ACR Meeting Abstracts

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  • Abstract Number: 2272 • 2018 ACR/ARHP Annual Meeting

    Missed Opportunity? Evaluation for Systemic Autoimmune Diseases in Patients with Uveitis May Not be Optimal: 5-Year Analysis of Patients Being Seen in a Tertiary Medical Center

    Nancy Harrison1, Swetha Boddeda2, Shweta Kishore2 and Vikas Majithia3, 1Rheumatolody, University of Mississippi Medical Center, Jackson, MS, 2Rheumatology, University of Mississippi Medical Center, Jackson, MS, 3Division of Rheumatology, University of Mississippi Medical Center, Jackson, MS

    Background/Purpose: Uveitis is the most common inflammatory eye disease and is frequently associated with secondary systemic autoimmune diseases (SSAID) excluding infection in 40-55% of cases.…
  • Abstract Number: 2273 • 2018 ACR/ARHP Annual Meeting

    High Dose Intravenous Methylorednisolone Induces Rapid Improvement in Severe Non-Infectious Uveitis. a Multicenter Study of 120 Patients

    Nuria Vegas-Revenga1, Vanesa Calvo-Río1, Íñigo González-Mazón2, Lara Sánchez-Bilbao2, Emma Beltrán3, Alejandro Fonollosa4, Olga Maiz-Alonso5, Ana Blanco6, Miguel Cordero-Coma7, Norberto Ortego Centeno8, Ignacio Torre-Salaberri9, Félix Francisco10, Santiago Muñoz-Fernández11, Maria del Mar Esteban Ortega12, Manuel Díaz-Llopis13, Mario Agudo2, Joaquin Cañal1, Juan Antonio Ventosa1, Rosalía Demetrio-Pablo1, Lucia C. Domínguez-Casas1, Miguel Angel González-Gay14 and Ricardo Blanco2, 1Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain, 2Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain, 3Rheumatology, Hospital General Universitario de Valencia. Spain, Valencia, Spain, 4Ophthalmology, Hospital de Cruces. Bilbao. Spain, Bilbao, Spain, 5Hospital Universitario Donostia. San Sebastian. Spain, Donostia, Spain, 6Ophtamology, Hospital Universitario Donostia. San Sebastian. Spain, San Sebastian, Spain, 7Ophthalmology, Hospital de León. Spain, León, Spain, 8Medicine Department, Hospital Universitario San Cecilio. Granada. Spain, Granada, Spain, 9Rheumatology, Hospital Universitario de Basurto. Bilbao. Spain, Bilbao, Spain, 10Rheumatology, Hospital Doctor Negrín. Las Palmas de Gran Canaria. Spain, Las Palmas de Gran Canaria, Spain, 11Hospital Universitario Infanta Sofia. Madrid. Spain, Mardid, Spain, 12Hospital Universitario Infanta Sofia. Madrid. Spain, Madrid, Spain, 13Hospital Universitario La Fe. Valencia. Spain, Valencia, Spain, 14Rheumatology. Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain

    Background/Purpose: In uveitis rapid and effective remission-inducing therapy is mandatory to avoid irreversible structural and functional damage.Our aim was to evaluate the efficacy and safety…
  • Abstract Number: 2274 • 2018 ACR/ARHP Annual Meeting

    Refractory and Severe Uveitic Cystoid Macular Edema Improves with Tocilizumab in Different Immune Mediated Diseases

    Nuria Vegas-Revenga1, Vanesa Calvo-Río1, Marina Mesquida2, Alfredo Adan2, M. Victoria Hernández2, Emma Beltrán3, Elia Valls Pascual4, David Diaz-Valle5, Gisela Díaz-Cordovés6, Marisa Hernandez Grafella7, Lucía Martínez-Costa8, Inmaculada Calvo9, Antonio Atanes10, Luis Francisco Linares11, Consuelo Modesto12, Elena Aurrecoechea13, Miguel Cordero-Coma14, Rosalía Demetrio-Pablo1, Lucia C. Domínguez-Casas15, José Luis Hernández1, Miguel Angel González-Gay1 and Ricardo Blanco15, 1Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain, 2Hospital Clinic. Barcelona. Spain, Barcelona, Spain, 3Rheumatology, Hospital del Mar. Barcelona. Spain, Barcelona, Spain, 4Hospital Universitario de Valencia. Spain, Valencia, Spain, 5Hospital Clínico San Carlos. Madrid. Spain, Madrid, Spain, 6Rheumatology, Hospital Regional Universitario de Málaga. Spain, Málaga, Spain, 7Hospital Doctor Peset. Valencia. Spain, Valencia, Spain, 8Hospital Doctor Peset. Valencia. Spain, Valencia., Spain, 9Hospital Universitario La Fe, Valencia, Spain, 10Complejo Hospitalario Universitario A Coruña. Spain, A Coruna, Spain, 11Rheumatology, Hospital Virgen de la Arrixaca. Murcia. Spain, Murcia, Spain, 12Hospital Universitari Vall d'Hebron. Barcelona. Spain, Barcenola, Spain, 13Rheumatology, Hospital de Sierrallana. Torrelavega. Spain, Torrelavega, Spain, 14Ophthalmology, Hospital de León. Spain, León, Spain, 15Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Universidad de Cantabria. Spain, Santander, Spain

     Background/Purpose: Cystoid macular edema (CME) represents the leading cause of blindness in uveitis of different immune mediated diseases (IMD). Our aim was to evaluate the…
  • Abstract Number: 2275 • 2018 ACR/ARHP Annual Meeting

    Autoimmune Encephalitis with Concomitant Systemic Rheumatologic Auto-Antibodies

    Nicole Droz1, Alexander Rae-Grant2 and Rula A Hajj-Ali3, 1Rheumatology, Cleveland Clinic Foundation, Cleveland, OH, 2Neurology, Cleveland Clinic Foundation, Cleveland, OH, 3Rheumatic and Immunologic Disease, Cleveland Clinic Foundation, Cleveland, OH

    Background/Purpose: Autoimmune encephalitis (AE) is a rapidly progressive encephalopathy presenting with neurologic and psychiatric manifestations and is often associated with antibodies targeting neuronal cell-surface or…
  • Abstract Number: 2276 • 2018 ACR/ARHP Annual Meeting

    Risk of Hemorrhagic Strokes in Patients with Adenosine Deaminase 2 Deficiency

    Patrycja M. Hoffmann1, Amanda Ombrello1, Deborah L. Stone2, Dean Follmann3, Karyl Barron4, Anne Jones5, Tina Romeo1, Camilo Toro6, Ariane Soldatos7, Arielle Hay8, Qing Zhou9, Ivona Aksentijevich10 and Daniel L. Kastner5, 1NHGRI, National Institutes of Health, Bethesda, MD, 2Inflammatory Disease Section, NHGRI/NIH, Bethesda, MD, 3NIAID, Bethesda, MD, 4National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, 5Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 6NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, 7NINDS, National Institutes of Health, Bethesda, MD, 8Nicklaus Children’s Hospital, Miami, FL, 9China, 10Metabolic, Cardiovascular, and Inflammatory Disease Genomics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD

    Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive condition characterized by recurrent fevers, early-onset ischemic strokes, livedo racemosa, polyarteritis nodosa, portal hypertension,…
  • Abstract Number: 2277 • 2018 ACR/ARHP Annual Meeting

    Colchicine: An Effective Treatment Option for Unclassified Autoinflammatory Diseases in Children

    Jasmin B. Kuemmerle-Deschner1, Anna Lena Schock2, Sandra Hansmann3 and Susanne Benseler4, 1Department of Pediatrics, Division of Rheumatology, University Hospital Tuebingen, Germany, Tuebingen, Germany, 2Department of Pediatrics, Division of Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, Tuebingen, Germany, 3Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Tuebingen, Germany, Tuebingen, Germany, 4Rheumatology, Department of Paediatrics, Alberta Children's Hospital, University of Calgary, Alberta, Canada, Calgary, AB, Canada

    Background/Purpose: Interleukin-1 (IL-1) inhibition was found to be an effective, yet very expensive treatment option for children with clinically and genetically defined autoinflammatory diseases (AID).…
  • Abstract Number: 2278 • 2018 ACR/ARHP Annual Meeting

    Cryopyrinopathy across Generations: Longterm Disease Outcome

    Sarka Fingerhutova1, Jana Franova2, Eva Hlavackova3, Eva Jancova4, Leona Prochazkova5, Marketa Tesarova6 and Pavla Dolezalová7, 1Paediatric Rheumatology Unit, General University Hospital in Prague and 1st Faculty of Medicine, Charles University, General University Hospital in Prague and 1st Faculty of Medicine, Prague, Czech Republic, 2Department of Paediatric Rheumatology, University Hospital Brno, Brno, Czech Republic, Brno, Czech Republic, 3Department of Allergology and Immunology, St Ann´s Hospital Brno, University Hospital, Brno, Czech Republic, Brno, Czech Republic, 4Department of Nephrology, General University Hospital in Prague and 1st Faculty of Medicine, Prague, Czech Republic, 5Department of Rheumatology, St Ann´s Hospital Brno, University Hospital, Brno, Czech Republic, Brno, Czech Republic, 6Laboratory for study of Mitochondrial Disorders, Department of Paediatrics and Adolescent Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, 7Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic

    Background/Purpose: Cryopyrinopathies are autoinflammatory disorders (AID) caused by mutations of NLRP3 gene that lead to interleukin-1 (IL-1) overproduction with the clinical picture of periodic fever.…
  • Abstract Number: 2279 • 2018 ACR/ARHP Annual Meeting

    Therapeutic Value of Canakinumab in Patients with Yao Syndrome

    Qingping Yao, Rheumatology, Allergy and Immunology, Stony Brook University Hospital, Stony Brook, NY; Rheumatology, Allergy, and Immunology, Stony Brook University School of Medicine, Stony Brook, NY

    Background/Purpose: Yao syndrome (YAOS, OMIM 617321), formerly termed nucleotide-binding, oligomerization domain 2(NOD2)-associated autoinflammatory disease, is characterized by periodic fever, dermatitis, arthritis, and swelling of the…
  • Abstract Number: 2280 • 2018 ACR/ARHP Annual Meeting

    Transcriptomic Analysis of Hidradenitis Suppurativa Skin Demonstrates Dysregulation of Antimicrobial Proteins and Inflammatory Pathways

    Victoria Shanmugam1, Derek Jones2, Matthew Bendall3 and Keith Crandall4, 1Rheumatology, The George Washington University, Washington, DC, 2The George Washington University, Washington, DC, 3Department of Microbiology, Immunology and Tropical Medicine, The George Washington University, Washington, DC, 4Department of Biology, The George Washington University, Washington, DC

    Background/Purpose: Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory disease of the apocrine sweat glands. The purpose of the current study was to identify transcripts…
  • Abstract Number: 2281 • 2018 ACR/ARHP Annual Meeting

    Treatment Choices and Response Rates of SAPHO Syndrome: Single Center Case Series

    Abdulsamet Erden1, Mustafa Ekici2, Alper Sari3, Berkan Armagan3, Levent Kilic3, Sule Apras Bilgen1, Ali Akdogan1, Omer Karadag1, Sedat Kiraz3 and Ihsan Ertenli1, 1Rheumatology, Hacettepe University, Faculty of Medicine, Ankara, Turkey, 2Hacettepe University Faculty of Medicine, Ankara, Turkey, 3Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Background/Purpose: SAPHO Syndrome is a chronic disease with bone, joint and skin involvement characterized by synovitis, acne, pustulosis, hyperostosis, osteitis. Treatment decisions of SAPHO syndrome…
  • Abstract Number: 2282 • 2018 ACR/ARHP Annual Meeting

    Measurement of the Pro-Coagulant Activity of Microparticles in Patients with Inflammatory Rheumatic Diseases: Prospective Study

    Christine Mekhail1, Xavier Guillot2, Clément Prati3, Philippe Saas4, Guillaume Mourey5 and Daniel Wendling6, 1Rheumatology, CHRU, Besançon, France, 2PEPITE EA 4267, Bourgogne-Franche-Comté university, Besançon, France, 3Rheumatology, University Hospital - Bourgogne Franche Comté University, Besancon, France, 4INSERM UMR1098, Besançon university hospital, Besançon, France, 5EFS, Besançon, France, 6Rheumatology, University Hospital - Bourgogne Franche Comté University, Besançon, France

    Background/Purpose: Microparticles (MPs) are small membrane-bound vesicles that arise from activated and dying cells. Although the majority of MPs in the blood originate from platelets,…
  • Abstract Number: 2283 • 2018 ACR/ARHP Annual Meeting

    Incidental Steroid Use May Worsen Outcomes in Patients with Heparin Induced Thrombocytopenia in the ICU Setting

    Roy Souaid1 and Connie Lu2, 1Internal medicine, Brown University, Providence, RI, 2Medical school, Brown university, Providence, RI

    Background/Purpose: Heparin induced thrombocytopenia (HIT) is the destruction of platelets in patients exposed to heparin products. While there are two types of HIT, the term…
  • Abstract Number: 2284 • 2018 ACR/ARHP Annual Meeting

    Safety and Efficacy of Lenabasum in Refractory Skin-Predominant Dermatomyositis Subjects Treated on an Open-Label Extension of Trial JBT101-DM-001

    Victoria P. Werth1,2, David Pearson1,2, Joyce Okawa1,2, Rui Feng3, Josef Concha1,2, Basil Patel1,2, Emily Hejazi1,2, Caitlin Cornwall4, Scott Constantine4 and Barbara White4, 1University of Pennsylvania, Philadelphia, PA, 2Philadelphia Veterans Affairs Medical Center, Philadelphia, PA, 3University of Pennsylvania, Philadelphia', PA, 4Corbus Pharmaceuticals, Inc., Norwood, MA

    Background/Purpose: Lenabasum is a synthetic, non-immunosuppressive, selective cannabinoid receptor type 2 agonist that activates resolution of innate immune responses. Lenabasum had acceptable safety and tolerability…
  • Abstract Number: 2285 • 2018 ACR/ARHP Annual Meeting

    Factors Associated with Corticosteroid Discontinuation, Complete Clinical Response and Remission in Patients with Juvenile Dermatomyositis

    Takayuki Kishi1,2, William Warren-Hicks3, Nastaran Bayat1, Ira Targoff4, Terri H Finkel5, Ellen Goldmuntz6, Michael Henrickson7, Bianca Lang8, Andrew Mammen9, Lauren M. Pachman10, Murray Passo11, Terrance P. O'Hanlon1, Frederick W. Miller1, Michael Ward9 and Lisa G. Rider1, 1Environmental Autoimmunity Group, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, 2Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan, 3EcoStat, Inc., Mebane, NC, 4VA Medical Center, University of Oklahoma Health Sciences Center and Oklahoma Medical Research Foundation, Oklahoma City, OK, 5Department of Pediatrics, Nemours Children's Health System/ Nemours Children's Hospital, Orlando, FL, 6NIAID, NIH, Bethesda, MD, 7Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 8Department of Pediatrics, IWK Health Centre and Dalhousie University, Halifax, NS, Canada, 9National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 10Cure JM Program of Excellence in Juvenile Myositis Research, Stanley Manne Children’s Research Institute, affiliated with Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 11Division of Rheumatology PTD, Medical University of South Carolina, Charleston, SC

    Background/Purpose: We examined patients in a large juvenile dermatomyositis (JDM) registry for frequency of and factors associated with final corticosteroid discontinuation (Steroid DC), complete clinical…
  • Abstract Number: 2286 • 2018 ACR/ARHP Annual Meeting

    Myositis and Fasciitis By Magnetic Resonance Imaging in Recent-Onset Polymyalgia Rheumatica and Effect of Tocilizumab Therapy

    Jean Patrick Laporte1, Florent Garrigues2, Anais Huwart2, Sandrine Jousse-Joulin3, Thierry Marhadour1, Dewi Guellec3, Divi Cornec4, Valérie Devauchelle-Pensec3 and Alain Saraux3, 1CHU Brest, Brest, France, 2Radiology, CHU Brest, Brest, France, 3Rheumatology, CHU Brest, Brest, France, 4Rheumatology and UMR1227, Lymphocytes B et Autoimmunité, CHU Brest, Brest, France

    Background/Purpose: To assess the prevalence of myofascial inflammatory lesions visible by magnetic resonance imaging (MRI) and their changes after tocilizumab therapy in active polymyalgia rheumatica…
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