrestha1 and Daniel Albert2, 1Dartmouth Medical School, Lebanon, NH, 2Medicine/Rheumatology, Dartmouth-Hitchcock Med Ctr, Lebanon, NH

    Background/Purpose: The Periodic Fever Syndromes (PFS) are a rapidly expanding group of disorders primarily of the innate immune system that often affect the inflammasome. In…
  • Abstract Number: 378 • 2017 ACR/ARHP Annual Meeting

    Musculoskeletal Features in Copa Syndrome

    William B. Lapin1, Monica Marcus2, Andrea A. Ramirez3, Marietta M. de Guzman3 and Levi B. Watkin4,5, 1Department of Pediatrics, Division of Immunology, Allergy and Rheumatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, 2Pediatric Immunology, Allergy and Rheumatology, Texas Children's Hospital, Houston, TX, 3Immunology, Allergy and Rheumatology, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, 4Department of Pediatrics, Division of Immunology, Allergy and Rheumatology, Baylor College of Medicine, Houston, TX, 5Texas Children's Hospital, Center for Human Immunobiology, Houston, TX

    Background/Purpose: COPA syndrome is a newly discovered primary immunodeficiency resulting in immune dysregulation showing autosomal dominant inheritance with incomplete penetrance. Its name is derived from…
  • Abstract Number: 379 • 2017 ACR/ARHP Annual Meeting

    H Syndrome: Five New Cases from the United States with Novel Features and Responses to Therapy

    Jessica Bloom1, Clara Lin2, Lisa F. Imundo3, Stephen Guthery4, Shelly Stepenaskie5, Csaba Galambos6, Amy Lowichik7 and John F. Bohnsack8, 1Pediatrics, Children's Hospital Colorado, Aurora, CO, 2Pediatric Rheumatology, Children's Hospital Colorado, Aurora, CO, 3Pediatrics, Columbia University Medical Center, New York, NY, 4Department of Pediatrics,, University of Utah, Salt Lake City, UT, 5Pathology and Dermatology, University of New Mexico, Albuquerque, NM, 6Pathology, Children's Hospital Colorado, Aurora, CO, 7Pathology, University of Utah, Salt Lake City, UT, 8Division of Allergy, Immunology and Pediatric Rheumatology, University of Utah, Salt Lake City, UT

    Background/Purpose: H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations…
  • Abstract Number: 380 • 2017 ACR/ARHP Annual Meeting

    Treatment Outcomes of Down Syndrome Arthropathy

    Jordan T. Jones1, Leena Danawala2, Nasreen Talib3 and Mara L Becker4, 1Rheumatology Division, Children's Mercy Kansas City, Kansas City, MO, 2University of Missouri-Kansas City School of Medicine, Kansas City, MO, 3General Pediatrics, Children's Mercy Kansas City, Kansas City, MO, 4Rheumatology, Children's Mercy Kansas City, Kansas City, MO

    Background/Purpose: Crude prevalence estimates indicate Down syndrome arthropathy (DA) is 3-8 times more common than juvenile idiopathic arthritis (JIA), however, DA is still largely under…
  • Abstract Number: 381 • 2017 ACR/ARHP Annual Meeting

    Down’s Arthritis (DA) – Clinical and Radiological Features of Arthritis in Children with Trisomy 21

    Charlene Foley, Emma Jane Mac Dermott and Orla Killeen, National Centre for Paediatric Rheumatology, Our Lady's Children's Hospital Crumlin, Dublin, Ireland

    Background/Purpose: Down's Arthritis (DA) was first reported in the literature in 1984. Crude estimates suggest higher incidence and prevalence rates of DA compared with Juvenile…
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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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