ACR Meeting Abstracts

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  • Abstract Number: 1670 • ACR Convergence 2020

    Low Copy Number of Long C4 Genes Is a Genetic Risk Factor for Childhood Onset SLE (cSLE) but Is Associated with Higher Age of Disease Onset

    Fatima Barbar-Smiley1, Danlei Zhou2, Joanne Drew2, Bi Zhou2, Cagri Yildirim-Toruner2, Vidya Sivaraman3, Wael Jarjour4, Stacy Ardoin2 and Chack-Yung Yu5, 1Nationwide Children's Hospital/The Ohio State University, Columbus, OH, 2Nationwide Children's Hospital, Columbus, OH, 3Nationwide Children's Hospital, Bexley, OH, 4The Ohio State University, Columbus, OH, 5Abigail Wexner Research Institute at Nationwide Children's Hospital, Columbus, OH

    Background/Purpose: Hypocomplementemia is a marked feature of systemic lupus erythematosus (SLE), which may be a result of consumption initiated by immune complexes between self-nuclear antigens…
  • Abstract Number: 1671 • ACR Convergence 2020

    Identifying Rare Genetic Variants in Childhood-onset Monogenic Systemic Lupus Erythematosus

    Melissa Misztal1, Fangming Liao2, Sergey Naumenko3, Andrea Knight4, Daniela Dominguez5, JingJing Cao2, Declan Webber6, Bhooma Thiruvahindrapuram7, Deborah Levy6, Andrew Paterson2, Earl D. Silverman8 and Linda Hiraki9, 1Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Oakville, ON, Canada, 2Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Toronto, Canada, 3The Centre for Computational Medicine, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada, 4Division of Rheumatology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, ON, Canada, 5Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada, 6Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 7The Centre for Applied Genomics, The Hospital for Sick Children, Toronto, 8Division of Rheumatology, The Hospital for Sick Children, Translational Medicine, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada, 9Division of Rheumatology, The Hospital for Sick Children, Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada

    Background/Purpose: Among children diagnosed with systemic lupus erythematosus (SLE), there exists monogenic forms of SLE, where rare variants in a single gene lead to disease.…
  • Abstract Number: 1672 • ACR Convergence 2020

    Renal Activity Index for Lupus Nephritis Distinguishes Active Renal Disease Among Childhood Systemic Lupus Erythematosus Patients

    Najla Aljaberi1, Arjun Mathur2, Steffy Jose3, Theresa Hennard4, Angela Merritt1, Qing Ma5, James Rose5, Rashmi Sahay6, Chunyan Liu6, Scott Wenderfer7 and Hermine I Brunner8, 1Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, 3Renal Section, Baylor College of Medicine and Texas Children's Hospital, Houston, 4Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5Divison of Nephrology and Hypertension, Cincinnati Children's Hospital Medical Center, Cincinnati, 6Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, 7Renal Section, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, 8PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Renal involvement in childhood-onset systemic lupus erythematosus (cSLE) is a major cause of morbidity and mortality. Current tools to identify lupus nephritis (LN) fall…
  • Abstract Number: 1673 • ACR Convergence 2020

    Principles of Pediatric Lupus Nephritis in a Contemporary Multi-Center Cohort

    Kathleen Vazzana1, Ankana Daga2, Beatrice Goilav3, Ekemini Ogbu4, Daryl Okamura5, Catherine Park6, Rebecca Sadun7, Emily Smitherman8, Brian Stotter9, Scott Wenderfer10 and Laura Lewandowski11, 1National Institute of Health/Children's National Hospital, Washington, DC, 2Harvard, Boston, MA, 3The Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, 4Johns Hopkins University, Baltimore, MD, 5Seattle's Children's, Seattle, WA, 6Emory University, Atlanta, GA, 7Duke University, Durham, NC, 8University of Alabama at Birmingham, Birmingham, AL, 9Washington University School of Medicine, St. Louis, MO, 10Renal Section, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, 11NIAMS, NIH, Rockville, MD

    Background/Purpose: Lupus nephritis (LN) is a well-established and life-threatening manifestation of systemic lupus erythematosus (SLE) that is more common in children than adults. The demographics…
  • Abstract Number: 1674 • ACR Convergence 2020

    Pediatric and Adolescent One Year Protocol Kidney Biopsies Should Be Performed, Even in Patients with Complete Remission of Their Lupus Nephritis

    Peter Yorgin1, Suhas Radhakrishna1, Caitlin Carter1, Johanna Chang1, Katayoon Shayan1, Lieuko Nguyen1, Peter Chiraseveenuprapund1 and Robert Sheets2, 1UCSD/Rady Children's Hospital, San Diego, CA, 2University of California San Diego, San Diego, CA

    Background/Purpose: The value of one-year protocol kidney biopsies in children who have proliferative lupus nephritis (LN) is unclear, particularly in patients who meet CARRA criteria…
  • Abstract Number: 1675 • ACR Convergence 2020

    Microstructural Damage Is Associated with Age at Disease-onset and Cognitive Impairment in Systemic Lupus Erythematosus

    Paulo Julio1, Renan Frittoli1, Aline Lapa1, Thais Caldeira1, Leticia Rittner1, Fernando Cendes1, Roberto Marini1, Paula Fernandes1, Lilian Costallat1 and Simone Appenzeller1, 1UNICAMP, Campinas, Brazil

    Background/Purpose: To compare corpus callosum (CC) volume and diffusion tensor imaging in systemic lupus erythematosus according to age of disease-onset. Methods: We selected 75 patients with…
  • Abstract Number: 1676 • ACR Convergence 2020

    Neuropsychiatric Involvement in Juvenile-onset Systemic Lupus Erythematosus (JSLE): Data from the UK JSLE Cohort Study

    Teresa Giani1, Eve Smith2, Rolando Cimaz3, Michael Beresford4 and Christian Hedrich2, 1AOU Meyer, Florence, Italy, 2University of Liverpool, Liverpool, United Kingdom, 3ASST Gaetano Pini-CTO Institute and Università degli Studi di Milano, Milan, Italy, Milan, Italy, 4Department of Women's & Children's Health, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom

    Background/Purpose: Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease, accounting for up to 20% of SLE cases. Though clinically similar to adult-onset disease,…
  • Abstract Number: 1677 • ACR Convergence 2020

    Schizophrenia Genetics and Neuropsychiatric Features in Childhood-Onset Systemic Lupus Erythematosus

    Ana C. Ulloa Baez1, Fangming Liao2, Raffaella Carlomagno3, Talia Diaz3, Daniela Dominguez4, Deborah Levy3, Lawrence Ng5, Earl D. Silverman6, Andrea Knight7 and Linda Hiraki8, 1Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada, 2Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Toronto, Canada, 3Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 4Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada, 5Division of Rheumatology, Hospital for Sick Children, Toronto, Canada, 6Division of Rheumatology, The Hospital for Sick Children, Translational Medicine, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada, 7Division of Rheumatology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, ON, Canada, 8Division of Rheumatology, The Hospital for Sick Children, Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada

    Background/Purpose: Prior studies indicate that schizophrenia and systemic lupus erythematosus (SLE) share genetic risk loci. Despite overlapping phenotypic features such as psychosis, little is known…
  • Abstract Number: 1678 • ACR Convergence 2020

    Hydroxychloroquine Blood Levels Predicts 6-Months Disease Activity in Juvenile Lupus Nephritis

    Verena Balbi1, Clovis Silva2, Tatiana Pedrosa3, Rosa Pereira3, Lucia Campos3, Elaine Leon3, Nilo Duarte3, Valdemir Carvalho4, Sandra Pasoto3, Debora Rosario3, Leticia Brandao3, Eloisa Bonfa3 and Nadia Aikawa3, 1Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao paulo, Brazil, 2Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Sao Paulo, Brazil, 3Hospital das Clínicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Sao Paulo, Brazil, 4Fleury Group, Sao Paulo, Sao Paulo, Sao Paulo, Brazil

    Background/Purpose: Renal involvement is reported in up to 80% of juvenile systemic lupus erithematosus (JSLE) and its among the most severe manifestations in these population. Antimalarials are one of…
  • Abstract Number: 1679 • ACR Convergence 2020

    Developing a Standardized Corticosteroid Dosing Regimen in Pediatric Proliferative Lupus Nephritis

    Nathalie Chalhoub1, Kelly Rouster-Stevens2, Marisa Klein-Gitelman3, Karen Onel4, Beatrice Goilav5, Sonia Savani6, Natasha Ruth6, Tingting Qiu7, Najla Aljaberi8, Jianghong Deng9, Angela Merritt8, Benjamin Laskin10, Anna Carmela Sagcal-Gironella11, Stacy Ardoin12, Deborah Levy13, Scott Wenderfer14, Bin Huang7, Hermine I Brunner15 and LaUNCH Project Investigators16, 1The University of Cincinnati College of Medicine, Cincinnati, OH, 2Emory University/Children's Healthcare of Atlanta, Atlanta, GA, 3Division of Rheumatology, Department of Pediatrics, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, 4Pediatric Rheumatology, Hospital for Special Surgery, New York, NY, 5The Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, 6Medical University of South Carolina, Charleston, SC, 7Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 8Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 9Beijing Children’s Hospital, Beijing, China (People's Republic), 10Children's Hospital of Philadelphia, Philadelphia, 11Hackensack Meridian School of Medicine at Seton Hall University, Hackensack, NJ, 12Nationwide Children's Hospital, Columbus, OH, 13Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 14Renal Section, Baylor College of Medicine and Texas Children's Hospital, Houston, TX, 15PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 16LUpus Nephritis and Cellcept precision dosing in cHildren (LaUNCH), Cincinnati, OH

    Background/Purpose: Corticosteroids (CS) remain the mainstay of therapy for childhood-onset systemic lupus erythematosus (cSLE). However, widely accepted strategies for oral (PO) or intravenous (IV) CS…
  • Abstract Number: 1680 • ACR Convergence 2020

    Lupus Anticoagulant as a Predictor of Adverse Outcomes in Children with Venous Thromboembolism

    Elizabeth Sloan1 and Ayesha Zia1, 1UT Southwestern Medical Center, Dallas, TX

    Background/Purpose: The presence of antiphospholipid antibodies, including lupus anticoagulant (LA), is a risk factor for development of venous thromboembolism (VTE) in children. The impact of…
  • Abstract Number: 1681 • ACR Convergence 2020

    Hemophagocytic Lymphohistiocytosis (HLH) Gene Variants in Childhood-onset SLE (cSLE) with Macrophage Activation Syndrome (MAS)

    Piya Lahiry1, Sergey Naumenko2, Fangming Liao3, Daniela Dominguez4, Andrea Knight5, Deborah Levy6, Melissa Misztal7, Lawrence Ng8, Earl D. Silverman9 and Linda Hiraki10, 1Hospital for Sick Children, Toronto, ON, Canada, 2The Centre for Computational Medicine, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada, 3Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Toronto, Canada, 4Division of Rheumatology, The Hospital for Sick Children, Toronto, Canada, 5Division of Rheumatology, The Hospital for Sick Children and Department of Paediatrics, University of Toronto, Toronto, ON, Canada, 6Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 7Genetics & Genome Biology, Research Institute, The Hospital for Sick Children, Oakville, ON, Canada, 8Division of Rheumatology, Hospital for Sick Children, Toronto, Canada, 9Division of Rheumatology, The Hospital for Sick Children, Translational Medicine, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada, 10Division of Rheumatology, The Hospital for Sick Children, Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, and Department of Paediatrics, University of Toronto., Toronto, ON, Canada

    Background/Purpose: Familial Hemophagocytic lymphohistiocytosis (fHLH) is an autosomal recessive, hyper-inflammatory, life-threatening disease. Macrophage activation syndrome (MAS) is also known as secondary HLH due to the…
  • Abstract Number: 1682 • ACR Convergence 2020

    Goal-Setting Improves Transition Readiness in Adolescents with Juvenile Idiopathic Arthritis and Systemic Lupus Erythematosus

    Teresa Semalulu1, Karen Beattie1, Jeanine McColl1, Arzoo Alam2, Steffy Thomas2, Julie Herrington3, Jan Willem Gorter2, Tania Cellucci2, Stephanie Garner1, Liane Heale2, Mark Matsos1 and Michelle Batthish4, 1McMaster University, Hamilton, ON, Canada, 2McMaster University, Hamilton, Canada, 3ACPAC - University of Toronto, Hamilton, ON, Canada, 4McMaster Children's Hospital, Hamilton, ON, Canada

    Background/Purpose: The transition from pediatric to adult rheumatology care is associated with increased morbidity, mortality and loss to follow-up. This is largely due to a…
  • Abstract Number: 1683 • ACR Convergence 2020

    Ready or Not? Measuring Readiness for Transition to Adult Care in Adolescents with JIA & jSLE

    Jeanine McColl1, Teresa Semalulu1, Arzoo Alam2, Steffy Thomas2, Julie Herrington3, Jan Willem Gorter2, Tania Cellucci2, Stephanie Garner2, Liane Heale2, Mark Matsos2, Karen Beattie1 and Michelle Batthish4, 1McMaster University, Hamilton, ON, Canada, 2McMaster University, Hamilton, Canada, 3The Canadian Arthritis Society, Hamilton, Canada, 4McMaster Children's Hospital, Hamilton, ON, Canada

    Background/Purpose: Transitioning from pediatric to adult care represents a particularly vulnerable period among patients with JIA and jSLE. The shift to adult care is often…
  • Abstract Number: 1684 • ACR Convergence 2020

    Increase in Emergency Department Visits and Hospitalizations, Decrease in Outpatient Visits Following Transition to Adult Rheumatologic Care

    Paul Jensen1, Jessica Greco2, Kenneth Jackson3 and Stacy Ardoin4, 1Intermountain Healthcare, St. George, UT, 2Ohio State University/Nationwide Children's Hospital, Columbus, OH, 3Ohio State University Medical Center, Columbus, OH, 4Nationwide Children's Hospital, Columbus, OH

    Background/Purpose: Many children with rheumatic disease have active disease as adults, and health care gaps often occur in the transition from pediatric to adult care.…
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