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  • Abstract Number: 0191 • ACR Convergence 2021

    Influence of Canakinumab Dosing on Long-term Efficacy and Safety in Patients with Cryopyrin-associated Periodic Syndromes (CAPS) – 30-months Interim Analysis of the RELIANCE Registry

    Jasmin Kuemmerle-Deschner1, Birgit Kortus-Goetze2, Prasad T. Oommen3, Aleš Janda4, Juergen Rech5, Tilmann Kallinich6, Frank Weller-Heinemann7, Gerd Horneff8, Ivan Foeldvari9, Catharina Schuetz10, Michael Borte11, Axel Braner12, Julia Weber-Arden13 and Norbert Blank14, 1Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, 2Division of Nephrology, University of Marburg, Marburg, Germany, 3Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 4Department of Pediatrics, University Hospital Ulm, Ulm, Germany, 5Friedrich-Alexander University (FAU) Erlangen-Nuernberg and Universitaetsklinikum Erlangen, Department of Internal Medicine 3 - Rheumatology and Immunology, Germany, Erlangen, Germany, 6Charité University Medicine, Nuremberg, Germany, 7Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, 8Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, 9Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, 10Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, 11ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Leipzig, Germany, 12Department of Medicine II, Rheumatology, University Hospital Frankfurt, Germany, Frankfurt a.M., Germany, 13Novartis Pharma GmbH, Nuremberg, Germany, 14Rheumatology, University Hospital Heidelberg, Eppelheim, Germany

    Background/Purpose: The IL-1 inhibitor canakinumab (CAN) induces rapid remission of symptoms of CAPS (Cryopyrin-associated periodic syndromes), a monogenic autoinflammatory disease with severe systemic inflammation, both…
  • Abstract Number: 0194 • ACR Convergence 2021

    Eye Opening: Use of IL-6 Inhibition in Non-Paraneoplastic Autoimmune Retinopathy

    Lisa Carnago1, Robert Keenan2, Glenn Jaffe1, Jordan Deaner1 and Dilraj Grewal1, 1Duke Health, Durham, NC, 2Florence Rheumatology Center, Articularis Healthcare, Florence, NC

    Background/Purpose: There is a lack of robust guidance and consistently effective treatment modalities in non-paraneoplastic autoimmune retinopathy (npAIR) with cystoid macula edema (CME), which is…
  • Abstract Number: 0193 • ACR Convergence 2021

    Tocilizumab for the Treatment of Familial Mediterranean Fever – a Randomized, Double Blind, Placebo-controlled Phase II Study

    Jorg Henes1, Sebastian Saur2, David Kofler3, Martin Krusche4, Theodoros Xenitidis5, Christoph Meisner6, Claudia Kedor7, Ina Koetter8, Hendrik Schulze-Koops9 and Eugen Feist10, 1University Hospital Tuebingen, Tuebingen, Germany, 2Universtiy Hostpiatl Tübingen, Tuebingen, Germany, 3Division of Rheumatology and Clinical Immunology, Department I of Internal Medicine; University Hospital Cologne, Cologne, Germany, 4Charite Berlin, Berlin, Germany, 5University Hospital Tuebingen, Rheumatology, Tuebingen, Germany, 6University Tuebingen, Statistics, Tuebingen, Germany, 7Charite University Hospital Berlin, Berlin, Germany, 8University Hospital Hamburg Eppendorf, Hamburg, Germany, 9Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, University of Munich, Munich, Germany, 10Helios Department of Rheumatology, Vogelsang-Gommern, Germany

    Background/Purpose: Familial Mediterranean Fever (FMF) is the most common auto-inflammatory disease characterized by chronic inflammation, recurrent episodes of fever, abdominal and thoracic pain due to…
  • Abstract Number: 0192 • ACR Convergence 2021

    Influence of Canakinumab Dosing on Efficacy and Safety of Long-term Treatment in Patients with Familial Mediterranean Fever – Interim Analysis of the RELIANCE Registry

    Jorg Henes1, Norbert Blank2, Tilmann Kallinich3, Frank Dressler4, Gerd Horneff5, Ivan Foeldvari6, Markus Hufnagel7, Birgit Kortus-Goetze8, Frank Weller-Heinemann9, Florian Meier10, Julia Weber-Arden11 and Jasmin Kuemmerle-Deschner12, 1University Hospital Tuebingen, Tuebingen, Germany, 2Rheumatology, University Hospital Heidelberg, Eppelheim, Germany, 3Charité University Medicine, Nuremberg, Germany, 4Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, 5Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, 6Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, 7Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, 8Division of Nephrology, University of Marburg, Marburg, Germany, 9Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, 101 Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany 2 Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt am Main, Germany, Frankfurt a.M., Germany, 11Novartis Pharma GmbH, Nuremberg, Germany, 12Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany

    Background/Purpose: Familial Mediterranean Fever (FMF) is characterized by severe systemic and organ inflammation. Successful treatment with rapid remission of symptoms and normalization of laboratory parameters…
  • Abstract Number: 0187 • ACR Convergence 2021

    Safety and Tolerability of Nintedanib in Patients with Autoimmune Disease-Related Interstitial Lung Diseases: Pooled Data from the SENSCIS and INBUILD Trials

    Vanessa Smith1, Shervin Assassi2, Yannick Allanore3, Lazaro Loaiza4, Inga Tschoepe5, Madhu Kanakapura4 and Elizabeth Volkmann6, 1Department of Rheumatology and Internal Medicine, Ghent University Hospital, Ghent, Belgium, 2University of Texas McGovern Medical School at Houston, Houston, TX, 3Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, 4Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, Ingelheim, Germany, 5Elderbrook Solutions, Bietigheim-Bissingen, Germany, Bietigheim-Bissingen, Germany, 6Department of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA

    Background/Purpose: The efficacy and safety of nintedanib have been investigated in patients with systemic sclerosis-associated ILD (SSc-ILD) in the SENSCIS trial and in patients with…
  • Abstract Number: 0198 • ACR Convergence 2021

    High Dose Intravenous Methylprednisolone Induces Rapid Improvement of Visual Acuity in Non-Infectious Uveitis of Different Immune Mediated Inflammatory Diseases

    Nuria Vegas-Revenga1, José Luis Martin-Varillas2, Vanesa Calvo-Río3, Iñigo Gonzalez-Mazon4, Lara Sánchez-Bilbao5, Emma Beltrán Catalán6, Alejandro Fonollosa7, Ana Blanco8, Ana Blanco9, Miguel Cordero Coma10, Norberto Ortego11, Ignacio Torre12, Felix Hernandez13, Santiago Muñóz-Fernández14, Maria del Mar Esteban-Ortega14, Manuel Diaz-Llopis15, Joaquin Cañal5, Juan Antonio Ventosa5, Rosalía Demetrio-Pablo16, Lucia Cristina Dominguez Casas17, Santos Castañeda18, Iván Ferraz-Amaro19, Miguel Ángel gonzalez-Gay20 and Ricardo Blanco3, 1Hospital Galdakao, Galdakao, Spain, 2Hospital Sierrallana, Torrelavega, Spain, 3Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 4H. U. Marques de Valdecilla, Santander, Spain, 5Hospital Universitario Marqués de Valdecilla, Santander, Spain, 6Hospital del Mar, Barcelona, Spain, 7Hospital Universitario de Cruces, Barakaldo, Spain, 8Hospital Universitario de Donostia, San Sebastián, 9Hospital Universitario de Donostia, Donostia, Spain, 10Hospital de León, Leon, Spain, 11Medicine Department, Universidad de Granada, Granada, Spain, 12Hospital Universitario de Basurto, Bilbao, Spain, 13Hospital Doctor Negrín, Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Spain, 14Hospital Universitario Infanta Sofia, Madrid, Spain, 15Hospital Universitario La Fe, Valencia, Spain, 16Hospital Universitario de Marqués de Valdecilla, Santander, Spain, 17Hospital Universtario San Agustin, Oviedo, Spain, 18Hospital Universitario de la Princesa, Madrid, Spain, 19Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain, 20Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Division of Rheumatology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa

    Background/Purpose: Rapid and effective remission-inducing therapy is mandatory in uveitis to avoid irreversible structural and functional damage. In some severe cases biological agents might be…
  • Abstract Number: 0197 • ACR Convergence 2021

    Successful Treatment of Severe COVID-19 Pneumonia with Simultaneous Tocilizumab and Anakinra – A Case Series

    Hildrun Haibel1, Stefan Angermair2, Michael Schumann2, Janis Vahldiek3, Denis Poddubnyy4 and Thomas Schneider2, 1Department of Gastroenterology, Infectiology and Rheumatology (including Nutrition Medicine), Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany, 2Charité University Medicine Berlin Campus Benjamin Franklin, Berlin, Germany, 3Charité University Medicine Berlin, Berlin, Germany, 4Department of Rheumatology, Charité – Universitätsmedizin, Berlin, Germany

    Background/Purpose: Severe and life threating COVID-19 pneumonia is often characterized by local and systemic immune-mediated hyperinflammation At the early disease stage activated monocytes are migrating…
  • Abstract Number: 0195 • ACR Convergence 2021

    JAK Inhibitors in Refractory Adult and Childhood-Onset Still’s Disease

    Louise GILLARD1, Stéphane Mitrovic2, Jacques Pouchot3, Fleur Cohen1, Martin Michaud4, Heloise Reumaux5, Isabelle Kone-Paut6 and Bruno Fautrel7, 1Pitié Salpêtrière Hospital, Paris, France, 2Pitié Salpêtrière Hospital - Institut Mutaliste Montsouris, Paris, France, 3AP-HP, RUEIL-MALMAISON, France, 4Joseph Ducuing Hospital, Toulouse, France, 5Jeanne de Flandre Hospital, Lille, France, 6APHP, Bicetre hospital, Le Kremlin bictre, France, 7Pitié Salpêtrière Hospital, APHP, Sorbonne Université, Paris, France

    Background/Purpose: Excessive and inappropriate production of pro-inflammatory cytokines such as interleukin IL-1, IL-6 or IL-18, is a pathogenic cornerstone in adult and childhood onset Still’s…
  • Abstract Number: 0199 • ACR Convergence 2021

    Rapid Improvement in Cystoid Macular Edema with High Dose Intravenous Methylprednisolone in Non-Infectious Uveitis of Different Immune Mediated Inflammatory Diseases

    Nuria Vegas-Revenga1, José Luis Martin-Varillas2, Vanesa Calvo-Río3, Iñigo Gonzalez-Mazon4, Lara Sánchez-Bilbao5, Emma Beltrán Catalán6, Alejandro Fonollosa7, Ana Blanco8, Ana Blanco9, Miguel Cordero Coma10, Norberto Ortego11, Ignacio Torre12, Felix Hernandez13, Santiago Muñóz-Fernández14, Maria del Mar Esteban-Ortega14, Manuel Diaz-Llopis15, Joaquin Cañal5, Juan Antonio Ventosa5, Rosalía Demetrio-Pablo16, Lucia Cristina Dominguez Casas17, Santos Castañeda18, Iván Ferraz-Amaro19, Miguel Ángel gonzalez-Gay20 and Ricardo Blanco3, 1Hospital Galdakao, Galdakao, Spain, 2Hospital Sierrallana, Torrelavega, Spain, 3Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 4H. U. Marques de Valdecilla, Santander, Spain, 5Hospital Universitario Marqués de Valdecilla, Santander, Spain, 6Hospital del Mar, Barcelona, Spain, 7Hospital Universitario de Cruces, Barakaldo, Spain, 8Hospital Universitario de Donostia, San Sebastián, 9Hospital Universitario de Donostia, Donostia, Spain, 10Hospital de León, Leon, Spain, 11Medicine Department, Universidad de Granada, Granada, Spain, 12Hospital Universitario de Basurto, Bilbao, Spain, 13Hospital Doctor Negrín, Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Spain, 14Hospital Universitario Infanta Sofia, Madrid, Spain, 15Hospital Universitario La Fe, Valencia, Spain, 16Hospital Universitario de Marqués de Valdecilla, Santander, Spain, 17Hospital Universtario San Agustin, Oviedo, Spain, 18Hospital Universitario de la Princesa, Madrid, Spain, 19Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain, 20Research group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Division of Rheumatology, Hospital Universitario Marqués de Valdecilla; School of Medicine, Universidad de Cantabria, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa

    Background/Purpose: Cystoid Macular Edema (CME) is the most important cause of blindness in non-infectious uveitis (NIU) (1). Corticosteroids and conventional and/or biological immunosuppressant may be…
  • Abstract Number: 0201 • ACR Convergence 2021

    Australian Psoriatic Arthritis (PsA) Biologic Disease-modifying Antirheumatic Drug (bDMARD) Treatment Pathways Using Sankey Diagrams and Predictive Analysis: A Combined Australian Rheumatology Association Database (ARAD) and Pharmaceutical Benefits Scheme (PBS) Analysis

    Ashley Fletcher1, Lyn March2, Marissa Lassere3, Catherine Hill4, Claire Barrett5, Graeme Carroll6, Premarani Sinnathurai7 and Rachelle Buchbinder8, 1Cabrini Health, Malvern, Australia, 2Institute of Bone and Joint Research, Kolling Institute, University of Sydney; Department of Rheumatology, Royal North Shore Hospital, Northern Sydney Local Health District, St Leonards, Sydney, Australia, 3St George Hospital; School of Population Health, University of New South Wales, Carlton, Australia, 4Queen Elizabeth Hospital, Woodville, Australia, 5Redcliffe Hospital, University of Queensland, Margate Beach, Australia, 6Fiona Stanley Hospital, Perth, Australia, 7Institute of Bone and Joint Research, Kolling Institute, University of Sydney; Department of Rheumatology, Royal North Shore Hospital, Northern Sydney Local Health District, Sydney, Australia, 8Cabrini Health/Monash University, Malvern, Australia

    Background/Purpose: To describe current bDMARD treatment patterns for ARAD participants with PsA, after combining with linked government PBS data.Methods: ARAD, a voluntary longitudinal observational database…
  • Abstract Number: 0200 • ACR Convergence 2021

    In Undifferentiated Arthritis, DMARD-treatment Intensified During the Last Decennia but Did Not Result in Improved Outcomes

    Marloes Verstappen, Xanthe Matthijssen and Annette H.M van der Helm-van Mil, Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: International guidelines stress timely DMARD-initiation in early arthritis, also when classification-criteria are not yet fulfilled. Consequently, undifferentiated arthritis (UA)-patients are increasingly treated despite placebo-controlled…
  • Abstract Number: 0196 • ACR Convergence 2021

    A Single Center, Double Blind, Randomized, Placebo-Controlled Trial of Anakinra in Adult Patients with Features of Cytokine Storm Syndrome in COVID-19

    Lesley Jackson1, Randy Cron1, Nitasha Khullar2, Christopher Chapleau3, Dongmei Sun4 and Winn Chatham1, 1University of Alabama at Birmingham, Birmingham, AL, 2University of Alabama Birmingham, Birmingham, AL, 3UAB Pharmacy, University of Alabama at Birmingham, Birmingham, AL, 4Division of Clinical Immunology and Rheumatology, The University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Some patients with COVID-19 develop respiratory distress and cytokine storm syndrome (CSS) which is characterized by hyperinflammation and may progress to multi-organ failure. Anakinra…
  • Abstract Number: 0190 • ACR Convergence 2021

    Long-term Safety of Canakinumab in Patients with Autoinflammatory Periodic Fever Syndromes – Interim Analysis of the RELIANCE Registry

    Jasmin Kuemmerle-Deschner1, Jorg Henes2, Birgit Kortus-Goetze3, Tilmann Kallinich4, Prasad T. Oommen5, Juergen Rech6, Frank Weller-Heinemann7, Gerd Horneff8, Ivan Foeldvari9, Aleš Janda10, Catharina Schuetz11, Frank Dressler12, Michael Borte13, Markus Hufnagel14, Axel Braner15, Florian Meier16, Michael Fiene17, Julia Weber-Arden18 and Norbert Blank19, 1Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, 2University Hospital Tuebingen, Tuebingen, Germany, 3Division of Nephrology, University of Marburg, Marburg, Germany, 4Charité University Medicine, Nuremberg, Germany, 5Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 6Friedrich-Alexander University (FAU) Erlangen-Nuernberg and Universitaetsklinikum Erlangen, Department of Internal Medicine 3 - Rheumatology and Immunology, Germany, Erlangen, Germany, 7Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, 8Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, 9Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, 10Department of Pediatrics, University Hospital Ulm, Ulm, Germany, 11Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, 12Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, 13ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Leipzig, Germany, 14Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, 15Department of Medicine II, Rheumatology, University Hospital Frankfurt, Germany, Frankfurt a.M., Germany, 161 Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany 2 Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt am Main, Germany, Frankfurt a.M., Germany, 17Department of Internal Medicine, District Hospital Demmin, Demmin, Germany, 18Novartis Pharma GmbH, Nuremberg, Germany, 19Rheumatology, University Hospital Heidelberg, Eppelheim, Germany

    Background/Purpose: Autoinflammatory periodic fever syndromes (PFS) are characterized by severe systemic and organ inflammation. In clinical trials, successful treatment was achieved with the interleukin-1β inhibitor…
  • Abstract Number: 0171 • ACR Convergence 2021

    Bone Erosions and Osteophytes in Premenopausal Women with Long-standing Rheumatoid Arthritis: Association with Systemic Bone Involvement Using HR-pQCT

    Mariana Perez1, Camille Figueiredo1, Lucas Sales1, ANA MEDEIROS-RIBEIRO1, Liliam Takayama1, Diogo Domiciano1, Karina Bonfiglioli1, Valeria Caparbo1 and Rosa Pereira2, 1Rheumatology Division, University of São Paulo, São Paulo, SP, Brazil, 2Rheumatology Division, University of São Paulo, São Paulo, Brazil

    Background/Purpose: Two patterns of bone involvement are described in rheumatoid arthritis (RA), systemic and localized. Systemic bone involvement is characterized by loss of generalized bone…
  • Abstract Number: 0142 • ACR Convergence 2021

    Description of a Scleroderma Cohort and Management of Lung Disease Risk at a Rural Academic Medical Center

    Jenna Marinock1, P. Daniel Nicholas2, Kirsten Koons3, Rakesh Kriplani4, Andrea Berger4 and David Bulbin5, 1Geisinger Commonwealth School of Medicine, Philadelphia, PA, 2Geisinger Medical Center, Danville, PA, 3Geisinger Medical Center, Lewisburg, PA, 4Department of Population Health Sciences, Geisinger Medical Center, Danville, PA, 5Geisinger Health System, Danville, PA

    Background/Purpose: Systemic sclerosis interstitial lung disease (SSc-ILD) and pulmonary hypertension (SSc-PH) are leading causes of morbidity and mortality in patients with SSc. Few screening guidelines…
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