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  • Abstract Number: 1243 • 2019 ACR/ARP Annual Meeting

    Anakinra Treatment in Recurrent Pericarditis: Single Center Experience

    Zeynep Toker Dincer1, Osman Corbali 2, Serdal Ugurlu 1 and Huri Ozdogan 1, 1Istanbul University - Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, ISTANBUL, Turkey, 2Istanbul University - Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, ISTANBUL

    Background/Purpose: Recurrent pericarditis (RP), however the etiology is unknown in the majority, may be observed in autoinflammatory diseases such as familial Mediterranean fever (FMF) and…
  • Abstract Number: 1244 • 2019 ACR/ARP Annual Meeting

    Tocilizumab – An Effective Rescue Therapy for Refractory Unclassified Autoinflammatory Diseases in Children

    Jasmin B. Kuemmerle-Deschner1, Daniel Sturm 1 and Susanne Benseler 2, 1University Hospital Tuebingen, Tuebingen, Germany, 2Alberta Children’s Hospital Research Institute, Calgary, Canada

    Background/Purpose: Evidence based treatment options for children and adults with unclassified autoinflammatory diseases (AID) are limited. Frequently, IL-1-inhibition is primarily tried to control the severe…
  • Abstract Number: 1245 • 2019 ACR/ARP Annual Meeting

    Efficacy of Canakinumab Treatment in Adult-onset Still’s Disease

    Corrado Campochiaro1, Alessandro Tomelleri 2, De Luca Giacomo 3, Nicola Farina 2, Elena Baldissera 2, Giulio Cavalli 2 and Lorenzo Dagna 2, 1Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy, 2Unit of Immunology, Rheumatology, Allergy and Rare Diseases. IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University., Milan, Italy, 3Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy., Milan, Italy

    Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare auto-inflammatory condition characterized by fever, arthritis, skin rash, and multi-organ inflammation. The pathogenesis of AOSD is centrally…
  • Abstract Number: 1246 • 2019 ACR/ARP Annual Meeting

    Using PROMIS Data to Assess Activity of Inflammatory Eye Disease

    Joshua Hedrick1, Rula Hajj-ali 2, Yuxuan Jin 3 and Sunil Srivastava 1, 1Cleveland Clinic, Cleveland, OH, 2Rheumatologic and Immunologic Disease/Cleveland Clinic, Cleveland, OH, 3Quantitative Health Science, Cleveland Clinic, Cleveland, OH

    Background/Purpose: Diagnosing and managing patients with inflammatory eye disease (IED) requires close interdisciplinary care with rheumatologists adjusting systemic, potentially toxic, therapies based on exam findings…
  • Abstract Number: 1247 • 2019 ACR/ARP Annual Meeting

    Adult-Onset Still’s Disease and Spondyloarthritis: Overlapping Syndrome or Incidental Association? A Series of 5 Cases

    Aly Kamissoko 1, Nolan Hassold 2, Alexis Mathian 3, Edouard Pertuiset 4, Gaetane Nocturne 2, Bruno Fautrel 5 and Stéphane Mitrovic6, 1AP-HP, Department of Rheumatology, Pitié-Salpêtrière University Hospital, Paris, France, Paris, France, 2Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France., Paris, France, 3AP-HP, Service de Médecine Interne 2, Hôpital Universitaire Pitié-Salpêtrière, Paris, France, Paris, France, 4Rheumatology Department, Pontoise Hospital, Pontoise, France, Pontoise, France, 5Pitié-Salpêtrière Hospital, Department of Rheumatology, AP-HP, Sorbonne University, UPMC university, Paris, Ile-de-France, France, 6AP-HP, Department of Rheumatology, Pitié-Salpêtrière University Hospital, Sorbonne Université, GRC 08, Pierre Louis Institute of Epidemiology and Public Health, Paris, France - Centre d’Etude et de Référence sur les Maladies AutoInflammatoires et les Amyloses (CEREMAIA), FAI2R network, Paris, France - Institut Mutualiste Montsouris, Paris, France, Paris, France

    Background/Purpose: Some forms of spondyloarthritis (SpA) such as SAPHO syndrome are classified as auto-inflammatory disorders. Association with adult onset Still’s disease (AoSD) has never been…
  • Abstract Number: 1248 • 2019 ACR/ARP Annual Meeting

    Clinical Manifestations and Management of US Patients with SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) Syndrome, a Retrospective Study

    Delamo Bekele1, Paras Karmacharya 2, Kerry Wright 2 and Clement Michet 1, 1Mayo Clinic Rochester, Rochester, MN, 2Mayo Clinic, Rochester, MN

    Background/Purpose: The SAPHO syndrome is a rare inflammatory disorder of bones, joints and skin first coined by Chamot in 1987 characterized by synovitis, acne, pustulosis,…
  • Abstract Number: 1249 • 2019 ACR/ARP Annual Meeting

    Pelvic Congestion Syndrome, an Uncommon Cause of Osteoarticular Pain

    Mireia Castillo-Vilella1, Jose Luis Tandaipan 2, Laura Berbel 3, Lluís Moga 3, Georgina Salvador 3, Silvia Martínez-Pardo 3 and Nuria Giménez 3, 1Hospital Universitari Sagrat Cor, Barcelona, Spain, 2Hospital Universitari Mutua Terrassa, Terrassa, Spain, 3Hospital Universitari Mútua Terrassa, Terrassa

    Background/Purpose: Background: The pelvic congestion syndrome (PCS) is an under and often misdiagnosed entity that appears more frequently in premenopausal age and multiparous women. The…
  • Abstract Number: 1250 • 2019 ACR/ARP Annual Meeting

    Can Patient-Reported Outcomes and Disease Activity Scores Predict Patient Acceptable Symptom State in Adult-Onset Still’s Disease?

    Emre Bilgin 1, Timuçin Kaşifoğlu 2, Ahmet Omma 3, Cemal Bes 4, Muhammet Çınar 5, Hakan Emmungil 6, Orhan Küçükşahin 7, Servet Akar 8, Kenan Aksu 9, Fatih Yıldız 10, Nilüfer Kanıtez 11, Abdulsamet Erden 12, Sezin Turan 13, Ediz Dalkılıç 14, Selime Ermurat 15, Mutlu Hayran 16 and Umut Kalyoncu17, 1Hacettepe University Vasculitis Centre, Ankara, Turkey, Ankara, Turkey, 2Eskişehir Osmangazi University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Eskişehir, Turkey, 3Ankara Numune Education and Research Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey, 4University of Medical Sciences, İstanbul, Turkey, 5University of Medical Sciences, Ankara, Turkey, 6Trakya University, Edirne, Turkey, 7Liv Hospital, Ankara, Turkey, 8Izmir Katip Celebi University, Faculty of Medicine, Division of Rheumatology, İzmir, Turkey, 9Ege University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İzmir, Turkey, 10Kahramanmaraş Sütçü İmam University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Kahramanmaraş, Turkey, 11Koç University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, İstanbul, Turkey, 12Ministry of Health Ankara City Hospital, Department of Internal Medicine, Division of Rheumatology, Ankara, Turkey, 13Trakya University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Edirne, Turkey, 14Uludağ University, Bursa, Turkey, 15Uludağ University, Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Bursa, Turkey, 16Hacettepe University, Faculty of Medicine, Department of Preventive Oncology, Ankara, Turkey, 17Hacettepe University Department of Rheumatology, Ankara, Turkey

    Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare, chronic and auto-inflammatory disorder with multisystemic involvement. Patient Acceptable Symptom State (PASS) is the highest level of…
  • Abstract Number: 1251 • 2019 ACR/ARP Annual Meeting

    The Impact of Aging on Familial Mediterranean Fever Patients

    Serdal Ugurlu 1, Okan Aydin 2, Bugra Han Egeli3, Asli Ece Soykut 2, Deniz Demir 2 and Huri Ozdogan 2, 1Istanbul University - Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, istanbul, Turkey, 2Istanul University-Cerrahpasa, istanbul, Turkey, 3İstanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey

    Background/Purpose: Familial Mediterranean Fever (FMF) is a monogenic autoinflammatory disorder with innate immune activation with an onset before age 20 in approximately 90% of the…
  • Abstract Number: 1252 • 2019 ACR/ARP Annual Meeting

    Pattern of Arthropathy in Patients with Cystic Fibrosis

    Daniel Pham 1, Mehrdad Maz 2, Michael Crosser 1 and Megan Krause1, 1University of Kansas, Kansas City, KS, 2The University of Kansas Medical Center, KANSAS CITY, KS

    Background/Purpose: Arthropathy is a rare but debilitating manifestation of cystic fibrosis (CF) that has no formal definition. This study attempts to characterize the spectrum of…
  • Abstract Number: 1253 • 2019 ACR/ARP Annual Meeting

    Novel Nonsense Variant and Entire Deletion of TNFAIP3 Cause Haploinsufficiency of A20 Clinically Distinct from Behçet’s Disease

    Naomi Tsuchida1, Yohei Kirino 1, Yutaro Soejima 1, Hideaki Nakajima 1, Satoko Miyatake 2 and Naomichi Matsumoto 2, 1Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan, 2Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan

    Background/Purpose: Haploinsufficiency of A20 (HA20) is caused by loss-of-function TNFAIP3 variants. Phenotypic and genetic features of HA20 remain uncertain; therefore, clinical distinction between HA20 and…
  • Abstract Number: 1254 • 2019 ACR/ARP Annual Meeting

    A Retrospective Medical Chart Review of Patients with Periodic Fever Syndromes Initiating Canakinumab in the United States

    Peter Hur1, Kathleen G. Lomax 1, Raluca Ionescu-Ittu 2, Ameur M. Manceur 2, Jipan Xie 3, Jordan Cammarota 4, Navneet Sanghera 5 and Alexei A. Grom 6, 1Novartis Pharmaceuticals Corporation, East Hanover, NJ, 2Analysis Group, Inc., Montreal, QC, Canada, 3Analysis Group, Inc., Los Angeles, CA, 4Analysis Group Inc.,, Washington, DC, 5Novartis Pharmaceuticals Corporations, East Hanover, NJ, 6Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Periodic fever syndromes (PFS) is a group of rare autoinflammatory diseases that includes cryopyrin-associated periodic syndromes (CAPS), hyperimmunoglobulin D syndrome/mevalonate kinase deficiency (HIDS/MKD), TNF…
  • Abstract Number: 1255 • 2019 ACR/ARP Annual Meeting

    Is Exon 2 Associated with FMF or a New Disease?

    Bilal Kulaksiz 1, Bugra Han Egeli 2, Okan Aydin 3 and Serdal Ugurlu4, 1Istanbul University- Cerrahpasa, istanbul, Turkey, 2İstanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 3Istanul University-Cerrahpasa, istanbul, Turkey, 4Istanbul University - Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, istanbul, Turkey

    Background/Purpose: In Familial Mediterranean Fever (FMF), patients having exon 10 mutations, specifically M694V, M680I, and V726A are more common and their disease profiles tend to…
  • Abstract Number: 1256 • 2019 ACR/ARP Annual Meeting

    Comparison of FMF Patients with Age of Onset Before 20 versus 40 Years and Over

    Serdal Ugurlu1, Okan Aydin 2 and Huri Ozdogan 2, 1Istanbul University - Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, istanbul, Turkey, 2Istanul University-Cerrahpasa, istanbul, Turkey

    Background/Purpose: Familial Mediterranean fever (FMF) is a disease with an onset before 20 years of age in 90% of the patients.  However late onset FMF defined…
  • Abstract Number: 1257 • 2019 ACR/ARP Annual Meeting

    Recommendation on Colchicine Dosing and Definition of Colchicine Resistance/Intolerance in the Management of Familial Mediterranean Fever

    Seza Ozen1, Erdal Sag 2, Eldad Ben-Chetrit 3, Marco Gattorno 4, Ahmet Gul 5, Philip Hashkes 6, Isabelle Kone-Paut 7, Helen Lachmann 8, Elena Tsitsami 9, Marinka Twilt 10, Fabrizio De Benedetti 11 and Jasmin B. Kuemmerle-Deschner 12, 1Hacettepe University Hospital, Ankara, Turkey, 2Hacettepe University, Ankara, Turkey, 3Hebrew University, Jerusalem, Israel, 4IRCCS Istituto Giannina Gaslini, Genova, Italy, 5Istanbul University, Istanbul, Turkey, 6Shaare-Zedek Medical Center, Jerusalem, Israel, 7Rhumatologie pédiatrique et CEREMAIA,, Université Paris-Sud Saclay, Paris, France, 8The Royal Free Hospital & University College London, London, United Kingdom, 9National and Kapodistrian University of Athens, Athens, Greece, 10University of Calgary, Calgary, AB, Canada, 11Bambino Gesù Children’s Hospital, Rome, Italy, 12University Hospital Tuebingen, Tuebingen, Germany

    Background/Purpose: Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease and colchicine is the drug of choice for its treatment. However, about 5-10%…
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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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