ACR Meeting Abstracts

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Abstracts tagged "Still’s disease"

  • Abstract Number: L09 • ACR Convergence 2024

    Efficacy and Safety of Tofacitinib in Patients with Active Systemic Juvenile Idiopathic Arthritis

    Hermine Brunner1, Caifeng Li2, Kogie Chinniah3, Yosef Uziel4, Olga Synoverska5, Sujata Sawhney6, Inmaculada Calvo Penades7, Ingrid Clara Louw8, Meiping Lu9, Pooja Nikunj Patel10, Pamela F. Weiss11, Cheng Chang12, Ivana Vranic13, Shixue Liu14, Annette Diehl15, Jose L. Rivas16, Carol A. Connell17, Gary G. Koch18, Alberto Martini19, Daniel J. Lovell1, Nicolino Ruperto20 and the PRINTO and PRCSG investigators, 1Cincinnati Children’s Hospital Medical Center, and University of Cincinnati, Cincinnati, OH, 2Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Bejing, China, 3Department of Paediatrics and Child Health, University of Kwa-Zulu, and Enhancing Care Foundation, Durban, South Africa, 4Pedriatric Rheumatology Unit, Department of Pediatrics, Meir Medical Center and Israel Tel Aviv University School of Medicine, Kfar Saba, Israel, 5Ivano-Frankivsk National Medical University, Ivano-Frankivsk, Ukraine, 6Sir Ganga Ram Hospital, New Delhi, India, 7Hospital Universitario y Politécnico La Fe, Valencia, Spain, 8Panorama Medical Centre, Cape Town, South Africa, 9Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China, 10Ann & Robert H. Lurie Children’s Hospital of Chicago; Northwestern University School of Medicine, Chicago, IL, 11Division of Rheumatology, Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, 12Pfizer Inc, New York, NY, 13Pfizer Ltd, Tadworth, United Kingdom, 14Pfizer Inc, Shanghai, China, 15Pfizer Inc, Collegeville, PA, 16Pfizer SLU, Madrid, Spain, 17Pfizer Inc, Groton, CT, 18University of North Carolina at Chapel Hill, Chapel Hill, NC, 19University of Genoa, Genoa, Italy, 20Università Milano Bicocca, Milano, and IRCCS Fondazione San Gerardo dei Tintori, Monza, Italy

    Background/Purpose: Tofacitinib (TOF) has been shown to be efficacious in the treatment of polyarticular course JIA, including systemic JIA (sJIA) without active systemic features. Here…
  • Abstract Number: L19 • ACR Convergence 2024

    Efficacy and Safety of Emapalumab in Children and Adults with Macrophage Activation Syndrome (MAS) in Still’s Disease: Results from a Phase 3 Study and a Pooled Analysis of Two Prospective Trials

    Alexei Grom1, Uwe Ullman2, Adnan Mahmood3, Josefin Blomkvist3, Brian Jamieson4 and Fabrizio De Benedetti5, 1Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, 2Sobi, Basel, Switzerland, 3Sobi, Stockholm, Sweden, 4Sobi, Inc., Morrisville, NC, 5Bambino Gesu Children's Hospital, Rome, Italy

    Background/Purpose: MAS is a life-threatening complication of Still’s disease, characterized by interferon-gamma (IFNg)-driven macrophage activation and systemic hyperinflammation. Emapalumab, an anti-IFNg antibody, binds free and…
  • Abstract Number: 1778 • ACR Convergence 2024

    A Novel Variant in IRAK2 Results in Immune Dysregulation in Systemic Juvenile Idiopathic Arthritis (sJIA)

    Mariana Correia Marques1, Brooke Boyd2, Alana Platukus3, Elizabeth Schmitz2, Hiroto Nakano2, Faiza Naz4, Anthony Cruz1, Stefania Dell'Orso4, Zuoming deng4 and Michael Ombrello5, 1National Institutes of Health, Bethesda, MD, 2Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, 31Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, 4National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, 5National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD

    Background/Purpose: Lung disease (LD) is poorly understood complication of Still’s disease with high fatality. HLA-DRB1*15 is a strong risk factor for the development of this…
  • Abstract Number: 2030 • ACR Convergence 2024

    The Development of the EULAR Score for the Definition of Disease Activity in Adult-onset Still’s Disease; The “DAVID” Project

    Piero Ruscitti1, Tanja Stamm2, valentin Ritschl2, Stéphane Mitrovic3, Charlotte Girard-Guyonvarc'h4, Helene Alexanderson5, Birgit Barten6, Carina Bostrøm7, Dorothea Fell8, Marco Gattorno9, Francis Guillemin10, Melanie Körner6, Martin Krusche11, Javier Llorca12, Pierre Quartier13, Angelo Ravelli9, Nicolas Rosine14, Maud Wieczorek15, Tanita Wilhelmer6, Miguel Angel Gonzalez-Gay16, Cem Gabay4, Bruno Fautrel17, Eugen Feist18 and Roberto Giacomelli19, and on behalf of GIRRCS–AOSD Study group, 1Division of Rheumatology, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy, L'Aquila, Italy, 2Institute of Outcomes Research, Center for Medical Data Science, Medical University of Vienna, Vienna, Austria, Vienna, Austria, 3Sorbonne Université – AP-HP, Hôpital Pitié Salpêtrière, Service de Rhumatologie, Paris, France – INSERM, Institut Pierre Louis d'Epidémiologie et de Santé Publique, UMR S1136, Paris France – CRI-IMIDATE Network, Paris, France, 4Division of Rheumatology, Department of Medicine, University Hospital of Geneva and Faculty of Medicine University of Geneva, Geneva, Switzerland, Geneva, Switzerland, 5Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, Stockholm, Sweden, Stockholm, Sweden, 6EULAR Research Partner, EULAR PARE, Zurich, Switzerland, Zurich, Switzerland, 7Department of Occupational Therapy and Physiotherapy, Karolinska University Hospital, Stockholm, Sweden, Stockholm, Sweden, 8Deutsche Rheuma-Liga Bundesverband e. V., Bonn, Germany, Bonn, Germany, 9IRCCS Giannina Gaslini, Genoa, Italy, 10Inserm, CHRU Nancy, Université de Lorraine, CIC-Clinical Epidemiology, Nancy, F-54000, France, Nancy, France, 11III Department of Medicine, Division of Rheumatology and Systemic Inflammatory Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany, Hamburg, Germany, 12Epidemiology, CIBER Epidemiología y Salud Pública (CIBERESP) and Department of Medical and Surgical Sciences, University of Cantabria, Santander, Spain, Santander, Spain, 13Université de Paris, Institut des Maladies Génétiques (IMAGINE Institute), Reference Centre for Rheumatic, AutoImmune, and Systemic Diseases in Children (RAISE), Department of Pediatric Immunology, Hematology and Rheumatology, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris 75015, France, Paris, France, 14Institut Pasteur, Université de Paris, Immunoregulation Unit, Department of Immunology, Paris, France, Paris, France, 15Swiss Centre of Expertise in Life Course Research LIVES, Lausanne and Geneva, Geneva, Switzerland, Geneva, Switzerland, 16University of Cantabria, Fundación Jimenez Díaz, Madrid, Madrid, Spain, 17INSERM, UMRS 1136, Institut Pierre Louis d'Epidémiologie et de Santé Publique, and Sorbonne University – Assistance Publique-Hôpitaux de Paris, Département de Rhumatologie, Hôpital Pitié-Salpêtrière, Paris, Ile-de-France, France, 18Department of Rheumatology, Helios Clinic Vogelsang-Gommern, cooperation partner of the Otto von Guericke University Magdeburg, Gommern, Germany, 19Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Roma, Italy

    Background/Purpose: Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown aetiology usually affecting young adults. Despite the evidence increasingly reporting the efficacy…
  • Abstract Number: 2572 • ACR Convergence 2024

    Biomarker-guided Treatment-and-stop-strategy for Recombinant IL-1Receptor Antagonist (Anakinra) in Patients with Systemic Juvenile Idiopathic Arthritis

    Remco Erkens1, Gerda Den Engelsman1, Sytze De Roock1, Dörte Hamann1, Dieneke Schonenberg-Meinema2, Merlijn van den Berg2, Mariken Gruppen3, Wineke Armbrust4, Elizabeth Legger5, Sylvia Kamphuis6, Marleen Verkaaik7, Ellen Schatorjé8, Esther Hoppenreijs8, Thomas Vogl9, Johannes Roth9, Petra Hissink Muller10, Joost Swart1, Marc Jansen1, Jorg van Loosdregt11 and Sebastiaan Vastert1, 1University Medical Center Utrecht, Utrecht, Netherlands, 2Amsterdam University Medical Center, Amsterdam, Netherlands, 3Amsterdam UMC, Amsterdam, Netherlands, 4University Medical Hospital Groningen, Groningen, Netherlands, 5University Medical Center Groningen, Groningen, Netherlands, 6Erasmus University Medical Center, Rotterdam, Netherlands, 7Erasmus Medical Center Rotterdam, Rotterdam, Netherlands, 8University Medical Center Radboud, Nijmegen, Nijmegen, Netherlands, 9University of Münster, Münster, Germany, 10Leiden University Medical Center, Leiden, Netherlands, 11University Medical Center Utrecht, La Jolla, CA

    Background/Purpose: Early initiation of treatment with the recombinant Interleukin 1 receptor antagonist (IL-1Ra), anakinra, in Still’s disease (SD), specifically systemic Juvenile Idiopathic Arthritis, patients is…
  • Abstract Number: 0297 • ACR Convergence 2024

    Characteristics of Adult-Onset Still’s Disease Skin Eruption in Individuals of Sub-Saharan Ancestry

    Benoit Suzon1, Eleonore de Fritsch1, Arthur Felix1, Fabienne Louis-sidney2, Emmanuelle Amazan1, Florence Moinet3, Aurore Abel1, Illitch Coco-Viloin1, Moustapha Dramé1, Christophe Deligny4 and Pernelle Thomas1, 1University Hospital of Martinique, Fort-de-France, Martinique, 2University Hospital Martinique, Fort De France, Martinique, 3University Hospital of Martinique, Fort de France, France, 4University Hospital of Martinique - National reference center for autoimmune disease, Internal Medicine, Fort-de-France, Martinique

    Background/Purpose: There is no data about adult-onset Still's disease (AOSD) skin eruption in individuals of sub-Saharan ancestry, from basic description to potential clinical implications.Methods: We conducted…
  • Abstract Number: 0316 • ACR Convergence 2024

    Development and Evaluation of the Adult-onset Still’s Disease Activity Index Based on Whole RNA-seq Analysis: A Novel Approach Independent of Tocilizumab Treatment

    Mayu Magi1, Hiroto Yoshida1, Hiroya Tamai2, Kotaro Matsumoto2, Keiko Yoshimoto2, Yoshihiro Matsumoto1, Tetsuhiro Soeda1 and Yuko Kaneko2, 1Chugai Pharmaceutical Co., Ltd., Kanagawa, Japan, 2Keio University School of Medicine, Tokyo, Japan

    Background/Purpose: Tocilizumab (TCZ), an anti-IL-6 receptor antibody, has been shown to be effective in the treatment of adult-onset Still's disease (AOSD), a systemic inflammatory disease.…
  • Abstract Number: 0381 • ACR Convergence 2024

    Two- and 3-Year Outcomes of the Childhood Arthritis and Rheumatology Research Alliance FROST Study of New-onset Systemic JIA Treatment

    Timothy Hahn1, George Tomlinson2, Yukiko Kimura3, Vincent Del Gaizo4, Carlos Valdes5 and Timothy Beukelman6, and for the CARRA FROST Investigators, 1Penn State Childrens Hospital, Hershey, PA, 2University of Toronto, Toronto, ON, Canada, 3Hackensack Meridian School of Medicine, New York, NY, 4Childhood Arthritis & Rheumatology Research Alliance (CARRA), Whitehouse Station, NJ, 5Genentech, Davie, FL, 6University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: The FiRst Line Options for sJIA Treatment (FROST) trial was a prospective observational study designed to compare the effectiveness of 4 Childhood Arthritis and…
  • Abstract Number: 0394 • ACR Convergence 2024

    Unveiling Major Challenges and Unmet Needs in the Therapeutic Approach to Systemic Juvenile Idiopathic Arthritis: The Patient Perspective

    Francesco Baldo1, Luciana Peixoto2, Remco Erkens3, Greta Rogani3, Claudia Bracaglia4, Dirk Foell5, Marco Gattorno6, Marija Jelusic7, Sebastiaan Vastert3, Rashmi Sinha2 and Francesca Minoia8, and on behalf of the PReS MAS/sJIA Working Party and the Systemic JIA Foundation, 1Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, 2Systemic JIA Foundation, Cincinnati, OH, 3University Medical Center Utrecht, Utrecht, Netherlands, 4IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, 5University Hospital Muenster, Muenster, Germany, 6IRCCS Giannina Gaslini, Genoa, Italy, 7University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, 8Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy

    Background/Purpose: Despite continuous improvements in the therapeutic options for children with systemic juvenile idiopathic arthritis (sJIA), access to medications significantly differs among centres and countries.…
  • Abstract Number: 0399 • ACR Convergence 2024

    Eosinophilia and Exposure to IL-1 and IL-6 Blocking Biologic Medications in a Systemic Juvenile Idiopathic Arthritis Patient Cohort

    Rachel Dickey1, Doel Dhar2, Srushti Gangireddy3, Henry H. Ong3, Wei-Qi Wei3 and Anna Patrick3, 1Lipscomb University College of Pharmacy, Athens, TN, 2Monroe Carell Jr Children's Hospital at Vanderbilt, Nashville, TN, 3Vanderbilt University Medical Center, Nashville, TN

    Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a severe disease with symptoms of fevers, rash, and arthritis. High levels of the inflammatory cytokines interlukin-1 (IL-1)…
  • Abstract Number: 0402 • ACR Convergence 2024

    Reporting of Clinical Features and Outcome Measures in Still’s Disease: A Systematic Literature Review of sJIA and AOSD Cohorts

    Mariana Correia Marques1, Erin Balay-Dustrude2, Claudia Bracaglia3, Marinka Twilt4, Karen Onel5, Simone Appenzeller6, Fatma Dedeoglu7, Esraa Eloseily8, Penelope Martinez Jimenez9, Rebecca Trachtman10, Francesca Minoia11 and Susan Shenoi12, and on behalf of the MAS/sJIA WP of PReS and CARRA sJIA Workgroup, 1National Institutes of Health, Bethesda, MD, 2University of Washington, Seattle, WA, 3IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, 4Alberta Children's Hospital, University of Calgary, Cumming School of Medicine, Calgary, Alberta, Canada, Calgary, AB, Canada, 5HSS, New York, NY, 6Department of Orthopedics, Rheumatology and Traumatology. University of Campinas, Campinas, Brazil, 7Boston Children's Hospital, Boston, MA, 8Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 9Icahn School of Medicine at Mount Sinai- Elmhurst Hospital Center, Rego Park, NY, 10Icahn School of Medicine at Mount Sinai, New York, NY, 11Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, 12Seattle Children's Hospital and Research Center, Mercer Island, WA, WA

    Background/Purpose: Multinational research is essential to globally improve the management of systemic juvenile idiopathic arthritis (sJIA), improve recognition of rare complications like lung disease, and…
  • Abstract Number: 0833 • ACR Convergence 2024

    Novel Loss of Function Variants in the Death Domain of Tumor Necrosis Factor Superfamily Receptor 1A (TNFRSF1A) in Children with Systemic Juvenile Idiopathic Arthritis (sJIA)

    Anthony Cruz1, Hiroto Nakano2, Sophia Chou3, Marissa Krantz4, Tianmin Fu5, Davide Randazzo6, Zuoming deng7, Hao Wu8 and Michael Ombrello9, and INCHARGE Consortium, 1National Institutes of Health, Bethesda, MD, 2NIAMS, NIH, Bethesda, MD, 3National Institutes of Health, Rockville, MD, 4University of Rochester, Rochester, NY, 5Ohio State University College of Medicine, Columbus, OH, 6NIAMS/NIH, Bethesda, MD, 7National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, 8Harvard Medical School, Boston, MA, 9National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition characterized by recurring fevers, arthritis and hyperinflammation, but its exact etiology is unknown.  Tumor necrosis…
  • Abstract Number: 0912 • ACR Convergence 2024

    Next-Generation Sequencing in Molecular Genetics of Adult-Onset Still’s Disease: Data from 23 Patients and Literature Review

    Belén Atienza-Mateo1, Diana Prieto-Peña1, Eztizen Labrador-Sánchez2, Natalia Palmou Fontana1, Rafael Benito Melero-Gonzalez3, Fred Anton Pages4, Carmen Alvarez Reguera5, Nerea Paz-Gandiaga6 and Ricardo Blanco-Alonso7, 1Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Cantabria, Spain, 2Hospital General de La Rioja, Logroño, Spain, 3CHU Ourense, O Carballino, Spain, 4Complejo Asistencial De Segovia, Segovia, Spain, 5Hospital Universitario Marqués de Valdecilla, Santander, Spain, 6Department of Genetics, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 7Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain

    Background/Purpose: Molecular genetic techniques are becoming increasingly essential tools for the diagnosis of monogenic systemic autoinflammatory diseases (SAIDs). However, their role in the diagnosis of…
  • Abstract Number: 1122 • ACR Convergence 2024

    Clinical Realities in Patients with Adult Onset Still’s Disease: Real-World Diagnosis and Management

    Chloe Heiting1, Karen Gambina2 and Bella Mehta3, 1Hospital for Special Surgery, New York, NY, 2Weill Cornell Medical College, New York, NY, 3Hospital for Special Surgery, Weill Cornell Medicine, New York, NY

    Background/Purpose: Adult Onset Still’s Disease (AOSD) is a rare systemic inflammatory disorder characterized by inflammatory polyarthritis, daily fever, and a transient salmon-pink maculopapular rash. Diagnosis…
  • Abstract Number: 1125 • ACR Convergence 2024

    Non-Classical Complications of Adult-Onset Still’s Disease: A Multicenter Spanish Study

    Javier Narvaez-García1, Maria Jesus García de Yebenes2, Susana Holgado3, Alejandro Olive4, Ivette Casafont-Sole3, Santos Castañeda5, Cristina Valero6, María Martín López7, Patricia Carreira8, Maribel Mora Limiñana9, Laura Nuño10, Angel Robles Marhuenda11, Pilar Bernabéu12, J Campos Esteban13, Genaro Graña14, Vera Ortiz15, Marisol Camacho16, Carmen Vargas17, Judith Sanchez-Manubens18 and Jordi Anton19, 1Hospital Universitario de Bellvitge, Barcelona, Spain, 2Instituto de Salud Musculoesquelética, Madrid, Spain, 3Hospital Universitari Germans Trias i Pujol, Badalona, Spain, 4Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain, 5Hospital Universitario de la Princesa, Madrid, Spain, 6Hospital de la Princesa, Madrid, Spain, 7General University Hospital of Ciudad Real, Ciudad de México, Spain, 8Hospital Universitario 12 de Octubre, Madrid, Madrid, Spain, 9Parc Sanitari Sant Joan de Deu, Sant Boi, Barcelona, Spain, 10Hospital Universitario La Paz, Madrid, Spain, 11Hospital Universitario La Paz, Madrid, Madrid, Spain, 12Rheumatology section, Dr. Balmis General University Hospital-ISABIAL, Alicante, Spain, Alicante, Spain, 13Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain, 14Complejo Hospitalario Universitario de A Coruña, La Coruña, Spain, 15Hospital General de Granollers, Barcelona, Spain, 16Hospital Universitario Virgen del Rocío, Sevilla, Spain, 17Hospital Universitario Virgen Macarena, Sevilla, Spain, 18Hospital Universitari Parc Taulí, Sabadell, Barcelona, Spain, 19Hospital Sant Joan de Déu, Barcelona, Spain

    Background/Purpose: Adult-onset Still's disease (AOSD) is a rare systemic disorder characterized typically by fever, arthritis, skin rash, leukocytosis, and hyperferritinemia, which are the hallmarks of…
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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.

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