Abstracts Archive - Page 811 of 2425 - ACR Meeting Abstracts

Abstract Number: 789 • 2019 ACR/ARP Annual Meeting
DNASE1L3 Variant in Hypocomplementemic Urticarial Vasculitis Syndrome Identifies a Different Clinical Phenotype
Marco Ranalli ¹, Chiara Passarelli ², Virginia Messia ³, Manuela Pardeo ³, Emanuela Sacco ⁴, Antonella Insalaco ³, Marina Vivarelli ⁵, Fabrizio De Benedetti ⁶ and Claudia Bracaglia⁷, ¹Pediatric Department, La Sapienza University of Rome, Rome, Italy, Rome, Italy, ²Unit of Laboratory of Medical Genetics, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁴Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, ⁵Division of Nephrology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁶Bambino Gesù Children’s Hospital, Rome, Italy, ⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
Background/Purpose: Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare disease characterized by persistent urticarial lesions and hypocomplementemia associated with systemic features involving musculoskeletal, pulmonary, renal…
Abstract Number: 790 • 2019 ACR/ARP Annual Meeting
Can High ANA Titre Combined with Clinical Features Predict Developing Autoimmune Conditions in Children?
Ovgu Kul Cinar¹, Charlene Foley ², Ali Al-Hussain ³, Kimberly Gilmour ⁴, Matthew Buckland ⁴ and Muthana Al-Obaidi ⁵, ¹Great Ormond Street Hospital, Lonodn, United Kingdom, ²Great Ormond Street Hospital, London, Ireland, ³Great Ormond Street Hospital, London, United Kingdom, ⁴Great Ormond Street Hospital NHS Trust, Camelia Botnar Laboratories, London, United Kingdom, ⁵Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom
Background/Purpose: Antinuclear antibodies (ANA) are autoantibodies that recognise cellular antigens found predominantly in the cell nucleus. They are associated with numerous autoimmune diseases such as…
Abstract Number: 791 • 2019 ACR/ARP Annual Meeting
Closing the Seronegative Gap in Pediatric Localized Scleroderma and Systemic Sclerosis
May Choi¹, Emily Mirizio ², Fernanda Quinteros ³, Katherine Buhler ⁴, Marvin Fritzler ¹ and Kathryn Torok ⁵, ¹Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, ²University of Pittsburgh, Pittsburgh, ³University of Calgary, Calgary, ⁴University of Calgary, Calgary, Canada, ⁵UPMC Children's Hospital of Pittsburgh, Pittsburgh
Background/Purpose: It has become increasingly recognized that extra-cutaneous manifestations, such as musculoskeletal and neurologic involvement, are common in pediatric patients with localized scleroderma (LS). We…
Abstract Number: 792 • 2019 ACR/ARP Annual Meeting
Speckle Tracking Echocardiography, a Sensitive Tool to Detect Early Cardiac Dysfunctions in Juvenile Systemic Sclerosis
Giovanni Civieri ¹, Biagio Castaldi ¹, Giorgia Martini ¹, Alessandra Meneghel ¹, Ornella Milanesi ¹ and Francesco Zulian², ¹Dept. of Woman's and Child's Health, university of Padua, Italy, Padua, Italy, ²Department of Woman and Child Health, University of Padua, Italy, Padova, Italy
Background/Purpose: Juvenile Systemic Sclerosis (JSSc) is a rare connective tissue disease in which cardiac involvement is burdened by high morbidity and mortality. The traditional cardiac…
Abstract Number: 793 • 2019 ACR/ARP Annual Meeting
Rituximab for Rapidly Progressive Juvenile Systemic Sclerosis: A Proof-of-concept Study in Four Patients
Roberto Dal Pozzolo ¹, Alessandra Meneghel ¹, Biagio Castaldi ¹, Giorgia Martini ¹, Renzo Marcolongo ² and Francesco Zulian³, ¹Dept. of Woman's and Child's Health, University of Padua, Italy, Padua, Italy, ²Clinical Immunology, Dept. of Medicine, University of Padua, Padua, Italy, ³Department of Woman and Child Health, University of Padua, Italy, Padova, Italy
Background/Purpose: Juvenile Systemic Sclerosis (JSSc) is a rare multi-systemic disease characterized by fibrous changes of the skin and internal organs [1]. Patients with “rapidly progressive”…
Abstract Number: 794 • 2019 ACR/ARP Annual Meeting
Mycophenolate Mofetil for the Treatment of Severe or Methotrexate-refractory Juvenile Localized Scleroderma
Laura Saggioro ¹, Giorgia Martini ¹, Roberta Culpo ¹, Alessandra Meneghel ¹ and Francesco Zulian², ¹Dept. of Woman's and Child's Health, University of Padua, Italy, Padua, Italy, ²Department of Woman and Child Health, University of Padua, Italy, Padova, Italy
Background/Purpose: Juvenile Localized Scleroderma (JLS), includes a number of conditions characterized by skin thickening with varying degree of severity. Many patients, particularly those with the…
Abstract Number: 795 • 2019 ACR/ARP Annual Meeting
Is the Presentation and Severity Different of the Juvenile Diffuse and Limited Subtype Systemic Sclerosis? Results of Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche ², Ozgur Kasapcopur ³, Amra Adrovic ⁴, Kathryn Torok ⁵, Valda Stanevicha ⁶, Maria Teresa Terreri ⁷, Flavio Sztajnbok ⁸, Ekaterina Alexeeva ⁹, Jordi Antón ¹⁰, Brian Feldman ¹¹, Maria Katsicas ¹², Vanessa Smith ¹³, Tadej Avcin ¹⁴, Rolando Cimaz ¹⁵, Mikhail Kostik ¹⁶, Thomas Lehman ¹⁷, Walter Sifuentes-Giraldo ¹⁸, Natalia Vasquez-Canizares ¹⁹, Simone Appenzeller ²⁰, Mahesh Janarthanan ²¹, Monika Moll ²², Dana Nemcova ²³, Maria José Santos ²⁴, Dieneke Schonenberg ²⁵, Christina Battagliotti ²⁶, Lillemor Berntson ²⁷, Blanca Bica ²⁸, Juergen Brunner ²⁹, Patricia Costa Reis ³⁰, Despina Eleftheriou ³¹, Liora Harel ³², Gerd Horneff ³³, Tilmann Kallinich ³⁴, Dragana Lazarevic ³⁵, Kirsten Minden ³⁶, Susan Nielsen ³⁷, Farzana Nuruzzaman ³⁸, Anjali Patwardhan ³⁹, Yosef Uziel ⁴⁰, Daniela Kaiser ⁴¹ and Nicola Helmus ¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, ⁴Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁵UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁶Riga Stradins University, Riga, Latvia, ⁷Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹National Medical Research Center of Children`s Health, Moscow, Russia, ¹⁰Hospital Sant Joan de Déu, Barcelona, Spain, ¹¹University of Toronto & The Hospital for Sick Children, Toronto, ON, Canada, ¹²Hospital de Pediatria, Buenos Aires, Argentina, ¹³Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, ¹⁴Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁵University Hospital Meyer, Florence, Italy, ¹⁶Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁷Hospital for Special Surgery, New York, ¹⁸Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁹Children’s Hospital at Montefiore, Bronx NY, ²⁰UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, ²¹Sri Ramachandra University, Chennai, India, ²²University of Tuebingen, Tuebingen, Germany, ²³General University Hospital, Prague, Czech Republic, ²⁴Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, ²⁵Amsterdam UMC, Amsterdam, Netherlands, ²⁶Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁷Uppsala University Hospital, Uppsala, Sweden, ²⁸Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁹Tirol Kliniken, Innsbruck, Austria, ³⁰Hospital de Santa Maria, Lisbon, Portugal, ³¹Great Ormond Street Hospital, London, United Kingdom, ³²Tel Aviv University, Tel Aviv, Israel, ³³Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³⁴Charite, Berlin, Germany, ³⁵KC Niš, Niš, Serbia, ³⁶German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, ³⁷Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, ³⁸Stony Brook Children's Hospital, Stony Brook, NY, ³⁹University of Missouri, Columbia, ⁴⁰Meir Medical Center, Kfar Saba, Israel, ⁴¹Luzerner Kantonsspital | Kinderspital, Luzern, Switzerland
Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with an estimated prevalence of 1 in 1 000 000 children. In the adult systemic scleroderma…
Abstract Number: 796 • 2019 ACR/ARP Annual Meeting
Under Detection of Interstitial Lung Disease in Juvenile Systemic Sclerosis (jSSc) Utilizing Pulmonary Function Tests: Results from the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Bernd Hinrichs ², Kathryn Torok ³, Maria José Santos ⁴, Ozgur Kasapcopur ⁵, Amra Adrovic ⁶, Valda Stanevicha ⁷, Flavio Sztajnbok ⁸, Maria Teresa Terreri ⁹, Ekaterina Alexeeva ¹⁰, Jordi Antón ¹¹, Maria Katsicas ¹², Vanessa Smith ¹³, Tadej Avcin ¹⁴, Rolando Cimaz ¹⁵, Mikhail Kostik ¹⁶, Thomas Lehman ¹⁷, Walter Sifuentes-Giraldo ¹⁸, Simone Appenzeller ¹⁹, Mahesh Janarthanan ²⁰, Monika Moll ²¹, Dana Nemcova ²², Dieneke Schonenberg ²³, Christina Battagliotti ²⁴, Lillemor Berntson ²⁵, Blanca Bica ²⁶, Juergen Brunner ²⁷, Patricia Costa Reis ²⁸, Despina Eleftheriou ²⁹, Liora Harel ³⁰, Gerd Horneff ³¹, Tilmann Kallinich ³², Dragana Lazarevic ³³, Kirsten Minden ³⁴, Susan Nielsen ³⁵, Farzana Nuruzzaman ³⁶, Anjali Patwardhan ³⁷, Yosef Uziel ³⁸ and Nicola Helmus ¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²Asklepios Klinik Nord - Heidberg, Hamburg, Germany, ³UPMC Children's Hospital of Pittsburgh, Pittsburgh, ⁴Rheumatology department, Hospital Garcia de Orta, Almada, Portugal, ⁵Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey, ⁶Cerrahpaşa Tıp Fakültesi, Istanbul, Turkey, ⁷Riga Stradins University, Riga, Latvia, ⁸Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ⁹Pediatric Rheumatology Unit, Universidade Federal de São Paulo, São Paulo, Brazil, ¹⁰National Medical Research Center of Children`s Health, Moscow, Russia, ¹¹Hospital Sant Joan de Déu, Barcelona, Spain, ¹²Hospital de Pediatria, Buenos Aires, Argentina, ¹³Dept. of Rheumatology, Ghent University Hospital, Ghent, Belgium; Dept. of Internal Medicine, Ghent University, Ghent, Belgium; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium, Gent, Belgium, ¹⁴Ljubljana University Medical Centre, Ljubljana, Slovenia, ¹⁵University Hospital Meyer, Florence, Italy, ¹⁶Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, ¹⁷Hospital for Special Surgery, New York, ¹⁸Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁹UNICAMP Universidade Estadual de Campinas, Sao Paulo, Brazil, ²⁰Sri Ramachandra University, Chennai, India, ²¹University of Tuebingen, Tuebingen, Germany, ²²General University Hospital, Prague, Czech Republic, ²³Amsterdam UMC, Amsterdam, Netherlands, ²⁴Children's Hospital Dr. Orlando Alassia, Santa Fee, Argentina, ²⁵Uppsala University Hospital, Uppsala, Sweden, ²⁶Hospital Universitário Clementino Fraga Filho (HUCFF/UFRJ), Rio de Janeiro, Brazil, ²⁷Tirol Kliniken, Innsbruck, Austria, ²⁸Hospital de Santa Maria, Lisbon, Portugal, ²⁹Great Ormond Street Hospital, London, United Kingdom, ³⁰Tel Aviv University, Tel Aviv, Israel, ³¹Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, ³²Charite, Berlin, Germany, ³³KC Niš, Niš, Serbia, ³⁴German Rheumatism Research Center and Charité University Medicine, Berlin, Germany, ³⁵Department of Pediatrics, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Copenhavn, Denmark, ³⁶Stony Brook Children's Hospital, Stony Brook, NY, ³⁷University of Missouri, Columbia, ³⁸Meir Medical Center, Kfar Saba, Israel
Background/Purpose: Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence in around 3 in a million children[1]. Pulmonary involvement in jSSc occurs in approximately 40…
Abstract Number: 797 • 2019 ACR/ARP Annual Meeting
Characteristics of Coexisting Localized Scleroderma and Juvenile Idiopathic Arthritis
Daniel Reiff¹, Courtney Crayne ², Melissa Mannion ³ and Randy Cron ¹, ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Alabama at Birmingham, Birmingham, ³University of Alabama at Birmingham, Alabama
Background/Purpose: Localized scleroderma (LS), including morphea and linear scleroderma, is an autoimmune disease where excessive collagen deposits underneath the skin lead to thickening, scarring, and…
Abstract Number: 798 • 2019 ACR/ARP Annual Meeting
Development of Large Vessel Vasculitis Including Aortitis in a Patient with Deficiency of the IL-1 Receptor Antagonist (DIRA) Points to Converging Roles of IL-1 and TNF in Vascular Pathogenesis Recapitulating Findings from a Murine Model
Gina Montealegre Sanchez¹, Adriana de Jesus ¹, Jenna Wade ², Katherine Townsend ¹, Arianne Soldatos ³, Alessandra Brofferio ⁴, Peter C. Grayson ⁵, Ginger Janow ⁶ and Raphaela Goldbach-Mansky ¹, ¹Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, ²Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, ³NIH/NINDS, Bethesda, ⁴NIH/NHLBI, Bethesda, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) of the National Institutes of Health, Bethesda, MD, Bethesda, MD, ⁶Joseph M. Sanzari Children's Hospital at Hackensack University Medical Center, NJ
Background/Purpose: Deficiency of interleukin-1-receptor antagonist (DIRA) is a rare autoinflammatory disease caused by autosomal recessive loss of function mutations in IL1RN and characterized by early-onset generalized pustulosis,…
Abstract Number: 799 • 2019 ACR/ARP Annual Meeting
Application of the Autoinflammatory Disease Activity Index (ADDI) to a Cohort of Patients in a Tertiary Hospital
Mireia Lopez-corbeto¹ and Estefania Moreno Ruzafa ¹, ¹Hospital Universitari Vall d'Hebron, Barcelona, Spain
Background/Purpose: Autoinflammatory diseases (AIDs) cause chronic systemic inflammation that can damage multiple organs. Recently, the ADDI index has been developed and validated in the four…
Abstract Number: 800 • 2019 ACR/ARP Annual Meeting
Cryopyrin-Associated Periodic Syndrome in Korea: 19 Years of Experience
Young Ho Kim¹, Bongjik Kim ², Byung Yoon Choi ², Haeng Jin Lee ³, Dae-Chul Jeong ⁴, Jinhee Han ², Hye-Rim Park ², Jayoung Oh ², Seungmin Lee ², Dooyi Oh ⁵ and Soyoung Lee ¹, ¹Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Seoul-t'ukpyolsi, Republic of Korea, ²Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seongnam, Kyonggi-do, Republic of Korea, ³Department of Ophthalmology, Seoul National University Hospital, Seoul, Republic of Korea, ⁴Department of Pediatrics, The Catholic University of Korea, Seoul, Seoul-t'ukpyolsi, Republic of Korea, ⁵Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seoul, Kyonggi-do, Republic of Korea
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) is rare auto-inflammatory disorder characterized by recurrent episodes fever with variable manifestation of systemic inflammation such as urticarial skin rash,…
Abstract Number: 801 • 2019 ACR/ARP Annual Meeting
Preliminary Analysis of Hearing Loss in a Neonatal-Onset Multisystem Inflammatory Disease (NOMID) Cohort Followed over a Mean of 10 Years: Normal Hearing at Baseline and Early Treatment with Anakinra Area Associated with Maintenance of Normal Hearing
Sara Alehashemi¹, Megha Garg ², Kelly King ³, Chris Zalewski ³, Adriana de Jesus ⁴, John Butman ⁵, Jonah Eisenberg ⁶, Carmen Brewer ³, Jeffrey KIm ⁷ and Raphaela Goldbach-Mansky ¹, ¹Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD, ²Rochester Regional Health, Rochester, NY, ³NIDCD/NIH, Bethesda, ⁴Translation Autoinflammatory Diseases Section/NIAID/NIH, Silver Spring, MD, ⁵CC/NIH, Bethesda, ⁶University of Michigan, Ann Arbor, ⁷NIDCD, Bethesda
Background/Purpose: Neonatal-onset multisystem inflammatory disease (NOMID), caused by gain-of-function mutation in the NLRP3 inflammasome, presents with systemic inflammation, rash, eye inflammation, aseptic meningitis and sensorineural…
Abstract Number: 802 • 2019 ACR/ARP Annual Meeting
Canakinumab Improves Patient-Reported Outcomes in Patients with Recurrent Fever Syndromes: Results from a Phase 3 Trial
Helen Lachmann¹, Bernard Lauwerys ², Paivi Miettunen ³, Tilmann Kallinich ⁴, Annette Jansson ⁵, Itzhak Rosner ⁶, Raffaele Manna ⁷, Saramaria Murias ⁸, Sinisa Savic ⁹, Serge Smeets ¹⁰, Fabrizio De Benedetti ¹¹ and Anna Simon ¹², ¹The Royal Free Hospital & University College London, London, United Kingdom, ²Université catholique de Louvain, Brussels, Brussels Hoofdstedelijk Gewest, Belgium, ³Alberta Children's Hospital, Calgary, ON, Canada, ⁴Charite, Berlin, Germany, ⁵Dr. von Hauner Children’s Hospital, Ludwig-Maximilians-University, Munich, Germany, ⁶Bnei Tzion, Haifa, Israel, ⁷Università Cattolica del Sacro Cuore, Rome, Lazio, Italy, ⁸University Hospital La Paz, Madrid, Madrid, Spain, ⁹St James’s University Hospital, Leeds, England, United Kingdom, ¹⁰Novartis, Amsterdam, Netherlands, ¹¹Bambino Gesù Children’s Hospital, Rome, Italy, ¹²Radboud University Medical Centre, Nijmegen, Netherlands
Background/Purpose: Recurrent fever syndromes have a significant impact on health-related quality of life (HRQoL).1 Canakinumab (CAN) has demonstrated efficacy and safety in patients with colchicine-resistant…
Abstract Number: 803 • 2019 ACR/ARP Annual Meeting
Hepatitis a Virus Vaccination in Autoinflammatory Diseases Under Canakinumab and Tocilizumab Treatment
Kenan Barut ¹, Amra Adrovic ², Sezgin Sahin ³, Mehmet Yıldız ², Oya Koker ², Gamze Yalcin ², Omer Faruk Beser ⁴, Bekir Kocazeybek ⁵, Pelin Yuksel ⁵ and Ozgur Kasapcopur⁶, ¹Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, İstanbul, Istanbul, Turkey, ²Department of Pediatric Rheumatology, Istanbul University Cerrahpasa, Istanbul, Istanbul, Turkey, ³Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Istanbul, Turkey, ⁴Department of Pediatrics, Okmeydani Education and Training Hospital, Istanbul, Istanbul, Turkey, ⁵Department of Microbiology, Istanbul University Cerrahpasa, Istanbul, Istanbul, Turkey, ⁶Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Istanbul, Turkey, Istanbul, Turkey
Background/Purpose: Autoimmune, autoinflammatory mechanism and drugs used in treatment increase the risk of liver disease in patients with chronic rheumatic diseases. Hepatitis A vaccine is…

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