Abstracts tagged "Vasculitis"

Abstract Number: 0567 • ACR Convergence 2022
Gain-of-Function Mutations in Lyn Kinase Cause a Novel Autoinflammatory Disease with Small Vessel Vasculitis and Liver Fibrosis
Adriana Almeida de Jesus¹, Guibin Chen², Dan Yang², Tomas Brdicka³, Natasha Ruth⁴, David Bennin⁵, Dita Cebecauerova⁶, Hana Malcova⁷, Helen Freeman⁸, Neil Martin⁹, Karel Svojgr⁶, Murray Passo⁴, Farzana Bhuyan¹⁰, Sara Alehashemi¹¹, Andre Rastegar¹², Kat Uss¹², Lela Kardova¹², Iris Duric³, Ebun Omoyinmi¹³, Petra Peldova⁶, Chyi-Chia Richard Lee¹⁴, David Kleiner¹⁴, Colleen Hadigan¹⁵, Stephen Hewitt¹⁴, Stefania Pittaluga¹⁴, Carmelo Carmona-Rivera¹⁶, Katherine R. Calvo¹⁵, Nirali Shah¹⁴, Miroslava Balascakova⁶, Danielle Fink¹⁷, Radana Kotalova⁶, Zuzana Parackova⁶, Lucie Peterkova⁶, Daniela Kuzilkova⁶, Vit Campr⁶, Lucie Sramkova⁶, Stephen Brooks¹⁶, Eric Meffre¹⁸, Rebecca Harper², Hyesun Kuehn¹⁵, Mariana Kaplan¹⁶, Paul Brogan¹⁹, Sergio Rosenzweig¹⁵, Zuoming Deng¹⁶, Anna Huttenlocher⁵, Susan Moir¹², Douglas Kuhns¹⁷, Manfred Boehm², Karolina Skvarova Kramarzova²⁰ and Raphaela Goldbach-Mansky²¹, ¹NIAID, NIH, Silver Spring, MD, ²National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, ³Laboratory of Leukocyte Signaling, Institute of Molecular Genetics of the Academy of Sciences of Czech Republic (ASCR), Prague, Czech Republic, ⁴Medical University of South Carolina, Charleston, SC, ⁵Departments of Pediatrics and Medical Microbiology and Immunology, University of Wisconsin-Madison, Madison, WI, ⁶Second Faculty of Medicine, Charles University/University Hospital Motol, Prague, Czech Republic, ⁷Second Faculty of Medicine, Charles University/University Hospital Motol, Praha, Czech Republic, ⁸Raigmore Hospital, Inverness, Scotland, United Kingdom, ⁹Royal Hospital for Children, Glasgow, Scotland, United Kingdom, ¹⁰National Institutes of Health, Bethesda, MD, ¹¹NIH/NIAID/TADS, Clarksville, MD, ¹²National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ¹³Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ¹⁴National Cancer Institute, National Institutes of Health, Bethesda, MD, ¹⁵Clinical Center, National Institutes of Health, Bethesda, MD, ¹⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁷National Institute of Allergy and Infectious Diseases, National Institutes of Health, Frederick, MD, ¹⁸Yale University, New Haven, CT, ¹⁹UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ²⁰Second Faculty of Medicine, Charles University/University Hospital Motol, Prague, ²¹NIH/NIAID, Potomac, MD
Background/Purpose: Lyn kinase is a member of the Src family of tyrosine kinases that regulates innate and adaptive immune responses. Lyn C-terminal tail tyrosine residue,…
Abstract Number: 1257 • ACR Convergence 2022
Assessing the Effectiveness of Tocilizumab in Giant Cell Arteritis by PET: Multicenter Study of 101 Patients of Clinical Practice
Julio Sánchez-Martín¹, Javier Loricera¹, Santos Castañeda², Clara Moriano³, F. Javier Narváez⁴, Vicente Aldasoro⁵, Olga Maiz⁶, Rafael Melero⁷, Juan Ignacio Villa Blanco⁸, Paloma Vela-Casampere⁹, Susana Romero Yuste¹⁰, José Luis Callejas¹¹, Eugenio De Miguel¹², Eva Galíndez-Agirregoikoa¹³, Francisca Sivera¹⁴, Jesus Fernandez¹⁵, Carles Galisteo¹⁶, Ivan Ferraz Amaro¹⁷, Lara Sánchez-Bilbao¹, Monica Calderon-Goercke¹⁸, Miguel Ángel González-Gay¹⁹ and Ricardo Blanco²⁰, ¹Hospital Universitario Marqués de Valdecilla, Santander, Spain, ²Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, ³Complejo Asistencial Universitario de León, León, Spain, ⁴Rheumatology Department, Hospital Universitario de Bellvitge, Barcelona, Spain, ⁵Hospital Universitario de Navarra, Pamplona, Spain, ⁶Hospital Universitario de Donostia, San Sebastián, Spain, ⁷Complexo Hospitalario Universitario de Vigo, Vigo, Spain, ⁸Sierrallana Hospital, Torrelavega, Spain, ⁹Hospital General Universitario Alicante, Alicante, Spain, ¹⁰Complexo Hospitalario Universitario, Pontevedra, Spain, ¹¹Hospital San Cecilio, Granada, Spain, ¹²Hospital Universitario La Paz, Madrid, Spain, ¹³Basurto University Hospital, Bilbao, Spain, ¹⁴Hospital Universitario de Elda, San Vicente del Raspeig, Spain, ¹⁵Hospital de La Coruña, La Coruña, Spain, ¹⁶Hospital Parc Tauli,, Sabadel, Spain, ¹⁷Division of Rheumatology. Hospital Universitario de Canarias. Spain., Santa Cruz de Tenerife, Spain, ¹⁸Hospital de Sierrallana, Torrelavega, Spain, ¹⁹Department 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, ²⁰Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Positron emission tomography (PET) is one of the tools available for the diagnosis of extracranial large-vessel vasculitis (1-5). Tocilizumab (TCZ) has shown efficacy in…
Abstract Number: 1566 • ACR Convergence 2022
Outcome of Vascular Involvement of Behçet Syndrome Treated with Infliximab: A Retrospective Cohort Study
gulen Hatemi¹, Nur Beyza Tukek², Sinem Nihal Esatoglu¹, Yesim Ozguler³, Sitki Safa Taflan², Melike Melikoglu¹, Serdal Ugurlu⁴, Izzet Fresko¹, Zekayi Kutlubay⁵, Sebahattin Yurdakul¹, Hasan Yazici⁶ and Vedat Hamuryudan⁷, ¹Istanbul University-Cerrahpaşa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²Istanbul University-Cerrahpaşa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Istanbul, Turkey, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, New York, NY, ⁴Istanbul University-Cerrahpaşa, Istanbul, Turkey, ⁵Istanbul University-Cerrahpaşa, Cerrahpasa Medical Faculty, Department of Dermatology, Istanbul, Turkey, ⁶Letter to Editor Rheumatology, Istanbul, Turkey, ⁷Istanbul University-Cerrahpaşa, Cerrahpaşa Medical School, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey
Background/Purpose: Vascular involvement is an important cause of morbidity and mortality in patients with Behçet syndrome (BS). We aimed to survey the efficacy and safety…
Abstract Number: 1676 • ACR Convergence 2022
Sarilumab in Patients with Relapsing Polymyalgia Rheumatica: A Phase 3, Multicenter, Randomized, Double Blind, Placebo Controlled Trial (SAPHYR)
Robert Spiera¹, Sebastian Unizony², Kenneth J. Warrington³, Jennifer Sloane Lazar⁴, Angeliki Giannelou⁵, Michael C Nivens⁶, Bolanle Akinlade⁵, Wanling Wong⁴, Yong Lin⁴, Frank Buttgereit⁷, Valerie Devauchelle⁸, Andrea Rubbert-Roth⁹ and Bhaskar Dasgupta¹⁰, ¹Hospital for Special Surgery, New York, NY, ²Massachusetts General Hospital, Winchester, MA, ³Mayo Clinic, ROCHESTER, MN, ⁴Sanofi, Bridgewater, NJ, ⁵Regeneron Pharmaceuticals, Inc., Tarrytown, NY, ⁶Regeneron Pharmaceuticals, Inc., Tarrytown, NY, NY, ⁷Charité Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin / DRFZ Berlin, Berlin, Germany, ⁸Université de Bretagne Occidentale, Brest, France, ⁹Division of Rheumatology, Cantonal Clinic St Gallen, St.Gallen, Switzerland, ¹⁰Anglia Ruskin University, East Anglia, United Kingdom
Background/Purpose: IL-6 is elevated in patients with active PMR and is associated with disease activity, relapse and severity. Clinical trials with IL-6 receptor (IL-6R) inhibitors…
Abstract Number: 0444 • ACR Convergence 2022
Prevalence of Frailty and Associated Factors in Patients with Vasculitis
Sebastian Sattui¹, John Stadler², Cristina Burroughs³, Kalen Larson², Christine Yeung⁴, Peter Merkel⁵ and Robert Spiera⁶, ¹University of Pittsburgh, Pittsburgh, PA, ²Vasculitis Foundation, Kansas City, MO, ³University of South Florida, Tampa, FL, ⁴University of Pennsylvania, Philadelphia, ⁵University of Pennsylvania, Philadelphia, PA, ⁶Hospital for Special Surgery, New York, NY
Background/Purpose: Frailty is a syndrome characterized by an increased vulnerability to stressors and is associated with disability and early mortality. Frailty may be accelerated in…
Abstract Number: 0473 • ACR Convergence 2022
Comprehensive Assessment of Cranial and Orbital Vasculature on MRI in Patients with Giant Cell Arteritis
Rennie Rhee, Shubhasree Banerjee, Vatsal Bhatt, Madhura Tamhankar, Naomi Amudala, Sherry Chou, Morgan Burke, Laurie Loevner, Peter Merkel and Jae Song, University of Pennsylvania, Philadelphia, PA
Background/Purpose: Vessel wall MRI depicts changes consistent with arterial wall inflammation. Unlike temporal artery biopsy, MRI visualizes several full-length cranial arteries in a single scan…
Abstract Number: 0568 • ACR Convergence 2022
The Absence of Blk Leads to Increased Inflammation in a Murine Model of Kawasaki Disease
Melissa Kleinau¹, Mohammed Massumi², Lysa Langevin², Paul Tsoukas², Harper Cheng², Suzanne Tam², Trang Duong² and Rae Yeung³, ¹University of Toronto, Toronto, ON, Canada, ²Peter Gilgan Centre for Research and Learning, Toronto, ON, Canada, ³The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Background/Purpose: Kawasaki Disease (KD) is the leading cause of acquired heart disease in children in developed countries characterized by inflammation of the coronary arteries leading…
Abstract Number: 1263 • ACR Convergence 2022
Impact of Cardiovascular Risk on the Diagnostic Accuracy of the Ultrasound Halo Score for Giant Cell Arteritis
Juan Molina¹, KATERINE FABIOLA LOPEZ GLORIA², Isabel Castrejon³, Juan Carlos Nieto González¹, Julia Martinez-Barrio¹, Ana M Anzola Alfaro⁴, Javier Rivera⁴ and José María Älvaro-Gracia¹, ¹Hospital General Universitario Gregorio Marañón, Madrid, Spain, ²Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain, Madrid, Spain, ³Hospital Universitario Gregorio Marañón, Madrid, Spain, ⁴Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
Background/Purpose: To evaluate the impact of cardiovascular risk (CVR) on the diagnostic accuracy of the ultrasonographic (US) Halo Score in patients with suspected giant cell…
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: 1849 • ACR Convergence 2022
Characteristics of Patients with Primary Retinal Vasculitis at a Tertiary Referral Center
Akash Gupta¹, Paola Rivera Morales² and Ninani Kombo², ¹Departmnt of Medicine, Yale University School of Medicine, New Haven, CT, ²Department of Ophthalmology & Visual Sciences, Yale School of Medicine, New Haven, CT
Background/Purpose: Primary retinal vasculitis (PRV) refers to inflammation in the retinal vasculature without any infectious etiology, systemic disease association, or concomitant ocular disease. PRV is…
Abstract Number: 0445 • ACR Convergence 2022
Cardiovascular Burden in Patients with ANCA-Associated and Non-ANCA Associated Vasculitis
Ana Arevalo¹, Palak Shah², Harshvardhan Zala³, Faris Haddadin⁴, Monil Majmundar⁵ and Andrea Ramirez Gomez¹, ¹University of Chicago, Chicago, IL, ²Metropolitan Hospital Center, New York Medical College, New York, NY, ³Icahn School of Medicine at Mount Sinai, New York, NY, ⁴Baylor College of Medicine, Houston, TX, ⁵University of Kansas Medical Center, Kansas City, KS
Background/Purpose: Several studies have reported that patients with ANCA-associated vasculitis (AAV) are at increased risk of cardiovascular complications from accelerated atherosclerosis with an excess cardiovascular…
Abstract Number: 0480 • ACR Convergence 2022
Treatment of Giant Cell Arteritis Patients with Ultra-short Glucocorticoids and Tocilizumab: Role of Imaging in a Prospective Study
Francesco Muratore¹, Giulia Cassone², Chiara Marvisi³, ELENA GALLI¹, Luigi Boiardi¹, GIULIA BESUTTI¹, Lucia Spaggiari¹, MASSIMILIANO CASALI¹, Pamela Mancuso¹, Annibale Versari¹, Paolo Giorgi Rossi¹ and Carlo Salvarani⁴, ¹Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy, ²Università di Modena e Reggio Emilia, Modena, Italy, ³Università di Modena e Reggio Emilia and National Institute of Arthritis & Musculoskeletal & Skin Diseases, National Institutes of Health, Bethesda, MD, ⁴Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Università di Modena e Reggio Emilia, Reggio Emilia, Italy
Background/Purpose: To evaluate the clinical and functional/morphological imaging response in a series of active large vessel (LV)-GCA patients treated with tocilizumab (TCZ) monotherapy after ultra-short-term…
Abstract Number: 0700 • ACR Convergence 2022
Accurate Identification of ANCA-Associated Vasculitis Cases from Clinical Notes Using Machine Learning
Liqing Wang¹, John Laurentiev¹, Claire Cook², Eli Miloslavsky², Hyon Choi³, Li Zhou¹ and Zachary Wallace², ¹Brigham and Women's Hospital, Boston, MA, ²Massachusetts General Hospital, Boston, MA, ³MASSACHUSETTS GENERAL HOSPITAL, Lexington, MA
Background/Purpose: ANCA-associated vasculitis (AAV) is a rare disease associated with substantial morbidity and mortality.1 To enable outcomes and comparative effectiveness studies using large, phenotypically diverse…
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: 1568 • ACR Convergence 2022
Evaluation for Allogeneic Hematopoietic Stem Cell Transplant Indications and Eligibility in a Prospective Cohort of Patients with VEXAS
Marcela Ferrada¹, Peter Grayson², Lorena Wilson³, David Beck⁴, Wendy Goodspeed⁵, Ivana Darden⁶, Emma Groarke⁶, Dennis Hickstein⁶ and Bhavisha Patel⁷, ¹National Institutes of Health, Bethesda, MD, ²National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³National Institutes of Health, Silver Spring, MD, ⁴New York University, New York, NY, ⁵National Institutes of Health (NIH), Bethesda, MD, ⁶NIH/NHLBI, Bethesda, MD, ⁷National Institutes of Health, Beltsville, MD
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly described genetic disease due to mutations in UBA1 in hematopoietic stem cells. Patients…

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