Abstracts tagged "Still’s disease"

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: 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: 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…
Abstract Number: 1768 • ACR Convergence 2024
A Potential Role of Longstanding IL-18 Stimulation in the Susceptibility for Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Greta Rogani¹, Remco Erkens¹, Marein Putmans¹, Rianne Scholman¹, Jorg van Loosdregt² and Sebastiaan Vastert¹, ¹University Medical Center Utrecht, Utrecht, Netherlands, ²University Medical Center Utrecht, La Jolla, CA
Background/Purpose: Macrophage Activation Syndrome (MAS) is a pathologic condition of immune hyperactivation, which occurs in 10-30% of cases of Still’s Disease (SD), the spectrum of…
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…

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