Abstracts tagged "Pediatric rheumatology"

Abstract Number: 1222 • ACR Convergence 2023
The Association Between Gingival Inflammation and Clinical Signs of Active Juvenile Dermatomyositis (JDM)
Albert Chow¹, Hyun Song², Laurie Brenchley³, Nastaran Bayat⁴, Mary Eckert⁵, Sean Koester⁶, adam Schiffenbauer⁷, Rita Volochayev⁷, Pamela Gardner³, Peggy Lee⁸, Jeffrey McLean⁹, Susan Shenoi¹⁰, Lisa Rider¹¹, Anne Stevens¹² and Neelendu Dey¹³, ¹Loma Linda University, Loma Linda, CA, ²Department of Dentistry, Seattle Children's Hospital, Seattle, WA, ³Office of the Clinical Director, NIDCR, National Institutes of Health, Bethesda, MD, ⁴Social Scientific Systems, DLH Holdings Corp, Silver Spring, MD, ⁵Center for Clinical and Translational Research, Seattle Children’s Research Institute, Seattle, WA, ⁶Translational Science and Therapeutics Division, Fred Hutchinson Cancer Center, Seattle, WA, ⁷Environmental Autoimmunity Group, Clinical Research Branch, National Institute of Environmental Health Sciences, National Institutes of Health, Bethesda, MD, ⁸Department of Oral Medicine, School of Dentistry, University of Washington, Seattle, WA, ⁹Department of Periodontics, University of Washington, Seattle, WA, ¹⁰Seattle Childrens Hospital, Mercer Island, WA, ¹¹NIEHS, NIH, Bethesda, MD, ¹²Janssen, Hansville, WA, ¹³Department of Medicine, Division of Gastroenterology, University of Washington, Seattle, WA
Background/Purpose: JDM is a rare vasculopathy of unknown etiology characterized by typical rashes and proximal weakness. JDM has been associated with gingivitis, which can be…
Abstract Number: 1239 • ACR Convergence 2023
Clinical Manifestations and Management of Takayasu Arteritis: A Multicenter Pediatric Cohort
Ana Luiza Altaffer¹, Kristina Ciaglia², Elizabeth Sloan³ and Marietta De Guzman⁴, ¹Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ²UT Southwestern and Scottish Rite for Children, Dallas, TX, ³UT Southwestern, Dallas, TX, ⁴Baylor College of Medicine, Houston, TX
Background/Purpose: Takayasu arteritis (TA) is a rare granulomatous vasculitis that affects large vessels, including the aorta, its major branches, and the pulmonary artery. Data on…
Abstract Number: 1454 • ACR Convergence 2023
Neuropsychiatric Systemic Lupus Erythematosus in Children: A Scoping Review
Alexandra Theisen¹, Ekemini Ogbu², Emily Beil³, Onengiya Harry⁴, Simone Appenzeller⁵, Ryan Kammeyer⁶, Hanne Van der Heijen⁷, Andrea Knight⁸, Hannah Craven⁹ and Martha Rodriguez¹⁰, ¹Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children's Hospital, St. Louis, MO, ²Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Johns Hopkins University, Cincinnati, OH, ³Baylor College of Medicine / Texas Children's Hospital, Houston, TX, ⁴Brenner Children's Hospital - Wake Forest Baptist Health, Winston-Salem, NC, ⁵UNICAMP, Campinas, Brazil, ⁶University of Colorado School of Medicine, Children's Hospital Colorado, Denver, CO, ⁷Boston Children's Hospital, Harvard Medical School, Boston, MA, ⁸The Hospital for Sick Children, Toronto, ON, Canada, ⁹Ruth Lilly Medical Library, Indiana University School of Medicine, Indianapolis, IN, ¹⁰Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN
Background/Purpose: Neuropsychiatric systemic lupus erythematosus (NPSLE) remains a challenging entity to diagnose and treat. The clinical heterogeneity of NPSLE coupled with the difficulty with attribution…
Abstract Number: 2039 • ACR Convergence 2023
Clinical, Immunologic, and Genetic Characteristics in Patients with Syndrome of Undifferentiated Recurrent Fevers (SURF)
Marci Macaraeg, Michael Matt, Elizabeth Handorf, Elizabeth Baker and Grant Schulert, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Unexplained recurrent fevers are a common presentation to pediatric rheumatology and a significant burden to affected families due to missed daycare or school days…
Abstract Number: 2061 • ACR Convergence 2023
Evaluation of a Tool to Enhance Training of the Physical Examination of the Temporomandibular Joint (TM Joint) in Juvenile Idiopathic Arthritis (JIA)
Tova Ronis¹, Nancy Pan², Rebecca Sadun³, Melissa Lerman⁴, Cory Resnick⁵, James Bost⁶, Peter Stoustrup⁷, Marinka Twilt⁸ and for the CARRA TMJ Arthritis Workgroup⁹, ¹Children's National Hospital, Chevy Chase, MD, ²Hospital for Special Surgery, New York, NY, ³Duke University, Durham, NC, ⁴Children's Hospital of Philadelphia, Philadelphia, PA, ⁵Harvard Medical School, Boston, MA, ⁶Children's Health Center, Children's National Medical Center, Washington, DC, ⁷Aarhus University, Aarhus, Denmark, ⁸Alberta Children's Hospital, Calgary, AB, Canada, ⁹Childhood Arthritis and Rheumatology Research Alliance, Washington, DC
Background/Purpose: Arthritis of the TM joint is a frequent finding in patients with JIA, potentially leading to dentofacial deformities, pain, and lower quality of life.1…
Abstract Number: 0359 • ACR Convergence 2023
Clinical Characteristics of Patients with Juvenile Idiopathic Arthritis Who Undergo Adalimumab Drug Level Testing and Anti-Drug Antibody Assessment
Chelsea Vallejos¹, Jennifer Cooper² and Ingrid Pan¹, ¹Children's Hospital Colorado, Aurora, CO, ²University of Colorado/Children's Hospital Colorado, Denver, CO
Background/Purpose: Adalimumab therapeutic drug monitoring (TDM) using established trough concentrations is not standard practice in JIA patients, unlike IBD. No specific guidelines outline a therapeutic…
Abstract Number: 0708 • ACR Convergence 2023
NFKB1 and NFKBIA: Relevant Players in the Pathogenesis of IgA Vasculitis?
Joao Carlos Batista-Liz¹, Verónica Pulito-Cueto¹, María Sebastián Mora-Gil¹, Belén Sevilla-Pérez², María Teresa Leonardo³, Norberto Ortego-Centeno⁴, Ana Peñalba³, Javier Narvaez⁵, luis martin penagos⁶, Emilio Rodrigo⁶, Lara Belmar-Vega⁶, Cristina Gomez-Fernandez⁷, Luis Caminal-Montero⁸, Paz Collado⁹, Miren Uriarte-Ecenarro¹⁰, Esther Vicente Rabaneda¹¹, Esteban Rubio¹², Manuel León Luque¹², Juan María Blanco-Madrigal¹³, Eva Galindez-Agirregoikoa¹⁴, Santos Castañeda¹⁵, Miguel A Gonzalez-Gay¹⁶, Ricardo Blanco¹⁷ and Raquel López-Mejías¹, ¹Rheumatology Department, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, ²Division of Pediatrics, Hospital Universitario San Cecilio, Granada, Spain, ³Division of Pediatrics, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁴Medicine Department, Universidad de Granada, Granada, Spain, ⁵Hospital Universitario de Bellvitge, Barcelona, Spain, ⁶Division of Nephrology, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-DIVAL, Santander, Spain, ⁷Division of Dermatology,Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, ⁸Internal Medicine Department, Hospital Universitario Central de Asturias, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Oviedo, Spain, ⁹Division of Rheumatology, Hospital Universitario Severo Ochoa, Madrid, Spain, ¹⁰Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, ¹¹Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ¹²Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain, ¹³Division of Rheumatology, Hospital Universitario de Basurto, Bilbao, Spain, ¹⁴Basurto University Hospital, Bilbao, Spain, ¹⁵Hospital Universitario de la Princesa, Madrid, Spain, ¹⁶IDIVAL and School of Medicine, UC, Santander; Department of Rheumatology, IIS-Fundación Jiménez Díaz, Madrid, Santander, Spain, ¹⁷Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Immunoglobulin A Vasculitis (IgAV) is a B-cell-mediated inflammatory disease. NF-kappa B (NF-kB) plays a key role in autoimmunity and inflammation1. In this regard, the…
Abstract Number: 1223 • ACR Convergence 2023
Extracorporeal Life Support for Childhood-Onset Systemic Lupus Erythematosus: An ELSO Registry Analysis
Carla Levin¹, Andrea Ontaneda², Danielle Guffey³, Peter Rycus⁴, Marc Anders², Eyal Muscal⁵ and Maria Pereira⁵, ¹Department of Pediatrics, Baylor College of Medicine, Houston, TX, ²Division of Critical Care, Department of Pediatrics, Baylor College of Medicine, Houston, TX, ³Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, TX, ⁴Extracorporeal Life Support Organization, Ann Arbor, MI, ⁵Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a heterogeneous multisystemic autoimmune disorder that can cause life-threatening complications. There is a paucity of data on the utility…
Abstract Number: 1240 • ACR Convergence 2023
Obvious Only in Retrospect: A Cohort of STING Associated Vasculopathy in Infancy (SAVI) Without Typical Rash
Ilaria Maccora¹, Patricia Vega-Fernandez², Kimberly Risma³ and Grant Schulert⁴, ¹PhD student, in the Area of Drugs and Innovative Treatments, NeuroFARBA Department, University of Florence. Meyer Children's Hospital, Firenze, Italy, ²Division of Rheumatology, Cincinnati Children's Hospital, Medical Centre, Cincinnati, OH, ³Division of Allergy and Immunology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is characterized by systemic inflammation, skin vasculopathy and interstitial lung disease. However, since the initial description knowledge…
Abstract Number: 1621 • ACR Convergence 2023
Maxillofacial MRI Augments Detection of Non-CNS Abnormalities in Pediatric Craniofacial Scleroderma
Daniel Glaser¹, Amy Metry², Elena DeRosas², Subramanian Subramanian² and Kathryn Torok³, ¹Yale University School of Medicine, New Haven, CT, ²University of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA
Background/Purpose: Craniofacial scleroderma is a rare subtype of linear scleroderma affecting the head and neck. Up to 40% of patients experience extracutaneous manifestations (ECMs), but…
Abstract Number: 2040 • ACR Convergence 2023
Distinguishing Multisystem Inflammatory Syndrome in Children from Typhus Using Artificial Intelligence: MISC vs. Endemic Typhus (AI-MET)
Angela Chun¹, Abraham Bautista-Castillo², Isabella Osuna¹, Kristiana Nasto¹, Flor Munoz³, Gordon Schutze¹, Sridevi Devaraj¹, Eyal Muscal⁴, Marietta De Guzman⁵, Kristen Sexson Tejtel¹, Ioannis Kakadiaris² and Tiphanie Vogel¹, ¹Baylor College of Medicine / Texas Children's Hospital, Houston, TX, ²Computational Biomedicine Lab University of Houston, Houston, TX, ³Baylor College of Medicine, Texas Children's Hospital, Houston, TX, ⁴Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, ⁵Baylor College of Medicine, Houston, TX
Background/Purpose: Multisystem inflammatory syndrome in children (MIS-C) following SARS-CoV2infection is a recognized mimic of other inflammatory disorders, including Kawasaki Disease and macrophage activation syndrome. However,…
Abstract Number: 2062 • ACR Convergence 2023
A Mental Health Workshop for Pediatric Rheumatology Fellows
Caitlan Pinotti¹, Alison Manning², Suzanne Edison³ and Rebecca Sadun¹, ¹Duke University, Durham, NC, ²Lifespan, East Providence, RI, ³Cure JM Foundation, Seattle, WA
Background/Purpose: Many children with a rheumatic disease have concurrent mental and behavioral health challenges, but only ~40% of these symptoms are brought to medical attention.…
Abstract Number: 0028 • ACR Convergence 2023
Adenosine Deaminase 2 Is Expressed as a Short Isoform Lacking Deaminase Activity in the Endothelium: Implications for DADA2 Vasculitis
Andrew Porter¹, Robert Maughan¹, Charis Pericleous¹, Lida Kabir¹, Richard Stratton², Dorian Haskard¹, Pui Lee³, Taryn Youngstein¹ and Justin Mason¹, ¹National Heart & Lung Institute, Imperial College London, London, United Kingdom, ²University College London, London, United Kingdom, ³Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive condition caused by biallelic variants in the Adenosine deaminase 2 (ADA2) gene. Clinical manifestations…
Abstract Number: 0360 • ACR Convergence 2023
Increasing the Etanercept Dose in Juvenile Idiopathic Arthritis Patients: Does It Help Reaching the Treatment Target? A Post-hoc Analysis of the Best4Kids Randomised Clinical Trial
Bastiaan van Dijk¹, Sytske Anne Bergstra¹, Merlijn van den Berg², Dieneke Schonenberg-Meinema², Lisette van Suijlekom-Smit², Marion van Rossum³, Yvonne Koopman⁴, Rebecca Ten Cate¹, CF Allaart¹, Danielle Brinkman¹ and Petra Hissink Muller¹, ¹Leiden University Medical Center, Leiden, Netherlands, ²Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam, Netherlands, ³Amsterdam Rheumatology and Immunology Center | Reade, Amstelveen, Netherlands, ⁴HagaZiekenhuis Juliana Children's Hospital, The Hague, Netherlands
Background/Purpose: The relation between etanercept dose and clinical outcomes of juvenile idiopathic arthritis (JIA) is unclear. Most studies only evaluated doses up to 0.8 mg/kg/week…
Abstract Number: 0760 • ACR Convergence 2023
Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still’s Disease Treated with Emapalumab: The REAL-HLH Study
Carl Allen¹, Shanmuganathan Chandrakasan², Michael Jordan³, Jennifer Leiding⁴, Abiola Oladapo⁵, Priti Pednekar⁶, Kelly Walkovich⁷ and John Yee⁵, ¹Baylor College of Medicine, Houston, TX, ²Emory University School of Medicine, Atlanta, GA, ³Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, ⁴Division of Allergy and Immunology, Department of Pediatrics, Johns Hopkins University; bluebird bio, Cambridge, MA, ⁵Sobi, Inc., Waltham, MA, ⁶PRECISIONheor, Bethesda, MD, ⁷Division of Pediatric Hematology Oncology, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI
Background/Purpose: Macrophage activating syndrome (MAS) is a rare, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD). MAS (a form…

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