Abstracts tagged "Pediatric rheumatology"

Abstract Number: 1766 • ACR Convergence 2024
STAT2-Associated Type I Interferonopathy: A Masquerade of Infectious Susceptibility
Conor Gruber¹, Angelica Lee², Sofija Buta², Marta Martin Fernandez², Veronique Houdouin³, Jean-Laurent Casanova⁴, Alice Hadchouel⁵, Jacinta Bustamante⁵ and Dusan Bogunovic², ¹Mount Sinai Hospital, New York, NY, ²Columbia University, New York, NY, ³Robert Debré Hospital, Paris, France, ⁴Rockefeller University, New York, NY, ⁵Hôpital Necker-Enfants Malades, Paris, France
Background/Purpose: Type I IFN (IFN-I) signaling is a potent inflammatory pathway fundamental to antiviral immunity. In humans, loss of IFN-I activity underlies severe viral disease,…
Abstract Number: 1925 • ACR Convergence 2024
Use of Geocoded Social Deprivation Indices to Understand Health Disparities in Pediatric Rheumatology
William Soulsby and Emily Von Scheven, University of California, San Francisco, San Francisco, CA
Background/Purpose: Social determinants of health (SDoH) may impact health outcomes via neighborhood-level factors, such as access to medical care and neighborhood safety. Therefore, geocoded measures…
Abstract Number: 2187 • ACR Convergence 2024
Outcomes Following Tonsillectomy in Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome
Kalpana Manthiram¹, Ana Ortega-Villa², Sivia Lapidus³, Mary Bowes², Tina Romeo⁴, Kathryn Garguilo⁵, Laura Failla², Hemalatha Srinivasalu⁶, Pamela Mudd⁷, Roberta DeBiasi⁸, Amanda Ombrello⁹, Karyl Barron¹⁰, Daniel Kastner¹¹ and Kathryn Edwards⁵, ¹National Institute of Allergy and Infectious Disease, Bethesda, MD, ²National Institute of Allergy and Infectious Diseases, Bethesda, MD, ³Hackensack University Medical Center, Montclair, NJ, ⁴NIH, Bethesda, MD, ⁵Vanderbilt University School of Medicine, Nashville, TN, ⁶Children's National Hospital, Washington, DC, ⁷Children's National Hospital, Washington, ⁸Children's National Hospital and Research Institute, Washington, DC, ⁹National Institutes of Health, Rockville, MD, ¹⁰NIAID, NIH, Bethesda, MD, ¹¹National Human Genome Research Institute, Bethesda, MD
Background/Purpose: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. Tonsillectomy leads to cessation of…
Abstract Number: 2205 • ACR Convergence 2024
Presence of Nailfold Capillary Changes Correlates with More Severe Organ Involvement in Juvenile Systemic Scleroderma. Results of the Juvenile Scleroderma Inception Cohort
Ivan Foeldvari¹, Jens Klotsche², Kathryn Torok³, Ozgur Kasapcopur⁴, Amra Adrovic⁵, Brian Feldman⁶, Jordi Anton⁷, Sindhu R. Johnson⁸, Flavio Sztajnbok⁹, Maria Teresa Terreri¹⁰, Ana Sakamoto¹¹, Valda Stanevica¹², Dieneke Schonenberg-Meinema¹³, Ekaterina Alexeeva¹⁴, Maria Katsicas¹⁵, Raju Khubchandani¹⁶, Sujata Sawhney¹⁷, vanessa smith¹⁸, Eslam Al-Abadi¹⁹, Simone Appenzeller²⁰, Tadey Avcin²¹, Mikhail Kostik²², Thomas Lehman²³, Hana Malcova²⁴, edoardo marrani²⁵, Clare Pain²⁶, Anjali Patwardhan²⁷, Walter Alberto Sifuentes-Giraldo²⁸, Natalia Vasquez-Canizares²⁹, Sima Abu Alsaoud³⁰, Patricia Costa Reis³¹, Mahesh Janarthanan³², Dana Nemcova³³, Maria Jose Santos³⁴ and Nicola Helmus³⁵, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ⁵Cerrahpasa Medical School, Istanbul, Turkey, ⁶Division of Rheumatology, The Hospital for Sick Children; Departments of Pediatrics and Medicine, Faculty of Medicine; The Institute for Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada, ⁷Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁸University of Toronto, Toronto, Canada, ⁹UFRJ/UERJ, SAO PAULO, Brazil, ¹⁰UNIFESP, São Paulo, SP, Brazil, ¹¹Federal University of São Paulo (UNIFESP), São Paulo, SP, Brazil, ¹²Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹³Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁴National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia, ¹⁵Hospital Garrahan, Buenos Aires, Argentina, ¹⁶SRCC Childrens Hospital, Mumbai, India, ¹⁷Sir Ganga Ram Hospital, Sector 37 noida, Uttar Pradesh, India, ¹⁸Ghent University Hospital, Gent, Belgium, ¹⁹Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ²⁰Unicamp, Campinas, SP, Brazil, ²¹University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²²Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²³Hospital for Special Surgery, New York, NY, ²⁴Motol University Hospital, Prague, Czech Republic, ²⁵Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, ²⁶Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, ²⁷University of Missouri-Columbia, Department of Child Health, 404 N Keene Street, Columbia MO 65210, Columbia, ²⁸Hospital Universitario Ramon y Cajal, Madrid, Spain, ²⁹Children’s Hospital at Montefiore, Bronx, NY, ³⁰Caritas baby Hospital, East Jerusalem, Israel, ³¹Hospital de Santa Maria, Lisbon, Portugal, ³²SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³³MD, Prague, Czech Republic, ³⁴Hospital Garcia de Orta and Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ³⁵Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany
Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence in 3 in 1 000 000 children. Positive nailfold capillaroscopy (NF+) finding correlate with more…
Abstract Number: 2622 • ACR Convergence 2024
Quality of Care for Childhood-Onset Lupus Nephritis: Suboptimal Completion of Disease Activity Monitoring
Emily Smitherman¹, Justin Leach¹, Aimee Hersh², Melissa Mannion¹, Jinoos Yazdany³ and Jeffrey Curtis⁴, ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Utah, Salt Lake City, UT, ³UCSF, San Francisco, CA, ⁴University of Alabama at Birmingham, Hoover, AL
Background/Purpose: Poorly controlled childhood-onset lupus nephritis (cLN) can lead to end-stage kidney disease (ESKD), requiring kidney replacement therapies with substantial financial and quality of life…
Abstract Number: 0183 • ACR Convergence 2024
Sensitivity of 2019 EULAR/ACR SLE Criteria and Initial Organ Manifestations for Black and Hispanic Children with Juvenile Systemic Lupus Erythematosus (jSLE) at a Large Tertiary Care Center
Michelle Butts¹, Emily Beil¹, Danielle Guffey², Andrea Ramirez¹, Cagri Yildirim-Toruner³, Erin Peckham-Gregory¹ and Marietta De Guzman¹, ¹Baylor College of Medicine, Houston, TX, ²Baylor College of Medicine, Houston, ³Baylor College of Medicine/ Texas Children's Hospital, Houston, TX
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a systemic autoimmune disorder with diverse features, posing classification challenges. In 2019, EULAR and ACR developed new classification criteria to…
Abstract Number: 0396 • ACR Convergence 2024
Inflammatory Markers of Autoimmune Uveitis in the Eye, Tears and Blood
Maryrose Hahn¹, Madison Mangin², Kellen Winden³, Pui Lee⁴, Mindy Lo², Bharti Nihalani-Gangwani², Yasmin Massoudi⁵, Tate Valerio⁵, Amanda Colombo⁵, Jessica Scott⁶, Stephen Anesi⁶, C. Stephen Foster⁶, Peter Nigrovic⁷, sheila Angeles-Han⁸, Peter Chang⁶ and Margaret Chang², ¹Boston Children's Hospital, Georgetown, MA, ²Boston Children's Hospital, Boston, MA, ³Boston Children's Hospital, Boston, MA, ⁴Boston Children's Hospital, Newton, MA, ⁵Massachusetts Eye Research and Surgery Institution, Waltham, MA, ⁶Massachusetts Eye Research and Surgery Institution, Waltham, ⁷Boston Children's Hospital, Brookline, MA, ⁸Cincinnati Children's Hospital, Cincinnati, OH
Background/Purpose: Autoimmune uveitis is an inflammatory disorder of the eye that is associated with significant morbidity, including vision-threatening complications and chronic reliance on immunosuppressive therapies.…
Abstract Number: 0831 • ACR Convergence 2024
JDM Proteomic Signature at Disease Onset and Progression Highlights Persistent Dysregulation of Cell Death, Redox and Innate Immune Signaling
Jessica Neely¹, Jeff Dvergsten², Zilan Zheng¹, Chioma Madubata³, Hanna Kim⁴, Sara Sabbagh⁵, Sophia Matossian⁶, Christine Goudsmit⁶, Celine Berthier⁶ and Jessica Turnier⁷, and for the CARRA Registry Investigators and Translational Medicine for Juvenile Myositis Working Group, ¹UCSF, San Francisco, CA, ²Duke University Hospital, Hillsborough, NC, ³University of California, San Francisco, San Francisco, CA, ⁴National Institute of Arthritis Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁵Medical College of Wisconsin, Milwaukee, WI, ⁶University of Michigan, Ann Arbor, MI, ⁷University of Michigan, Saline, MI
Background/Purpose: Juvenile dermatomyositis (JDM) patients frequently have incomplete response to therapy. We utilized exploratory proteomics to advance understanding of dysregulated proteins and biological pathways that…
Abstract Number: 1264 • ACR Convergence 2024
Improving Mental Health Care for Youth with Juvenile Dermatomyositis Through Integration of Mental Health Screening into Pediatric Rheumatology Clinic
Emily Datyner, Lisa Buckley, Brittany Nelson and Alaina Davis, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN
Background/Purpose: The negative impact of juvenile dermatomyositis (JDM) on emotional health is well-recognized. Barriers to appropriate mental health treatment include limited availability of providers and…
Abstract Number: 1282 • ACR Convergence 2024
Investigating the Genetics of Depression and Anxiety in Children and Adolescents with and Without Systemic Lupus Erythematosus
Indrani Das¹, Nicholas Gold², Christie Burton¹, Jennifer Crosbie¹, JingJing Cao¹, Daniela Dominguez¹, Sefi Kronenberg³, Lawrence Ng², Alene Toulany¹, Hiu-Ki Rachel Tran¹, Gwyneth Zai⁴, Andrea Knight⁵ and Linda Hiraki², ¹The Hospital for Sick Children, Toronto, Canada, ²The Hospital for Sick Children, Toronto, ON, Canada, ³Holland-Bloorview Kids Rehabilitation Hospital., Toronto, Canada, ⁴Centre for Addiction and Mental Health, Toronto, Canada, ⁵Division of Rheumatology, The Hospital for Sick Children; Neurosciences and Mental Health, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada
Background/Purpose: Patients with childhood-onset systemic lupus erythematosus (cSLE) have a higher prevalence of depression and anxiety compared to healthy peers. Patients with cSLE also have…
Abstract Number: 1767 • ACR Convergence 2024
Endothelial Cell-Driven JAG/NOTCH Signaling in Localized Scleroderma Patients
Theresa Hutchins¹, Anwesha Sanyal¹, Deren Esencan¹ and Kathryn Torok², ¹University of Pittsburgh, Pittsburgh, PA, ²Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA
Background/Purpose: Localized scleroderma is a rare autoimmune disease primarily affecting the skin and underlying tissue. While its exact pathogenesis remains unclear, studies suggest links to…
Abstract Number: 1926 • ACR Convergence 2024
Race/ethnicity Is Associated with Disease Severity in Pediatric Patients with ANCA-associated Vasculitis Hospitalized in the United States
Rob Valdovinos¹, William Soulsby² and Emily von Scheven², ¹UCSF Benioff Children's Hospital, San Francisco, CA, ²University of California, San Francisco, San Francisco, CA
Background/Purpose: Race and ethnicity are societal constructs that contribute to health disparities. Small studies have found that Hispanic patients with ANCA-associated vasculitis (AAV) are more…
Abstract Number: 2188 • ACR Convergence 2024
Parent Verses Patient Perspectives of Health-Related Quality of Life Among Children with Vasculitis
Clare Peckenpaugh¹, Aimee Hersh², Sara Stern², Erin Treemarcki², Peter Merkel³ and Karen James², ¹University of Utah Department of Pediatrics, Salt Lake City, UT, ²University of Utah, Salt Lake City, UT, ³University of Pennsylvania, Philadelphia, PA
Background/Purpose: Vasculitis is a group of chronic, relapsing diseases that can lead to significant morbidity due to both disease and treatment, impacting patients’ health related…
Abstract Number: 2206 • ACR Convergence 2024
There Is No Difference in Major Organ Involvement Andantibody Pattern Between Diffuse and Limited Subtypejuvenile Onsetsystemic Scleroderma Patients
Ivan Foeldvari¹, Jens Klotsche², Kathryn Torok³, Ozgur Kasapcopur⁴, Amra Adrovic⁵, Brian Feldman⁶, Flavio Sztajnbok⁷, Jordi Anton⁸, Sindhu R. Johnson⁹, Maria Teresa Terreri¹⁰, Ana Sakamoto¹¹, Raju Khubchandani¹², Valda Stanevica¹³, Gülcan Özomay Baykal¹⁴, Dieneke Schonenberg-Meinema¹⁵, Eslam Al-Abadi¹⁶, Ekaterina Alexeeva¹⁷, Maria Katsicas¹⁸, Sujata Sawhney¹⁹, vanessa smith²⁰, Simone Appenzeller²¹, Tadey Avcin²², Mikhail Kostik²³, Thomas Lehman²⁴, Suzanne Li²⁵, Hana Malcova²⁶, edoardo marrani²⁷, Clare Pain²⁸, Anjali Patwardhan²⁹, Walter Alberto Sifuentes-Giraldo³⁰, Natalia Vasquez-Canizares³¹, Sima Abu Alsaoud³², Patricia Costa Reis³³, Hajek, Stefanie Hajek, Stefanie³⁴, Mahesh Janarthanan³⁵, Dana Nemcova³⁶, Siri Opsahl Hetlevik³⁷, Maria Jose Santos³⁸, Cristina Battagliotti³⁹, Lillemor Berntson⁴⁰, blanca e r bica⁴¹, Jürgen Brunner⁴², Liora Harel⁴³, Gerd Horneff⁴⁴, Daniela Kaiser⁴⁵, Dragana Lazarevic⁴⁶, Farzana Nuruzzaman⁴⁷, Mihaela Sparchez⁴⁸ and Nicola Helmus¹, ¹Hamburg Centre for Pediatric and Adolescence Rheumatology, Hamburg, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Istanbul University-Cerrahpasa, Cerrahpasa Medical School, istanbul, Turkey, ⁵Cerrahpasa Medical School, Istanbul, Turkey, ⁶Division of Rheumatology, The Hospital for Sick Children; Departments of Pediatrics and Medicine, Faculty of Medicine; The Institute for Health Policy, Management and Evaluation, Dalla Lana School of Public Health, University of Toronto, Toronto, ON, Canada, ⁷UFRJ/UERJ, SAO PAULO, Brazil, ⁸Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁹University of Toronto, Toronto, Canada, ¹⁰UNIFESP, São Paulo, SP, Brazil, ¹¹Federal University of São Paulo (UNIFESP), São Paulo, SP, Brazil, ¹²SRCC Childrens Hospital, Mumbai, India, ¹³Childrens Clinical University Hospital, Zemgales priekšpilseta, Riga, Latvia, ¹⁴Umraniye Training and Research Hospital, Istanbul, Turkey, ¹⁵Emma Children’s Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, Netherlands, ¹⁶Birmingham Women’s and Children’s Hospital NHSFT, Birmingham, United Kingdom, ¹⁷National Medical Research Center of Children's Health, Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russia, ¹⁸Hospital Garrahan, Buenos Aires, Argentina, ¹⁹Sir Ganga Ram Hospital, Sector 37 noida, Uttar Pradesh, India, ²⁰Ghent University Hospital, Gent, Belgium, ²¹Unicamp, Campinas, SP, Brazil, ²²University Children's Hospital University Medical Center Ljubljana, Ljubljana, Slovenia, ²³Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, ²⁴Hospital for Special Surgery, New York, NY, ²⁵Hackensack University Medical Center, Hackensack, ²⁶Motol University Hospital, Prague, Czech Republic, ²⁷Dipartimento Neurofarba, Università di Firenze, Firenze, Italy, ²⁸Alderhey Children's NHS Foundation Trust, Liverpool, United Kingdom, ²⁹University of Missouri-Columbia, Department of Child Health, 404 N Keene Street, Columbia MO 65210, Columbia, ³⁰Hospital Universitario Ramon y Cajal, Madrid, Spain, ³¹Children’s Hospital at Montefiore, Bronx, NY, ³²Caritas baby Hospital, East Jerusalem, Israel, ³³Hospital de Santa Maria, Lisbon, Portugal, ³⁴Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany, ³⁵SRI RAMACHANDRA INSTITUTE OF HIGHER EDUCATION AND RESEARCH, Chennai, Tamil Nadu, India, ³⁶MD, Prague, Czech Republic, ³⁷Oslo University Hospital, Oslo, Norway, ³⁸Hospital Garcia de Orta and Centro Académico de Medicina de Lisboa, Lisboa, Portugal, ³⁹Hospital de Niños Dr Orlando Alassia, Santa Fe, Argentina, ⁴⁰Dept. of Women’s and Children’s Health, Uppsala University, Uppsala, Sweden, ⁴¹UNIVERSIDADE FEDERAL DO RIO DE JANEIRO, Rio de Janeiro, Brazil, ⁴²Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, ⁴³Faculty of Medicine, Tel Aviv University, Tel Aviv; Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, Petach Tikva, HaMerkaz, Israel, ⁴⁴Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁴⁵Childrens Hospital Lucerne, Lucerne, Switzerland, ⁴⁶Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Faculty of Medicine, University of Niš, Nis, Serbia, ⁴⁷Stony Brook Children's Hospital, Stony Brook, NY, ⁴⁸Emergency Clinical Hospital for Children, Cluj-Napoca, Romania
Background/Purpose: In adult systemic sclerosis they are significant differences in clinical presentation of diffuse and limited subtype. In juvenile systemic sclerosis (jSSc) are the differences less…
Abstract Number: 2623 • ACR Convergence 2024
Cardiovascular Health Is Suboptimal in Most Patients with Juvenile-Onset Lupus and Dermatomyositis: Baseline Visit Findings from the Lupus Erythematous and Dermatomyositis Stress and Cardiovascular Health Cohort Study
Kaveh Ardalan¹, Angel Davalos², Hwanhee Hong², Bryce Reeve², Christoph Hornik², M. Athony Moody², Donald Lloyd-Jones³, Eveline Wu⁴, Audrey Ward¹, Rebecca Sadun⁵, Jeff Dvergsten⁶, Ann Reed², Mark Connelly⁷ and Laura Schanberg⁸, ¹Duke University School of Medicine, Durham, NC, ²Duke University School of Medicine, Durham, ³Northwestern University Feinberg School of Medicine, Chicago, ⁴University of North Carolina School of Medicine, Chapel Hill, NC, ⁵Duke University, Durham, NC, ⁶Duke University Hospital, Hillsborough, NC, ⁷Children’s Mercy Kansas City/University of Missouri-Kansas City School of Medicine, Kansas City, ⁸Duke University Medical Center, DURHAM, NC
Background/Purpose: Juvenile lupus (JSLE) and dermatomyositis (JDM) are associated with premature cardiovascular disease (CVD). The American Heart Association (AHA) cardiovascular health (CVH) score is the…

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