ACR Meeting Abstracts

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Abstracts tagged "Autoinflammatory diseases"

  • Abstract Number: 0083 • ACR Convergence 2020

    Chronic Nonbacterial Osteomyelitis Is Associated with HLA-B*27

    Daire O'Leary1, Orla Killeen2 and Anthony Wilson1, 1UCD Centre for Arthritis Research, Dublin, Ireland, 2National Centre for Paediatric Rheumatology, CHI at Crumlin, Dublin, Ireland

    Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is an auto-inflammatory condition primarily affecting children with an estimated prevalence of 1 per 105 - 106.  It is characterized…
  • Abstract Number: 0947 • ACR Convergence 2020

    STING Gain-of-Function in Radio-resistant Cells Supports a Lymphocyte Dependent Auto-inflammatory Lung Disease

    Kevin Gao1, Mona Motwani1, Ann Marshak-Rothstein2 and Katherine Fitzgerald1, 1University of Massachusetts medical school, worcester, MA, 2University of Massachusetts medical school, Newton, MA

    Background/Purpose: cGAS-STING is a cytosolic dsDNA sensing pathway whose regulation is vital to immune homeostasis. Pediatric patients with constitutively active STING mutations develop an autoinflammatory…
  • Abstract Number: 1455 • ACR Convergence 2020

    Functional Characterization of PLCG2 Mutations Found in Subjects with Autoinflammation and PLCG2-Associated Antibody Deficiency and Immune Dysregulation (APLAID) Reveals Both Hypermorphic and Hypomorphic Mutants

    Kathleen Baysac1, Charles Fisher2, Hiroto Nakano2, Joshua Milner3 and Michael Ombrello4, 1NIAMS, NIH, Bethesda, MD, 2NIAMS, NIH, Bethesda, 3Division of Allergy, Immunology and Rheumatology Columbia University Medical Center, New York, NY, 4Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD

    Background/Purpose: PLCG2-associated antibody deficiency and immune dysregulation (PLAID) and autoinflammatory PLAID (APLAID) are autosomal dominant diseases caused by mutations of PLCG2. APLAID is clinically characterized by episodic…
  • Abstract Number: 0095 • ACR Convergence 2020

    Systematic Evaluation of Nine Monogenic Autoinflammatory Diseases Reveals Common and Disease-Specific Correlations with Allergy-Associated Features

    Moses Kitakule1, Brian Dizon2, Cristhian Gutierrez-Huerta1, Sarah Blackstone2, Aarohan Burma1, Aran Son1, Natalie Deuitch1, Sofia Rosenzweig1, Hirsh Komarow1, Deborah Stone3, Anne Jones4, Michele Nehrbecky1, Patrycja Hoffmann5, Tina Romeo6, Adriana de Jesus7, Sara Alehashemi8, Megha Garg9, Sofia Torreggiani10, Gina Montealegre Sanchez11, Katelin R. Honer10, Karyl Barron1, Ivona Aksentijevich4, Amanda Ombrello12, Raphaela Goldbach-Mansky13, Daniel Kastner14, Joshua Milner15, Pamela Frischmeyer-Guerrerio1 and Daniella Schwartz16, 1National Institutes of Health, Bethesda, 2National Institutes of Health, BETHESDA, MD, 3NIH, Bethesda, 4National Human Genome Research Institute, Bethesda, MD, 5NIH, Vienna, VA, 6NIH, Bethesda, MD, 7Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, 8Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, 9NIH/NIAID, Rochester, NY, 10Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, 11NIAID/NIH, Rockville, MD, 12National Human Genome Research Institute/National Institutes of Health, Bethesda, MD, 13Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD, 14National Human Genome Research Institute (NHGRI), NIH, Bethesda, MD, 15Division of Allergy, Immunology and Rheumatology Columbia University Medical Center, New York, NY, 16National Institutes of Health Clinical Center, Bethesda, MD

    Background/Purpose: Monogenic autoinflammatory diseases (AID) are caused by mutations in innate immune signaling genes. The effect of these mutations on the risk of allergy is…
  • Abstract Number: 0965 • ACR Convergence 2020

    Repetitive Inhalant Lipopolysaccharide Exposure in the Setting of Arthritis Induction Potentiates Pro-Fibrotic Inflammatory Lung Disease in Mice

    Madison Wolfe1, Ted Mikuls1, Geoffrey Thiele1, Amy Nelson1, Michael Duryee1, Rohit Gaurav1, Bryant England1, Debra Romberger1 and Jill Poole1, 1University of Nebraska Medical Center, Omaha, NE

    Background/Purpose: Rheumatoid arthritis (RA) is associated with several lung diseases. Various airborne exposures have been implicated as RA disease risk factors including cigarette smoke and…
  • Abstract Number: 1479 • ACR Convergence 2020

    Long-Term Effectiveness of Canakinumab in Autoinflammatory Diseases – Interim Analysis of the CAPS Subgroup from the RELIANCE Registry

    Jasmin Kuemmerle-Deschner1, Birgit Kortus-Goetze2, Michael Borte3, Ivan Foeldvari4, Gerd Horneff5, Ales Janda6, Tilmann Kallinich7, Prasad T. Oommen8, Catharina Schuetz9, Frank Weller-Heinemann10, Julia Weber-Arden11 and Norbert Blank12, 1University Hospital Tuebingen, Tuebingen, Germany, 2Division of Nephrology, University of Marburg, Germany, Marburg, Germany, 3ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Germany, 4Head of the Hamburg Centre for Pediatric and Adolescence Rheumatology, Budapest, Hungary, 5Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, 6Department of Pediatrics, University Hospital Ulm, Germany, Ulm, Germany, 7Charite, Berlin, Germany, 8Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 9Department of Pediatric Immunology, University Hospital Dresden, Dresden, Germany, Dresden, Germany, 10Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, Bremen, Germany, 11Novartis Pharma GmbH, Nuremberg, Germany, 12Department of Hematology, Oncology and Rheumatology, Internal Medicine V, University Hospital of Heidelberg, Eppelheim, Germany

    Background/Purpose: In the treatment of monogenic autoinflammatory diseases (AID), a heterogeneous group of diseases with excessive interleukin (IL)-1β release and severe systemic and organ inflammation,…
  • Abstract Number: 0097 • ACR Convergence 2020

    Does Testing for SAA Is More Beneficial Than CRP for the Follow-up of Patients with FMF?

    Mert Oztas1, Serdal Ugurlu1, Oguzhan Selvi2, Bilgesu Ergezen1 and Huri Ozdogan1, 1Istanbul University-Cerrahpasa, Istanbul, Turkey, 2Okmeydani Research and Training Hospital, Istanbul, Turkey

    Background/Purpose: In order to follow subclinical inflammation and adjust the therapy for an optimal disease control, clinicians seek for readily accessible, affordable and reproducible markers.…
  • Abstract Number: 1035 • ACR Convergence 2020

    Study of Ocular Sarcoidosis and Clusters of Clinical Associations in a Series of 383 Patients with Systemic Sarcoidosis from a Single Hospital

    Inigo Gonzalez-Mazon1, Carmen Alvarez-Reguera2, Lara Sanchez-Bilbao2, David Martinez-Lopez3, Alba Herrero Morant2, Jorge Javier Gaitan-Valdizan4, Raul Fernandez-Ramon2, Rosalia Demetrio-Pablo2 and Ricardo Blanco2, 1Hospital Universitario Marques de Valdecilla, Bezana, Spain, 2Hospital Universitario Marques de Valdecilla, Santander, Spain, 3Hospital Universitario Marques de Valdecilla, Santander (SPAIN), Spain, 4Hospital Universitario Marqués de Valdecilla, Santander, Spain

    Background/Purpose: Sarcoidosis is an inflammatory disease which can affect multiple organs. The most frequent affected organs are lungs, skin and eyes. Ocular involvement is a…
  • Abstract Number: 1531 • ACR Convergence 2020

    Characterization of Cytokine/chemokine Profile in Patient-derived M1/ M2 Macrophages to Identify Biomarkers for Genetically-defined Systemic Autoinflammatory Diseases

    Farzana Bhuyan1, Adriana Almeida de Jesus2, Kim Johnson3, Jacob Mitchell4, Yan Huang5 and Raphaela Goldbach-Mansky5, 1NIH, bhetesda, MD, 2Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, 3NIH, NIAID, Bethesda, 4Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, 5NIH, Bethesda

    Background/Purpose: Genetic mutations in key regulatory molecules of the innate immune system cause autoinflammatory diseases through propagation of hyperinflammatory responses. Monocytes/ macrophages regulate inflammatory processes…
  • Abstract Number: 0161 • ACR Convergence 2020

    Validation of Patient-reported Outcomes (PRO) Lung Questionnaires for Systemic Juvenile Idiopathic Arthritis (SJIA) Patients at Risk for Lung Disease

    Kim Nguyen1, Christopher Towe2, Shima Yasin3, Alexei Grom4, Hermine I Brunner5 and Grant Schulert5, 1Veterans Affairs Cincinnati, University of Cincinnati Medical Center, Cincinnati, 2Cincinnati Children's Medical Hospital Center, Cincinnati, 3Cincinnati Children's Hospital Medical Center, Cincinnati, 4Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) involves dysregulation of inflammation and innate immunity, and can cause life-threatening complications including lung disease (LD). However, there are…
  • Abstract Number: 1063 • ACR Convergence 2020

    Spirulina Stimulates Inflammatory Cytokine Production Through the STING and TLR Pathways in Dermatomyositis in Vitro

    Christina Bax1, Yubin Li1, Spandana Maddukuri2, Adarsh Ravishankar3, Jay Patel3, Daisy Yan1, Josef Symon Concha1 and Victoria Werth1, 1University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, PA, 2University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Montville, NJ, 3University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia

    Background/Purpose: Spirulina, a popular herbal supplement, stimulates the immune system, as determined by in vitro and in vivo studies. Our recent epidemiologic data suggest that Spirulina…
  • Abstract Number: 1578 • ACR Convergence 2020

    Rituximab Hypersensitivity in Rheumatic and Inflammatory Diseases: Role of Skin Testing

    Sravani Penumarty1, Javier Quintero Betancourt2, Eugenio Capitle2 and Reena Khianey2, 1Rutgers, Eastvale, CA, 2Rutgers, Newark, NJ

    Background/Purpose: Infusion-related reactions have been reported with rituximab, a monoclonal antibody targeting the CD20 antigen on B cells, and may result in discontinuation of the…
  • Abstract Number: 0162 • ACR Convergence 2020

    Identifying Sleep Problems in Systemic Juvenile Idiopathic Arthritis (SJIA) Patients with Patient-reported Outcomes (PRO) Questionnaires

    Kim Nguyen1, Christopher Towe2, Shima Yasin3, Alexei Grom4, Hermine I Brunner5 and Grant Schulert5, 1Veterans Affairs Cincinnati, University of Cincinnati Medical Center, Cincinnati, 2Cincinnati Children's Medical Hospital Center, Cincinnati, 3Cincinnati Children's Hospital Medical Center, Cincinnati, 4Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) can cause severe and chronic multisystem involvement. Medical therapies including high-dose corticosteroids can have significant side effects affecting sleep.…
  • Abstract Number: 1152 • ACR Convergence 2020

    IL-18: A Biomarker That Reflects Disease Activity, Could It Be the Next Disease Activity Measure in Systemic Juvenile Idiopathic Arthritis?

    Shima Yasin1, Thuy Do2, Sanjeev Dhakal2, Elizabeth Baker2, Alexei Grom3 and Grant Schulert4, 1Cincinnati Children's Hospital Medical Center, Liberty twp, OH, 2Cincinnati Children's Hospital Medical Center, Cincinnati, 3Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with prominent innate immune activity. Disease presentation and flares could largely mimic infections with fever…
  • Abstract Number: 1636 • ACR Convergence 2020

    8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab

    Dror Mevorach1 and Netanel Karbian1, 1Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel

    Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…
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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.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

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