Abstracts tagged "Pediatric rheumatology"

Abstract Number: 1765 • ACR Convergence 2024
CD8+ T Cells and Monocytes from Children with Macrophage Activation Syndrome Demonstrate Specific Transcriptional Changes Consistent with T Cell Activation and Expansion of Monocytes Shaped by Interferon and TLR Signaling
Susan Canny¹, Hannah DeBerg², Griffin Gessay², Ailing Lu³, Mary Eckert⁴, Andrea La Bella⁵, Hayley Waterman², Danish Nadeem², Susan Shenoi⁶, Joyce Hui-Yuen⁷, Daniel Campbell², Betsy Barnes⁸ and Jessica Hamerman², ¹University of Washington, Seattle, WA, ²Benaroya Research Institute, Seattle, WA, ³Northwell Health, Manhasset, NY, ⁴Seattle Children's Hospital, Seattle, WA, ⁵Cohen Children's Medical Center, Queens, NY, ⁶Seattle Children's Hospital and Research Center, Mercer Island, WA, WA, ⁷North Shore LIJ Health System, Great Neck, NY, ⁸Feinstein Institutes for Medical Science, Manhasset, NY
Background/Purpose: Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (sHLH), is a potentially fatal complication of rheumatic diseases. MAS is characterized by a…
Abstract Number: 1918 • ACR Convergence 2024
Exploring the Childhood Opportunity Index and Distance to Care in a Cohort of Children with Rheumatic Disease
Kristina Ciaglia¹ and Alaina Beauchamp², ¹UT Southwestern Medical Center and Scottish Rite for Children, Dallas, TX, ²UT Southwestern Medical Center, Dallas
Background/Purpose: Children from disadvantaged neighborhoods and socioeconomic backgrounds experience worse outcomes and delays in care.¹ The Childhood Opportunity Index (COI) is a multidimensional validated tool…
Abstract Number: 2186 • ACR Convergence 2024
The Exploration of Secondary Amplified Musculoskeletal Pain Syndrome (AMPS) in Pediatric Patients with Rheumatologic Disease
Sarah Ruthen¹, Mackenzie McGill¹, Nellie P Butler¹, Dori Abel¹, Melissa Argraves² and Sabrina Gmuca¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²Nationwide Children's Hospital, Columbus, OH
Background/Purpose: The American College of Rheumatology defines amplified musculoskeletal pain syndrome (AMPS) as the umbrella term for non-inflammatory musculoskeletal pain, and most commonly occurs in…
Abstract Number: 2204 • ACR Convergence 2024
Guidance for Stem Cell Therapy for Juvenile Systemic Sclerosis Patients
Ivan Foeldvari¹, Kathryn Torok², Juliana Silva³, Paulina Horvei⁴, Christopher Denton⁵, Franziksa Rosser⁶, Tamas Constantin⁷, Patricia Costa Reis⁸, Megan Curran⁹, Maurizio Cutolo¹⁰, Joerg Henes¹¹, Bernd Hinrichs¹², Kim Fligelstone¹³, Suzanne Li¹⁴, Susan Maillard³, Pia Moinzadeh¹⁵, Catherine Orteu¹⁶, Clare Pain¹⁷, Clarissa Pilkington¹⁸, Linda Schraven¹⁹ and vanessa smith²⁰, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ³Great Ormond Street Hospital, London, United Kingdom, ⁴Division of Blood and Marrow Transplantation and Cellular Therapies, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁵University College London, Northwood, United Kingdom, ⁶Division of Pulmonology Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁷Semmelweis University, Budapest, Hungary, ⁸Hospital de Santa Maria, Lisbon, Portugal, ⁹Children's Hospital Colorado, Colorado, ¹⁰Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genova, Italy, ¹¹University Hospital Tuebingen, Tuebingen, Germany, ¹²Children's pulmonology, Asklepios Klinik Nord – Heidberg, Hamburg, Germany, ¹³FESCA, London, United Kingdom, ¹⁴Hackensack University Medical Center, Hackensack, ¹⁵University Hospital Cologne, Cologne, Germany, ¹⁶Royal Free London NHS Foundation Trust, London, United Kingdom, ¹⁷Alder Hey Children’s Foundation NHS Trust, Liverpool, United Kingdom, ¹⁸Great Ormond Street Hospital, London, United Kingdom, ¹⁹Dutch representative of Fesca, London, United Kingdom, ²⁰Ghent University Hospital, Gent, Belgium
Background/Purpose: Hemopoetic stem cell transplantation(HSTC) and cellular therapies(CT) are a promising therapeutic options for juvenile systemic sclerosis(jSSc) patients. As pediatric patients have a unique potential…
Abstract Number: 2618 • ACR Convergence 2024
Population Health Management for Juvenile Idiopathic Arthritis in the Pediatric Rheumatology Care and Outcomes Improvement Network
Julia Harris¹, Catherine Bingham², Sheetal Vora³, Cagri Yildirim-Toruner⁴, Kerry Ferraro⁵, Erik Friedrichsen⁶, Danielle Bullock⁷, Jon Burnham⁸, Tzielan Lee⁹, Daniel Lovell¹⁰, Ted Wimmel¹¹, Delores Mincarelli¹¹, Mayur Patil¹², Magen Phillips¹¹ and Esi Morgan¹³, ¹Children's Mercy Kansas City, Overland Park, KS, ²Penn State Children’s Hospital, Hershey, ³Atrium Health Levine Children's Hospital, Charlotte, NC, ⁴Baylor College of Medicine/ Texas Children's Hospital, Houston, TX, ⁵JIA parent and CHOP volunteer, Lower Gwynedd, PA, ⁶Seattle Children's Hospital, Seattle, ⁷M Health Fairview Masonic Children’s Hospital, Minneapolis, ⁸Children's Hospital of Philadelphia, Philadelphia, PA, ⁹Stanford University School of Medicine, Palo Alto, CA, ¹⁰Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹¹Hive Networks, Newport, ¹²VSquare Infotech Inc, Edison, NJ, ¹³Seattle Children's Hospital, Seattle, WA
Background/Purpose: The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) is a learning health network with a vision that children with rheumatic disease will have…
Abstract Number: 0117 • ACR Convergence 2024
Validation of the 2023 American College of Rheumatology/European League Against Rheumatism Antiphospholipid Syndrome Classification Criteria in the Pediatric Population
Daniel Bar¹, Stanley Niznik², Shiri Spielman³, Rotem Semo-Oz⁴, Assaf Barg³, Sarina Levy-Mendelovich³, Gili Kenet², Nancy Agmon- Levin² and Irit Tirosh⁵, ¹Edmond and Lilly Safra Children's Hospital, Sheba Medical Center, Herzliya, HaMerkaz, Israel, ²Sheba Medical Center, Tel Aviv, Israel, ³Edmond and Lilly Safra Children's Hospital, Sheba Medical Center, Tel Aviv, Israel, ⁴Edmond and Lilly Safra Children's Hospital, Sheba Medical Center, Herzliya, Israel, ⁵Edmond and Lilly Safra Children's Hospital, Sheba Medical Center, Savyon, Israel
Background/Purpose: Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by arterial, venous or microvascular thrombosis, obstetric morbidity and/or non-thrombotic manifestations. The 2023 ACR/EULAR APS…
Abstract Number: 0395 • ACR Convergence 2024
Assessment of Enthesitis by Pediatric Rheumatology Providers
Erin Treemarcki¹, Shirley Tse², Marisa Klein-Gitelman³, Adam Mayer⁴, Hemalatha Srinivasalu⁵, Heather Walters⁶ and Melissa Oliver⁷, and the CARRA Juvenile Spondyloarthritis Workgroup and the CARRA investigators, ¹University of Utah, Salt Lake City, UT, ²SickKids, Toronto, ON, Canada, ³Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ⁴University of Pennsylvania/Children's Hospital of Philadelphia, Philadelphia, PA, ⁵Children's National Hospital, Washington, DC, ⁶Northwell Health, New Hyde Park, NY, ⁷Indiana University, Indianapolis, IN
Background/Purpose: Enthesitis is a characteristic feature of enthesitis related arthritis (ERA) but can be found in other juvenile idiopathic arthritis (JIA) subtypes. It occurs with…
Abstract Number: 0830 • ACR Convergence 2024
Activation of Autoreactive Lymphocytes in the Lung by STING Gain-of-function Mutation Expressing Radioresistant Cells
Kevin Gao¹, Kristy Chiang¹, Sharon Subramanian¹, Xihui Yin², Paul Utz³, Kerstin Nundel¹, Katherine A. Fitzgerald⁴ and Ann Marshak-Rothstein¹, ¹UMass Chan Medical School, Worcester, MA, ²Stanford University School of Medicine, Palo Alto, CA, ³Stanford University, Stanford, CA, ⁴UMass Chan Medical School, Worchester, MA
Background/Purpose: Gain-of-function mutations in STING, a critical mediator of dsDNA sensing, lead to a severe autoinflammatory syndrome known as STING-Associated Vasculopathy with onset in Infancy…
Abstract Number: 1263 • ACR Convergence 2024
Assessing Lupus Low Disease Activity State in the Pediatric Rheumatology Clinic: Baseline Data Collection from a Pediatric Lupus Collaborative
Emily Smitherman¹, Julia Harris², Aimee Hersh³, Jennifer Huggins⁴, Ashley Lytch⁵, Hanh Ho⁶, Ashley Meyer⁷, Megan Quinlan-Waters⁴, Livie Timmerman⁸ and Jon Burnham⁹, ¹University of Alabama at Birmingham, Birmingham, AL, ²Children's Mercy Kansas City, Overland Park, KS, ³University of Utah, Salt Lake City, UT, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵Children's Mercy Hospital, Kansas City, MO, ⁶Children's Mercy Kansas City, Kansas City, ⁷Children's Hospital of Philadelphia, Philadelphia, ⁸University of Alabama at Birmingham, Gardendale, AL, ⁹Children's Hospital of Philadelphia, Bryn Mawr, PA
Background/Purpose: Achievement of a lupus low disease activity state (LLDAS) has been associated with less organ damage, fewer disease flares, and improved health-related quality of…
Abstract Number: 1281 • ACR Convergence 2024
The Brazilian Registry of Juvenile Dermatomyositis: Relationship Between Ultraviolet Radiation and Laboratory Markers of Juvenile Dermatomyositis in a Tropical Country
Beatriz Carneiro¹, Adriana Elias², Claudia Saad-Magalhaes³, Erick Kill², Sylvia Farhat², Teresa Robazzi⁴, Ana Julia Moraes⁵, Sheila Oliveira⁶, Luciana Carvalho⁷, Taciana Fernandes³, Carlos Rabello⁸, Maria Teresa Terreri⁹, Silvana Sacchetti¹⁰, Simone Appenzeller¹¹, Clovis Silva¹², Claudio Len¹³, Darcisio Antonio¹⁴, Iloite Scheibel¹⁵, Melissa Fraga¹⁶, Marcia Bandeira¹⁷, Andre Cavalcante¹⁸, Ana Paula Vecchi¹⁹, Roberto Marini²⁰, Flavio Sztajnbok²¹, Nadia Aikawa²², lucia Maria Campos², Katia Kozu² and Maria Paula Ribeiro², ¹University of São Paulo, São Paulo, SP, Brazil, ²University of Sao Paulo, São Paulo, SP, ³São Paulo State University, Botucatu, ⁴University of Bahia, Salvador, ⁵Para Federal University, Belém do Pará, ⁶Rio de Janeiro Federal University, Rio de Janeiro, Brazil, ⁷University of São Paulo (Ribeirao Preto), Ribeirao Preto, ⁸Albert Sabin Children's Hospital, Fortaleza, ⁹UNIFESP, São Paulo, SP, Brazil, ¹⁰Santa Casa Medical School, São Paulo, SP, Brazil, ¹¹Unicamp, Campinas, SP, Brazil, ¹²Rheumatology Division and Pediatric Rheumatology Unit, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, São Paulo, SP, Brazil, ¹³Federal University of Sao Paulo, São Paulo, SP, ¹⁴São Paulo State University, Botucatu, SP, Brazil, ¹⁵Conceição's Hospital of Porto Alegre, Porto Alegre, ¹⁶Universidade Federal de São Paulo, São Paulo, SP, Brazil, ¹⁷Pequeno Principe Hospital, Curitiba, ¹⁸Pernambuco Federal University, Recife, ¹⁹Children's Hospital of Goiania, Goiânia, ²⁰Campinas State University, Campinas, ²¹UFRJ/UERJ, SAO PAULO, Brazil, ²²Rheumatology Division and Pediatric Rheumatology Unit, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil
Background/Purpose: Ultraviolet (UV) waves are risk factors for juvenile dermatomyositis (JDM), when compared to other types of myositis without skin involvement. Patients with JDM have…
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…

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