ACR Meeting Abstracts

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Abstracts tagged "Behçet’s Syndrome"

  • Abstract Number: 1248 • ACR Convergence 2023

    Thrombotic Manifestations in Pediatric Behçet Disease Patients: A Multicentre Comparative Study from EUROFEVER Registry

    Maria Vincenza Mastrolia1, Caterina Matucci-Cerinic2, Seza Ozen3, Ozgur Kasapcopur4, Carla Gaggiano5, Isabelle Kone-Paut6, Luca Cantarini5, Perrine Dusser7, Ümmüşen Kaya-Akça8, Mehmet Yildiz9, Juergen Brunner10, Giovanni Filocamo11, Romina Gallizzi12, Antonella Insalaco13, Serena Pastore14, Donato Rigante15, Judith Sanchez-Manubens16, Elena Tsitsami17, Marco Gattorno18 and Gabriele Simonini19, 1NEUROFARBA Department, University of Florence, Florence, Italy, 2DINOGMI, University of Genoa; IRCCS Istituto Giannina Gaslini, UOC Rheumatology and autoinflammatory diseases, Genova, Italy, 3Hacettepe University Medical Faculty, Ankara, Turkey, 4Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey, 5Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy, 6Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAIA, Bicêtre Hospital, APHP, University of Paris Saclay, Paris, France, 7Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France, 8Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey, 9Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Instanbul, Turkey, 10Medical University Innsbruck; Department of Pediatrics, Pediatric Rheumatology, Innsbruck, Austria, 11Pediatric Immunorheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, MIlan, Italy, 12Department of Medical of Health Sciences, Magna Graecia University, Catanzaro, Italy, 13Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, 14Institute for Maternal and Child Health, RCCS “Burlo Garofolo", Trieste, Italy, 15Department of Life Sciences and Public Health, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Italy, 16Pediatric Rheumatology, Section Pediatric Service, Parc Taulí Sabadell University Hospital, Institute for Research and Innovation I3PT, Sant Joan de Deu Hospital, Autonomous University of Barcelona, Barcelona, Spain, 17Pediatric Rheumatology Unit, 1st Department of Pediatrics, Children's Hospital "Aghia Sophia", University of Athens, Athens, Greece, 18UOC Reumatologia e Malattie Autoinfiammatorie, Genoa, Italy, 19Rheumatology Unit, ERN ReConnet Center, Meyer Children's Hospital IRCCS, Florence Italy. NeuroFARBA Department, University of Florence, Florence, Italy

    Background/Purpose: Arterial and venous thrombosis occurs in 6.6 to 38.4% of pediatric Behçet disease (BD) and cerebral sinus is one of the most involved districts.…
  • Abstract Number: 1554 • ACR Convergence 2023

    Therapeutic Approaches and Predictive Factors of Relapse in Severe Ocular Involvement in Behçet’s Disease: A Multicentre Retrospective Study

    Audrey Hermant1, Yann Nguyen2, Fleur Cohen3, Antoine Baudet4, Alban DEROUX5, Vincent Poindron6, Cécile-Audrey Durel7, Nicolas Girszyn8, Noémie Le Gouellec9, Benedicte Rouviere10, Helder Gil11, Francois Maurier12, Nicolas Noel13, Loic Raffray14, Hubert De Boysson15, Benoit Faucher16, Bertrand Godeau17, Pierre Lozach18, Claude Bachmeyer19, Alice Berezne4, Gilles Blaison20, Brice Castel21, Guillaume Gondran22, Matthieu Groh23, Stéphane Giorgiutti24, Jeremy Keraen25, Julie Magnant26, Sebastien Monnier27, Philippe Blanche28, Dominique Monnet29, Antoine Brezin29 and Benjamin Terrier30, 1Hopital Cochin, Paris, France, 2Department of Internal Medicine, Hôpital Beaujon, AP-HP, Clichy, France., Montmorency, France, 3Sorbonne Université, Paris, France, 4CH Annecy, Annecy, France, 5CHU de Grenoble, Grenoble, France, 6Strasbourg, Strasbourg, France, 7CHU Lyon, Lyon, France, 8Rouen, Rouen, France, 9CH Valenciennes, Valenciennes, France, 10CHU Brest, Brest, France, 11CHU Besancon, Besançon, France, 12CH Metz, Metz, France, 13CHU Bicetre, Le Kremlin-Bicêtre, France, 14CHU la Réunion, La Réunion, France, 15CHU Caen, Caen, France, 16CHU Marseille, Marseille, France, 17CHU Henri Mondor, Créteil, France, 18CH Le Mans, Le Mans, France, 19CHU Tenon, Paris, France, 20Hôpital Louis PASTEUR, Colmar, France, 21CH Tarbes, Tarbes, France, 22CHU Limoges, Limoges, France, 23National Referral Center for Hypereosinophilic Syndrome (CEREO), Hôpital Foch, Suresnes, France, 24CHRU Strasbourg, Strasbourg, France, 25CH Quimper, Quimper, France, 26CHU Tours, Tours, France, 27CH Versailles, Versailles, France, 28Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 29CHU Cochin, Paris, France, 30Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France

    Background/Purpose: The management of severe ocular involvement in Behçet's disease (BD) is an important issue with major functional implications due to the risk of blindness.…
  • Abstract Number: 1555 • ACR Convergence 2023

    Axial Spondyloarthritis in Patients with Gastrointestinal Involvement of Behçet Syndrome

    Musab Ozturk1, Sinem Nihal Esatoglu2, Ibrahim Hatemi3, Aykut Ferhat Celik3, Osman Aykan Kargin4, Ahmet Oz4, Erkan Yilmaz5, Didar Ucar6, Melike Melikoglu2, Hasan Yazici7, Ibrahim Adaletli4 and Gulen Hatemi2, 1Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Istanbul, Turkey, 2Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 3Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Division of Gastroenterology, Istanbul, Turkey, 4Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Radiology, Istanbul, Turkey, 5Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Tissue Typing Laboratory, Istanbul, Turkey, 6Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Ophthalmology, Istanbul, Turkey, 7Academic Hospital, Istanbul, Turkey

    Background/Purpose: Controlled studies have shown that radiographic sacroiliitis was not increased in Behçet syndrome (BS), compared to other inflammatory diseases. However, gastrointestinal involvement of Behçet…
  • Abstract Number: 127 • 2023 Pediatric Rheumatology Symposium

    What’s in a Name? A20 Protein Expression in an in Vitro Model of A20 Haploinsufficiency

    Patricia Pontes Aires1, DANIELA GERENT PETRY PIOTTO1, Andre Cunha1, Sandro Perazzio2 and Maria Teresa Terreri3, 1Universidade Federal de São Paulo, São Paulo, Brazil, 2Universidade de So Paulo; Fleury Laboratories, São Paulo, Brazil, 3UNIFESP, São Paulo, Brazil

    Background/Purpose: Tumor necrosis factor alpha (TNF-alpha) induced protein 3 gene, or TNFAIP3, encodes the A20 protein, an important regulator of the NF-κB pathway. Since its…
  • Abstract Number: 0695 • ACR Convergence 2022

    Epidemiology of Behçet’s Disease in Northern Spain

    Carmen Alvarez Reguera1, guillermo Suárez-Amorin1, Rosalía Demetrio-Pablo1, Raúl fernández-ramón1, Alba Herrero-Morant2, Lara Sánchez-Bilbao1, David Martínez-López1, José Luis Martín-Varillas3, Santos Castañeda4, Miguel Ángel González-Gay5 and Ricardo Blanco6, 1Hospital Universitario Marqués de Valdecilla, Santander, Spain, 2Hospital Universitario Marqués de Valdecilla, Ontinyent, Spain, 3Hospital de Laredo, Laredo, Cantabria, Spain, 4Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, 5Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, 6Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Background/Purpose: The prevalence of Behçet’s disease (BD) has a considerable geographical and temporal variability. Data regarding epidemiology in Spain are limited. Our study aimed to…
  • Abstract Number: 1567 • ACR Convergence 2022

    Refractory Macular Edema Due to Behçet’s Disease. Multicenter Comparative Study of Adalimumab and Certolizumab Pegol

    José Luis Martín-Varillas1, Lara Sánchez-Bilbao2, Vanesa Calvo Río3, Alf Adán4, Inés Hernanz Rodríguez4, Miguel Cordero Coma5, David Díaz Valle6, Patricia Fanlo7, Juan Ramón de Dios8, A. Garcia-Aparicio9, Sergio Rodriguez Montero10, Vega Jovani Casano11, Patricia Moya Alvarado12, eva Peña Sainz-Pardo13, José Luis Hernández2 and Ricardo Blanco14, 1Hospital de Laredo, Laredo, Cantabria, Spain, 2Hospital Universitario Marqués de Valdecilla, Santander, Spain, 3Valdecilla Hospital, Santander, Spain, 4H. Clinic, Barcelona, Spain, 5Hospital de León, León, Spain, 6Hospital Clínico San Carlos, Madrid, Spain, 7Complejo Hospitalario de Navarra, Navarra, Spain, 8Hospital de Araba, Araba, Spain, 9Hospital Universitario de Toledo, Toledo, Spain, 10Virgen de Valme University Hospital, Sevilla, Spain, 11National Health system, Alicante, Spain, 12Hospital Parc Tauli, Barcelona, Spain, 13Hospital Universitario 12 de Octubre, Madrid, Spain, 14Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Background/Purpose: Cystoid Macular Edema (CME) is the leading cause of blindness in non-infectious uveitis. Behçet's disease (BD) is one of the diseases most frequently associated…
  • Abstract Number: 1041 • ACR Convergence 2022

    Tofacitinib in Refractory Uveitis – an Observational Study

    Nikhil Gupta1 and Deepankur Mahajan2, 1Centre for Arthritis and Rheumatological diseases in Delhi, New Delhi, India, 2Mahajan Eye Centre, New Delhi, India

    Background/Purpose: To evaluate the effect of generic tofacitinib in refractory uveitis patients.Methods: It was a prospective observational study of 23 patients of refractory anterior uveitis.…
  • Abstract Number: 1701 • ACR Convergence 2022

    High TNFAIP3-Transfected Cell Activity: An In Vitro Model for Phagocyte Study of Monogenic Behçet’s Disease Pathophysiology

    Patricia Pontes Aires1, André Cunha1, DANIELA GERENT PETRY PIOTTO1, Maria Teresa Terreri2 and Sandro Perazzio1, 1Universidade Federal de São Paulo, São Paulo, Brazil, 2Universidad Federal São Paulo, São Paulo, Brazil

    Background/Purpose: A20 haploinsufficiency (A20HA), an important monogenic etiology of Behçet's disease (BD), is caused by loss-of-function mutations in TNFAIP3. Although monocytes play an important role…
  • Abstract Number: 1237 • ACR Convergence 2022

    Assessment of Common Femoral Vein Intima-Media Thickness by Ultrasound in Behçet’s Disease: Comparative Study of Patients with or Without Vascular Involvement in a National Referral Center

    Diana Prieto-Peña1, Alfonso del Peral-Fanjul2, Belén Atienza-Mateo1, Veronica Pulito-Cueto1, Miguel Ángel González-Gay3 and Ricardo Blanco4, 1Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 2Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Santander, Spain, Spain, 3Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, 4Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Background/Purpose: Clinical vascular involvement is present in up to 40% of patients with Behçet’s disease (BD). Increased venous wall thickness assessed with ultrasound (US) has…
  • Abstract Number: 1837 • ACR Convergence 2022

    Eales Disease and Vogt-Koyanagi-Harada Syndrome: Not Everything Is Anterior Uveitis in Rheumatology

    Sonia Pastor Navarro1, Olga Compán Fernández1, Marta Ibáñez Martínez1, Belén Miguel Ibáñez1, Mireia Molina Pérez2, Ángel Beltrán Mazo2, Olga Martínez González1, Carlos Montilla Morales1, Ana Isabel Turrión Nieves1, Susana Gómez Castro3 and Cristina Hidalgo Calleja1, 1Hospital Universitario de Salamanca, Salamanca, Spain, 2Hospital Universitario de Salamanca, Salamanca, Castilla y Leon, Spain, 3Hospital Universitario Salamanca, Salamanca, Spain

    Background/Purpose: Ocular inflammatory involvement for being closely related to systemic autoimmune diseases is monitored and treated in our rheumatology visits. The most frequently studied by…
  • Abstract Number: 1251 • ACR Convergence 2022

    Venous Halo Sign Detected with Superb Microvascular Imaging in Behçet Syndrome

    Yasemin Kayadibi1, Yesim Ozguler2, Melike Melikoglu3, Sinem Nihal Esatoglu3, Ahmet Ustundag1, Ugur Kimyon4, Ayse Kalyoncu Ucar1, Ibrahim Adaletli1 and gulen Hatemi3, 1Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Department of Radiology, Istanbul, Turkey, 2Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, New York, NY, 3Istanbul University-Cerrahpaşa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 4Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Department of Internal Medicine, Istanbul, Turkey

    Background/Purpose: Superb microvascular imaging (SMI) is a novel technique that provides a more sensitive assessment of small vessels compared to color Doppler US (CDUS), by…
  • Abstract Number: 2231 • ACR Convergence 2022

    Earlier and More Aggressive Treatment of Major Organ Involvement with Biologics May Prevent Relapses or Further New Organ Involvement in Behçet’s Disease

    Tugce Bozkurt1, Murat Karabacak1, Hakan Karatas1, seda kutluğ ağaçkıran2, Tulin Ergun3, Haner Direskeneli4 and Fatma Alibaz-Oner5, 1Marmara University, Istanbul, Turkey, 2Marmara University School of Medicine, Division of Rheumatology, Istanbul, Turkey, 3Marmara University, School of Medicine, Department of Dermatology, Istanbul, Turkey, 4Marmara University, Rheumatology, Istanbul, Istanbul, Turkey, 5Marmara University, Rheumatology, Istanbul, Turkey

    Background/Purpose: Behçet's disease (BD) is a chronic inflammatory disease affecting the eye, nervous system (CNS), vascular and gastrointestinal systems (GIS) as well as skin, mucosa,…
  • Abstract Number: 1264 • ACR Convergence 2022

    Does Vein Wall Thickness Have Prognostic Value in Behcet’s Disease? A Prospective Follow up Study

    Kerem Abacar1, Rabia Ergelen2, Fatma Temiz2, Yasin Yildiz2, Tulin Ergun3, Haner Direskeneli4 and Fatma Alibaz-Oner1, 1Marmara University, Rheumatology, Istanbul, Turkey, 2Marmara University, School of Medicine, Departmant of Internal Medicine, Istanbul, Turkey, 3Marmara University, School of Medicine, Department of Dermatology, Istanbul, Turkey, 4Marmara University, Rheumatology, Istanbul, Istanbul, Turkey

    Background/Purpose: We reported the first controlled ultrasound study showing increased common femoral vein(CFV) thickness in Behçet's Disease(BD),1 and showed that this is a distinctive feature…
  • Abstract Number: 1556 • ACR Convergence 2022

    Distinct Clinical and Therapeutic Features Between Parenchymal and Non-parenchymal Manifestations in Neurobehçet’s Disease

    Alba Herrero-Morant1, José Luis Martín-Varillas2, Santos Castañeda3, Olga Maiz4, Julio Sánchez-Martín5, Norberto Ortego6, Enrique Raya7, Águeda Prior-Español8, Clara Moriano9, Rafael Melero10, Genaro Graña-Gil11, Ana Urruticoechea12, Angel Ramos13, Marta Loredo-Martínez14, Eva Salgado-Pérez15, Francisca Sivera16, Ignacio Torre17, F. Javier Narváez18, Jose Luis Andreu19, Olga Martinez20, Ricardo Gómez-de la Torre21, Sabela Fernandez-Aguado22, Susana Romero Yuste23, iñigo Gonzalez-Mazon5, Carmen Alvarez Reguera5, David Martínez-López5, José Luis Hernández5, Miguel Ángel González-Gay24 and Ricardo Blanco25, 1Hospital Universitario Marqués de Valdecilla, Ontinyent, Spain, 2Hospital de Laredo, Laredo, Cantabria, Spain, 3Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, 4Hospital Universitario de Donostia, San Sebastián, Spain, 5Hospital Universitario Marqués de Valdecilla, Santander, Spain, 6Medicine Department, Universidad de Granada, Granada, Spain, 7Hospital San Cecilio, Granada, Spain, 8Hospital Universitario Germans Trias i Pujol, Barcelona, Spain, 9Complejo Asistencial Universitario de León, León, Spain, 10Complexo Hospitalario Universitario de Vigo, Vigo, Spain, 11Hospital Universitario de A Coruña, A Coruña, Spain, 12Hospital Can Misses, Ibiza, Spain, 13Complejo Hospitalario de Soria, Soria, Spain, 14Hospital Clínico Lozano Blesa, Zaragoza, Spain, 15Complejo Hospitalario Universitario de Ourense, Santiago de Composte, Spain, 16Hospital Universitario de Elda, San Vicente del Raspeig, Spain, 17Hospital de Basurto, Basurto, Spain, 18Rheumatology Department, Hospital Universitario de Bellvitge, Barcelona, Spain, 19Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain, 20Hospital Clínico Universitario de Salamanca, Zamora, Spain, 21Hospital Universitario Central de Asturias, Oviedo, Spain, 22Hospital Universitario de Cabueñes, Gijon, Spain, 23Complexo Hospitalario Universitario, Pontevedra, Spain, 24Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, 25Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Background/Purpose: Ocular and Neurobehçet's Disease (NBD) are the most severe manifestations of Behcet's disease. NBD can be classified as a) primary neural parenchymal lesions, also…
  • Abstract Number: 1557 • ACR Convergence 2022

    Microvascular Involvement in Behçet’s Disease: Study of Nailfold Capillaroscopy in Patients from a National Referral Center

    Belén Atienza-Mateo1, Alfonso del Peral-Fanjul2, Veronica Pulito-Cueto3, Diana Prieto-Peña1, Miguel Ángel González-Gay4 and Ricardo Blanco5, 1Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and in Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, 2Research Group on Genetic Epidemiology and Atherosclerosis in Systemic Diseases and Metabolic Bone Diseases of the Musculoskeletal System, IDIVAL, Santander, Spain, Spain, 3IDIVAL, Santander, Spain, 4Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, 5Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

    Background/Purpose: Ocular and mucocutaneous manifestations of Behçet's disease (BD) are globally recognized and widely studied [Arthritis Rheumatol. 2019 Dec;71(12):2081-2089, Clin Exp Rheumatol. 2020 Sep-Oct;38 Suppl…
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