Abstracts tagged "Still’s disease"

Abstract Number: L06 • 2019 ACR/ARP Annual Meeting
Interferon-gamma (IFN-γ) Neutralization with Emapalumab and Time to Response in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (s-JIA) who failed High-Dose Glucocorticoids
Fabrizio De Benedetti¹, Paul Brogan ², Alexei A. Grom ³, Pierre Quartier ⁴, Rayfel Schneider ⁵, Jordi Antón ⁶, Claudia Bracaglia ⁷, Manuela Pardeo ⁸, Giulia Marucci ⁸, Emanuela Sacco ⁹, Despina Eleftheriou ¹⁰, Charalampia Papadopoulou ¹¹, Philippe Jacqmin ¹², maria ballabio ¹³ and Cristina de Min ¹³, ¹Bambino Gesù Children's Hospital, Rome, Italy, ²Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Necker Hospital, Paris, France, ⁵Hospital for Sick Children, Toronto, ON, Canada, ⁶Hospital Sant Joan de Déu, Barcelona, Spain, ⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁸Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, ¹⁰UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section,, london, United Kingdom, ¹¹UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section, london, United Kingdom, ¹²MnS, Dinant, Belgium, ¹³Sobi AG, basel, Switzerland
Background/Purpose: MAS is a severe complication of rheumatic diseases, most frequently sJIA and adult-onset Still’s disease. It is characterized by fever, hepatosplenomegaly, liver dysfunction, cytopenias,…
Abstract Number: 1247 • 2019 ACR/ARP Annual Meeting
Adult-Onset Still’s Disease and Spondyloarthritis: Overlapping Syndrome or Incidental Association? A Series of 5 Cases
Aly Kamissoko ¹, Nolan Hassold ², Alexis Mathian ³, Edouard Pertuiset ⁴, Gaetane Nocturne ², Bruno Fautrel ⁵ and Stéphane Mitrovic⁶, ¹AP-HP, Department of Rheumatology, Pitié-Salpêtrière University Hospital, Paris, France, Paris, France, ²Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France., Paris, France, ³AP-HP, Service de Médecine Interne 2, Hôpital Universitaire Pitié-Salpêtrière, Paris, France, Paris, France, ⁴Rheumatology Department, Pontoise Hospital, Pontoise, France, Pontoise, France, ⁵Pitié-Salpêtrière Hospital, Department of Rheumatology, AP-HP, Sorbonne University, UPMC university, Paris, Ile-de-France, France, ⁶AP-HP, Department of Rheumatology, Pitié-Salpêtrière University Hospital, Sorbonne Université, GRC 08, Pierre Louis Institute of Epidemiology and Public Health, Paris, France - Centre d’Etude et de Référence sur les Maladies AutoInflammatoires et les Amyloses (CEREMAIA), FAI2R network, Paris, France - Institut Mutualiste Montsouris, Paris, France, Paris, France
Background/Purpose: Some forms of spondyloarthritis (SpA) such as SAPHO syndrome are classified as auto-inflammatory disorders. Association with adult onset Still’s disease (AoSD) has never been…
Abstract Number: 1324 • 2018 ACR/ARHP Annual Meeting
Anakinra Treatment in Refractory Cases of Adult-Onset Still Disease: Case Series
Serdal Ugurlu¹, Berna Yurttas¹, Gul Guzelant¹, Bilgesu Ergezen² and Huri Ozdogan³, ¹Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, ³Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey
Background/Purpose: Despite methotrexate and steroid treatment, in cases of Adult-onset Still’s disease (AOSD) it is usually difficult to maintain clinic stability. In refractory cases, Anakinra…
Abstract Number: 1325 • 2018 ACR/ARHP Annual Meeting
Canakinumab Treatment in Adult-Onset Still’s Disease: Case Series
Serdal Ugurlu¹, Gul Guzelant¹, Berna Yurttas¹, Bilgesu Ergezen¹, Ediz Dalkiliç², Timucin Kasifoglu³, Burcu Yagız⁴ and Huri Ozdogan¹, ¹Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²Department of Internal Medicine, Division of Rheumatology, Uludağ University Faculty of Medicine, Bursa, Turkey, ³Department of Internal Medicine, Division of Rheumatology, Eskisehir Osmangazi University Faculty of Medicine, Eskişehir, Turkey, ⁴Uludag University, Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Bursa, Turkey
Background/Purpose: In Adult-onset Still’s disease (AOSD), cases refractory to typical DMARDs, Canakinumab (an anti-IL-1ß monoclonal antibody) has been reported to be effective in a limited…
Abstract Number: 1414 • 2018 ACR/ARHP Annual Meeting
Pharmacovigilance of Biologics for Systemic Juvenile Idiopathic Arthritis Patients By the German Biologics Registry
Gerd Horneff¹, Gerd Ganser², Toni Hospach³, Ivan Foeldvari⁴, Michael Borte⁵, Frank Weller-Heinemann⁶, Kirsten Minden⁷ and Ariane Klein⁸, ¹Asklepios Kinderklinik St. Augustin GmbH, Sankt Augustin, Germany, ²Klinik für Kinder-und Jugendrheumatologie, Nordwestdeutsches Rheumazentrum, Sendenhorst, Germany, ³Pediatrics, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany, ⁴Hamburg Centre for Pediatric Rheumatology, Hamburg, Germany, ⁵St Georg Hospital, Leipzig, Germany, ⁶PRINTO, Genoa, Italy, ⁷Charité–Universitätsmedizin Berlin, Berlin, Germany, ⁸Center of Pediatrics and Neonatology, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany
Background/Purpose: Long-term surveillance of biologic drugs is particularly important in pediatric patients(pts). Since 2001, the German Biologics JIA Registry (BIKER) is allowing to follow up…
Abstract Number: 364 • 2017 ACR/ARHP Annual Meeting
Canakinumab First or Second Choice in Systemic Juvenile Idiopathic Arthritis – Experience from Clinical Practice
Gerd Horneff¹, Ivan Foeldvari², Klaus Tenbrock³, K Minden⁴ and Jasmin B. Kuemmerle-Deschner⁵, ¹Asklepios Clinic, Sankt Augustin, Germany, ²Kinder- und Jugenrheumatologie, Hamburger Zentrum Kinder-und Jugendrheumatologie, Hamburg, Germany, ³University Aachen, Aachen, Germany, ⁴Charité – University of Medicine Berlin, Berlin, Germany, ⁵Pediatrics, University Hospital Tübingen, Tübingen, Germany
Background/Purpose: Canakinumab (CAN) has demonstrated its efficacy and safety in systemic juvenile idiopathic arthritis in clinical trials. We report on the experience with CAN in…
Abstract Number: 1885 • 2017 ACR/ARHP Annual Meeting
Epidemiology of Hospitalized Adult Onset Still’s Disease in United States
Bella Y. Mehta¹, William Briggs² and Petros Efthimiou³, ¹Rheumatology, Hospital for Special Surgery/Weill Cornell Medicine/Mailman School of Public Health, New York, NY, ²New York Presbyterian/ Brooklyn Methodist Hospital, New York, NY, ³Medicine/Rheumatology, New York University School of Medicine/NYU Langone Health, New York, NY
Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Still’s Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report…
Abstract Number: 2109 • 2017 ACR/ARHP Annual Meeting
Hyperferritinemic Syndrome in a General Hospital
Ignacio Javier Gandino¹, Florencia Pierini², Jose Maximiliano Martinez P³, Santiago Ruta², Marina Scolnik² and Enrique R Soriano⁴, ¹Reumatologia, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, ²Rheumatology Unit, Internal Medicine Service, Hospital Italiano de Buenos Aires, CABA, Argentina, ³Rheumatology, Internal Medicine Service, Rheumatology Unit, Internal Medicine Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano de Buenos Aires, and Fundacion PM Catoggio, Buenos Aires, Argentina, ⁴Argentina, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina
Background/Purpose: Hyperferritinemia is associated with inflammatory conditions, such as rheumatologic diseases. Objectives: To determine which diseases are associated with hyperferritinemia in a tertiary hospital; to…
Abstract Number: 70 • 2017 Pediatric Rheumatology Symposium
Impact of Systemic Juvenile Idiopathic Arthritis/Still’s Disease on Adolescents as Evidenced Through Social Media Posting
Renee F Modica¹, Kathleen G Lomax², Pamela Batzel³, Armelle Cassanas³ and Melissa E Elder¹, ¹Department of Pediatrics, University of Florida, Gainesville, FL, ²Novartis Pharmaceuticals Corporation, East Hanover, NJ, ³Treato, Princeton, NJ
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA)/Still’s disease is a rare form of chronic arthritis in pediatrics. The patient perspective of living with the disease is…
Abstract Number: 8 • 2017 Pediatric Rheumatology Symposium
Examination of Reported Risk Loci from Candidate Gene Studies of Systemic Juvenile Idiopathic Arthritis Identifies Link between IL1RN Variation and both Disease Susceptibility and Response to Interleukin-1 Directed Therapy
Victoria Arthur¹, Emily Shuldiner¹, Anne Hinks², The International Childhood Arthritis Genetics (INCHARGE) Consortium³, Patricia Woo⁴, Wendy Thomson², Elaine F. Remmers⁵ and Michael J. Ombrello¹, ¹Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD, ²Arthritis Research UK Centre for Genetics and Genomics,The University of Manchester, Manchester, United Kingdom, ³INCHARGE Consortium, Bethesda, MD, ⁴Paediatric Rheumatology Unit, University College London, London, United Kingdom, ⁵Inflammatory Disease Section, NHGRI, NIH, Bethesda, MD
Background/Purpose: Systemic JIA (sJIA) is a childhood inflammatory disease whose pathophysiology is poorly understood. sJIA is phenotypically heterogeneous with variable manifestations and responses to treatment.…
Abstract Number: 2442 • 2015 ACR/ARHP Annual Meeting
The Family Journey to Diagnosis with Systemic Juvenile Idiopathic Arthritis As Evidenced through Changing Social Media Presence
Renee F Modica¹, Kathleen G Lomax², Pamela Batzel³, Leah Shapardanis³, Kimberly A Compton³ and Melissa E Elder⁴, ¹UF Health Shands Children’s Hospital, Gainesville, FL, ²Medical Affairs, Immunology and Dermatology, Novartis Pharmaceuticals Corporation, East Hanover, NJ, ³TREATO, Princeton, NJ, ⁴Pediatrics, Univ of Florida, Gainesville, FL
Background/Purpose: Patients with systemic juvenile idiopathic arthritis (SJIA) frequently encounter delays from symptom onset to SJIA diagnosis (dx), partly due to the broad differential of…
Abstract Number: 1405 • 2015 ACR/ARHP Annual Meeting
Tocilizumab Serum Levels and Antidrug Antibodies and Its Relationship with Disease Activity in Rheumatic Diseases
Samantha Rodriguez-Muguruza¹, Melania Martínez-Morillo¹, Juana Sanint², Bibiana Quirant Sr.³, Aina Teniente Sr.⁴, Agueda Prior⁵, Anne Riveros-Frutos⁶, Susana Holgado⁷, Maria Lourdes Mateo⁷, Alejandro Olivé⁸, Jeronima Cañellas⁵ and Xavier Tena⁷, ¹Rheumatology, Hospital Universitario Germans Trias i Pujol, Barcelona, Spain, ²Rheumatology, Hospital Universitario Germans Trias i Pujol, Badalona, Spain, ³Immunology, Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, ⁴immunology, Hospital Universitari Germans Trias i Pujol, barcelona, Spain, ⁵Rheumatology, Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, ⁶Microbiology, Hospital Universitario Germans Trias i Pujol, Badalona, Spain, ⁷Rheumatology, Hospital Germans Trias i Pujol, Badalona, Spain, ⁸Hospital Germans Trias i Pujol, Badalona, Spain
Background/Purpose: Tocilizumab (TCZ) is a humanized anti-IL-6 receptor-blocking monoclonal antibody used for the treatment of rheumatoid arthritis (RA), idiopathic juvenile arthritis (IJA) and off-label in…
Abstract Number: 1401 • 2015 ACR/ARHP Annual Meeting
Is There Any Difference Between Autoimmune or Hemato-Oncology Etiology of Macrophage Activation Syndrome?
César Antonio Egües Dubuc, Miren Uriarte Ecenarro, Nerea Errazquin Aguirre, Olga Maiz Alonso, Iñaki Hernando Rubio and Joaquin Maria Belzunegui Otano, Rheumatology, Donostia University Hospital, Donostia, Spain
Background/Purpose: Macrophage activation syndrome (MAS) is a group of diseases, especially autoimmune (AI) and hemato-oncology (HO). So it will be interesting to find any clinical…
Abstract Number: 265 • 2015 ACR/ARHP Annual Meeting
Tocilizumab Compared with Anakinra in Refractory Adult-Onset Still’s Disease. Multicenter Study of 75 Patients
Leyre Riancho-Zarrabeitia¹, Vanesa Calvo-Río¹, Ricardo Blanco¹, Jose L. Hernández², Francisco Ortiz-Sanjuán¹, Alejandro Olivé³, Anne Riveros Frutos⁴, Santos Castañeda⁵, Javier Narváez⁶, Maria Luisa Velloso Feijoo⁷, M. Victoria Hernández⁸, Alberto Sifuentes Giraldo⁹, Olga Maiz-Alonso¹⁰, Esteban Rubio Romero¹¹, Cristina Mata-Arnaiz¹², Adela Gallego Flores¹³, Jordi del Blanco¹⁴, Catalina Gómez-Arango¹⁵, Sara Manrique-Arija¹⁶, Maria Carmen Ordoñez¹⁷, Inmaculada Ros Vilamajo¹⁸, Miguel Angel Caracuel-Ruiz¹⁹, Mercedes Freire²⁰, Jose Llobet²¹, Carlos Marras²², Concepción Moll Tuduri²³, Chamaida Plasencia²⁴, Rosa Roselló²⁵, Ana Urruticoechea²⁶, Inmaculada Jiménez-Moleón²⁷, José Antonio Bernal²⁸, Vera Ortiz-Santamaría²⁹, Juan Ramón De Dios³⁰, Mireia Moreno³¹, Jordi Fiter³², Marina de los Riscos³³, Patricia E. Carreira³³, María José Rodríguez Valls Sr.³⁴, Carmen Gonzalez-Vela³⁵, Javier Loricera¹, Natalia Palmou¹ and Miguel Angel Gonzalez-Gay¹, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Santander, Spain, ²Internal Medicine, Hospital Universitario Marqués de Valdecilla. IDIVAL, Santander, Spain, ³Rheumatology, Hospital U German Trias i Pujol, Badalona, Spain, ⁴Rheumatology, Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, ⁵Rheumatology, H.U. La Princesa, Madrid, Spain, ⁶Rheumatology, Hospital Universitario de Bellvitge, Barcelona, Spain, ⁷H Valme, Sevilla, Spain, ⁸Arthritis Unit, Rheumatology, Hospital Clinic, Barcelona, Spain, ⁹Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹⁰Rheumatology, HU Donostia, San Sebastián, Spain, ¹¹Rheumatology Department,, Hospital Universitario Virgen del Rocío, Sevilla, Spain, ¹²Rheumatology, Hospital Laredo, Santander, Spain, ¹³Rheumatology, Hospital de Mérida, Mérida, Spain, ¹⁴Rheumatology, H Sant Jaume, Calella, Spain, ¹⁵Rheumatology, Hospital Basurto,, Santander, Spain, ¹⁶Rheumatology, Hospital Universitario Carlos Haya, Malaga, Spain, ¹⁷HRU Carlos Haya, Malaga, Spain, ¹⁸Hospital Son Llàtzer, Palma de Mallorca, Spain, ¹⁹Plaza Cruz Roja, 1, Hospital Reina Sofia, Córdoba, Spain, ²⁰Hospital Universitario Juan Canalejo, La Coruña, Spain, ²¹Hospital San Pau, Barcelona, Spain, ²²Hospital Universitario Virgen de la Arrixaca, Murcia, Spain, ²³H Mateu Orfila, Mahón, Spain, ²⁴Rheumatology Unit, La Paz University Hospital-Rheumatology, Madrid, Spain, ²⁵H San Jorge, Huesca, Spain, ²⁶Hospital Can Misses, Ibiza, Spain, ²⁷Rheumatology, Hospital San Cecilio, Granada, Spain, ²⁸Sección de Reumatología, Hospital General Universitario de Alicante, Alicante, Spain, ²⁹Rheumatology, Hospital General. Granollers., Granollers, Spain, ³⁰Rheumatology, HU Álava, Vitoria, Spain, ³¹Hospital de Sabadell - Institut Universitari Parc Taulí, Sabadell, Spain, ³²Rheumatology, HU Son Espases. Palma de Mallorca., Palma de Mallorca, Spain, ³³Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain, ³⁴Rheumatology, Rheumatolgy Unit. Hospital Jerez, Jerez, Spain, ³⁵Pathology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Santander, Spain
Background/Purpose: Interleukin (IL)-1 and IL-6 are pivotal cytokines in the pathogenesis of adult-onset Still’s disease (AOSD). Our aim was to compare the efficacy and safety of…
Abstract Number: 1240 • 2014 ACR/ARHP Annual Meeting
Efficacy of Anakinra in Refractory Adult-Onset Still´s Disease: Multicenter Study of 41 Patients
Leyre Riancho-Zarrabeitia¹, Ricardo Blanco¹, Alejandro Olivé², Anne Riveros-Frutos², Santos Castañeda³, Maria Luisa Velloso Feijoo⁴, Javier Narváez⁵, Inmaculada Jiménez-Moleón⁶, Olga Maiz-Alonso⁷, Maria Carmen Ordóñez⁸, José Antonio Bernal⁹, M. Victoria Hernández¹⁰, Alberto Sifuentes Giraldo¹¹, Catalina Gomez Arango¹², Eva Galindez-Agirregoikoa¹², Vera Ortiz-Santamaría¹³, Jordi del Blanco¹⁴, Juan Ramón De Dios¹⁵, Mireia Moreno¹⁶, Jordi Fiter¹⁷, Marina de los Riscos¹⁸, Patricia Carreira¹⁸, María José Rodríguez Valls Sr.¹⁹, Francisco Ortiz-Sanjuán¹, Trinitario Pina Murcia²⁰, Montserrat Santos-Gómez¹ and Miguel A González-Gay¹, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL. Santander. Spain, Santander, Spain, ²Rheumatology, Hospital Universitario Germans Trias i Pujol, Badalona, Spain, ³Rheumatology, Hospital Universitario de La Princesa, IISP, Madrid, Spain, ⁴H Valme, Sevilla, Spain, ⁵Rheumatology, Hospital Universitario de Bellvitge. Barcelona. Spain, Barcelona, Spain, ⁶Rheumatology, Hospital San Cecilio, Granada, Spain, ⁷Rheumatology, HU Donostia, San Sebastián, Spain, ⁸Rheumatology, Hospital Regional Universitario Carlos Haya., Málaga, Spain, ⁹Rheumatology, HGU. Alicante., Alicante, Spain, ¹⁰Arthritis Unit, Department of Rheumatology, Hospital Clinic, Barcelona, Barcelona, Spain, ¹¹Rheumatology, HU Ramón y Cajal, Madrid, Spain, ¹²Rheumatology, Hospital Universitario Basurto, Bilbao, Spain, ¹³Rheumatology, Hospital General. Granollers., Granollers, Spain, ¹⁴Rheumatology, H Sant Jaume, Calella, Spain, ¹⁵Rheumatology, HU Álava, Vitoria, Spain, ¹⁶Rheumatology, University Hospital Parc Taulí, Sabadell, Spain, ¹⁷Rheumatology, HU Son Espases. Palma de Mallorca., Palma de Mallorca, Spain, ¹⁸Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain, ¹⁹Rheumatology, Rheumatolgy Unit. Hospital Jerez, Jerez, Spain, ²⁰Epidemiology, Genetics and Atherosclerosis Research Group on Systemic Inflammatory Diseases, Rheumatology Division, IDIVAL, Santander, Spain
Background/Purpose: Adult-onset Still's disease (AOSD) is frequently refractory to standard therapy. Anakinra (ANK), an interleukin-1 (IL-1) receptor antagonist, has demonstrated efficacy in single cases or…