Abstracts tagged "Vasculitis"

Abstract Number: 1075 • ACR Convergence 2022
Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis and a Vasculitic Phenotype
Benjamin Terrier¹, David Jayne², Bernhard Hellmich³, Jane Bentley⁴, Jonathan Steinfeld⁵, Steven Yancey⁶, Namhee Kwon⁷, Praveen Akuthota⁸, Paneez Khoury⁹, Lee Baylis¹⁰ and Michael Wechsler¹¹, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²University of Cambridge, Cambridge, United Kingdom, ³Klinik für Innere Medizin, Rheumatologie & Immunologie, Medius Kliniken, Universität Tübingen, Plochingen, Germany, ⁴Clinical Statistics, GlaxoSmithKline, Brentford, United Kingdom, ⁵Clinical Sciences, Respiratory, GSK (affiliation at time of research), Philadelphia, PA, ⁶Respiratory Therapeutic Area, GSK (affiliation at the time of research), Research Triangle Park, NC, ⁷Clinical Sciences, Respiratory, GSK, Brentford, United Kingdom, ⁸Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of California, San Diego, CA, ⁹Human Eosinophil Section, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ¹⁰Global Medical Affairs, GlaxoSmithKline, Durham, NC, ¹¹Department of Medicine, National Jewish Health, Denver, CO
Background/Purpose: Patients with eosinophilic granulomatosis with polyangiitis (EGPA) can have vasculitic or eosinophilic phenotypes. The MIRRA study demonstrated that patients with EGPA spent more time…
Abstract Number: 1551 • ACR Convergence 2022
The Utility of Imaging Studies (MRA and CTA) in Long-Term Monitoring for Patients with Takayasu Arteritis
Saja Almaaitah¹, Chao Zhang² and alexandra villa forte¹, ¹Cleveland Clinic Foundation, Cleveland, OH, ²Cleveland Clinic, Cleveland, OH
Background/Purpose: Takayasu Arteritis (TA) is a complex large vessel vasculitis that leads to arterial stenosis, occlusion and aneurysmal formation. Assessment of disease activity is a…
Abstract Number: 1575 • ACR Convergence 2022
Characteristics of an Internet-Based, International Cohort of Patients with a Self-Reported Diagnosis of Urticarial Vasculitis
Jason Springer¹, Tanaz Kermani², Dianne Shaw³, Kalen Larson⁴, Cristina Burroughs⁵ and Peter Merkel⁶, ¹Vanderbilt University Medical Center, Nashville, TN, ²University of California Los Angeles, Los Angeles, CA, ³Vasculitis Foundation, Chapel Hill, NC, ⁴Vasculitis Foundation, Kansas City, MO, ⁵University of South Florida, Tampa, FL, ⁶University of Pennsylvania, Philadelphia, PA
Background/Purpose: Urticarial vasculitis is a markedly rare disease, with an annual incidence of < 1 per million. Hypocomplementemia is associated with systemic features and a…
Abstract Number: 2232 • ACR Convergence 2022
Clinical Features and Long-term Outcomes of Patients with Systemic Polyarteritis Nodosa Diagnosed Since 2005: Data from 196 Patients
Julien ROHMER¹, ludovic trefond², Yann Nguyen³, Christian Agard⁴, Jean Sebastien ALLAIN⁵, Alice Berezne⁶, Pierre Charles⁷, Pascal Cohen⁸, guillaume gondran⁹, Matthieu GROH¹⁰, Tessa HUSCENOT¹¹, carole lacout¹², Estibaliz Lazaro¹³, Jonathan London¹⁴, Francois Maurier¹⁵, Arsene Mekinian¹⁶, Isabelle Nubourgh¹⁷, Rafik MESBAH¹⁸, Xavier Puéchal⁸, Laurent Perard¹⁹, Mathieu Puyade²⁰, Gregory Pugnet²¹, Viviane Queyrel²², Diane Rouzaud²³, Arthur Roux²⁴, Cecile-Audrey DUREL¹⁹, Loïc Guillevin⁸ and Benjamin Terrier⁸, ¹APHP, Suresnes, France, ²Chu clermont ferrand, Clermont-Ferrand, France, ³AP-HP.Centre Universit Paris Cit Hôpital Cochin, Montmorency, France, ⁴Internal medicine, Nantes University Hospital, Nantes, France, ⁵CHU rennes, Rennes, ⁶CH Annecy, Annecy, France, ⁷Institut Mutualiste Montsouris, Service de Médecine Interne, Paris, France, ⁸National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ⁹CHU limoges, Limoges, France, ¹⁰Foch Hospital, Suresnes, France, ¹¹APHP, Boulogne, France, ¹²CHU Angers, Angers, France, ¹³Bordeaux Hospital University, Bordeaux, France, ¹⁴Hôpital Croix-Saint-Simon, Paris, France, ¹⁵Hôpitaux privés de Metz, Metz, France, ¹⁶AP-HP, Hopital Saint Antoine, Paris, France, ¹⁷Ch St Pierre, Brussels, Belgium, ¹⁸Boulogne Ch, Boulogne, France, ¹⁹CHU Lyon, Lyon, France, ²⁰Centre Hospitalier Universitaire de Poitiers, Poitiers, France, ²¹CHU Toulouse Purpan Service de Medecine Interne, Toulouse, France, ²²CHU NIce, Nice, France, ²³Bichat, Paris, France, ²⁴MGEN, Paris, France
Background/Purpose: The etiological landscape of systemic polyarteritis nodosa (PAN) has substantially changed since the onset of hepatitis B virus (HBV) vaccination and the discovery of…
Abstract Number: 0038 • ACR Convergence 2022
STING Antagonist (IFM4490) Inhibits STING Activation and STING Dependent Signal Transduction in Patients with STING-associated Vasculopathy with Onset in Infancy (SAVI)
Sachin Gaurav¹, Bin Lin², Sophia Park³, Shankar Venkatraman⁴, David Winkler⁴ and Raphaela Goldbach-Mansky⁵, ¹Translational Autoinflammatory Diseases Section, LCIM, NIAID,, Bethesda, MD, ²NIH, Bethesda, MD, ³NIAID, Bethesda, MD, ⁴IFM Therapeutics, Boston, MA, ⁵NIH/NIAID, Potomac, MD
Background/Purpose: SAVI is a rare, autoinflammatory type I interferonopathy caused by gain-of-function mutations that cause STING activation and excessive production of type I interferons and…
Abstract Number: 0463 • ACR Convergence 2022
Utilization of a Giant Cell Arteritis Fast-Track Program Independent of Ultrasound at a Single Center
Christina Costeas, Shubhasree Banerjee, Madhura Tamhankar, Naomi Amudala, Peter Merkel and Rennie Rhee, University of Pennsylvania, Philadelphia, PA
Background/Purpose: The Giant Cell Arteritis (GCA) Fast-Track program was implemented at our center with the goal of accelerating diagnosis of this rare and rapidly progressive…
Abstract Number: 0488 • ACR Convergence 2022
Discrepancies in Temporal Artery Biopsy Positivity Rate Among White and Black Patients Suspected of Having Giant Cell Arteritis: Experience from a Tertiary Academic Medical Center
Raj Vachhani¹, Peng Li², Mehdi Tavakoli², Lanning Kline² and Angelo Gaffo², ¹University of Alabama at Birmingham, Homewood, AL, ²University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: A prior study evaluated incidence rates of biopsy proven giant cell arteritis (GCA) among black and white patients, concluding that GCA occurs at a…
Abstract Number: 1076 • ACR Convergence 2022
ANCA-Associated Vasculitis Treated with Avacopan versus a Standard Prednisone Taper: Glucocorticoid Toxicity Index Scores by Domain
Naomi Patel¹, David Jayne², Peter Merkel³, Pirow Bekker⁴, yuqing zhang⁵, Huibin Yue⁴ and John Stone⁶, ¹Massachusetts General Hospital, Sale Creek, TN, ²University of Cambridge, Cambridge, United Kingdom, ³University of Pennsylvania, Philadelphia, PA, ⁴ChemoCentryx, San Juan Capistrano, CA, ⁵Massachusetts General Hospital, Quincy, MA, ⁶Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Background/Purpose: The ADVOCATE trial (Jayne DRW et al, N Engl J Med, 2021) was a randomized, double-blind, controlled trial of avacopan vs prednisone in addition…
Abstract Number: 1552 • ACR Convergence 2022
Rare Variant Analysis of Aortopathy Genes in Takayasu’s Arteritis
Hugh Alessi¹, Yiming Luo¹, Kaitlin Quinn² and Peter Grayson³, ¹NIH, Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: A set of genes has been associated with aortopathies, which are defined as non-inflammatory diseases where the integrity of large arteries is compromised leading…
Abstract Number: 1576 • ACR Convergence 2022
Clinical Presentation and Outcome of Patients with Chronic Abdominal Periaortitis
Umar Ghaffar¹, Hannah Langenfeld², Cynthia Crowson³, Tanaz Kermani⁴, Michelle Burke¹, Boyd Viers¹, Aaron Potretzke¹, Haraldur Bjarnason¹, Kenneth J. Warrington¹ and Matthew J. Koster¹, ¹Mayo Clinic, Rochester, MN, ²Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, ³Mayo Clinic, Eyota, MN, ⁴University of California Los Angeles, West Hills, CA
Background/Purpose: To evaluate the presentation features and outcomes of patients with chronic abdominal periaortitis in a large referral cohort. Methods: Using a proprietary medical text…
Abstract Number: 0044 • ACR Convergence 2022
Follistatin-like Protein 1 Alters T Cell Receptor Signaling Dynamics in Vitro, While Expression in Vivo Correlates with Disease and Deficiency Increases Acute Inflammation in a Mouse Model of Kawasaki Disease
Mateo Amezcua¹, Janice Huang¹, Miao Chen¹, Renee Escalona², Edward Dick² and Mark Gorelik¹, ¹Columbia University, New York, NY, ²Texas Biomedical Research Institute, San Antonio, TX
Background/Purpose: Kawasaki disease (KD) is an acute, febrile illness of childhood, associated with cardiac inflammation and vasculitis of coronary arteries and sometimes other medium sized…
Abstract Number: 0464 • ACR Convergence 2022
JAKINIB in Refractory Giant Cell Arteritis. National Multicenter Study of 15 Cases and Literature Review
Diana Prieto-Peña¹, Javier Loricera², Susana Romero Yuste³, Anne Riveros-Frutos⁴, F. Javier Narváez⁵, Eugenio De Miguel⁶, Valentina Emperiale⁷, Elena Becerra-Fernández⁸, Santos Castañeda⁹, Eztizen Labrador¹⁰, Olga Maiz¹¹, Miguel Ángel González-Gay¹² and Ricardo Blanco², 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, ²Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ³Complexo Hospitalario Universitario, Pontevedra, Spain, ⁴Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, ⁵Rheumatology Department, Hospital Universitario de Bellvitge, Barcelona, Spain, ⁶Hospital Universitario La Paz, Madrid, Spain, ⁷Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain, ⁸Hospital Universitario de Torrevieja, London, United Kingdom, ⁹Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, ¹⁰Hospital San Pedro, Logroño, Spain, ¹¹Hospital Universitario de Donostia, San Sebastián, Spain, ¹²Department 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
Background/Purpose: Giant Cell Arteritis (GCA) may be refractory to standard treatment with glucocorticoids and conventional and/or biologic disease-modifying antirheumatic drugs (cDMARDs/bDMARDs). The JAK/STAT signaling pathway…
Abstract Number: 0489 • ACR Convergence 2022
Giant Cell Arteritis Phenotypes and Diagnosis of Aortitis Among 1852 Patients Before and After 2016 Across 10 French Referral Centers
Laurent Sailler¹, Kim Ly², Hubert De Boysson³, Brigitte Granel⁴, Maxima Samson⁵, Christian Agard⁶, Laurence Bouillet⁷, Magnan Julie⁸, Marc Lambert⁹, Arsene Mekinian¹⁰, Lea Tarallo¹, Eric Liozon², Gregory Pugnet¹¹, Jean Luc Davignon¹, Achille Aouba¹², Aurélie Daumas⁴, Quentin Gomes de Pinho⁴, bernard bonnotte¹³, simon parreau², Samuel Deshayes¹² and GEFA Collaborative group¹⁴, ¹CHU Toulouse, Toulouse, France, ²CHU Limoges, Limoges, France, ³Department of Internal Medicine, UR4650 PSIR, Normandie Univ, UNICAEN, CHU de Caen Normandie, Caen, France, ⁴AP-HM, Marseille, France, ⁵Dijon University Hospital, Dijon, France, ⁶Internal medicine, Nantes University Hospital, Nantes, France, ⁷Grenoble University Hospital, Grenoble, France, ⁸CHU Tours, Tours, France, ⁹CHU Lille, Lille, France, ¹⁰AP-HP, Hopital Saint Antoine, Paris, France, ¹¹CHU Toulouse Purpan Service de Medecine Interne, Toulouse, France, ¹²CHU Caen, Caen, France, ¹³Centre Hospitalo-Universitaire de Dijon, Dijon, France, ¹⁴Saint Joseph Hospital, Marseille, France
Background/Purpose: In 2016, French Internal medicine physicians collaborating in the Groupe d'Etude Français de l'Arterite à cellules géantes (GEFA) created a common database to build…
Abstract Number: 1080 • ACR Convergence 2022
Performance of Risk Prediction Models for Relapse and Severe Infection at the End of 18-month Rituximab Maintenance Therapy in ANCA-associated Vasculitides: Data from the MAINRITSAN Trials
Florence Delestre¹, Pierre Charles², Loïc Guillevin³, Raphaël Porcher⁴ and Benjamin Terrier³, ¹cochin Hospital, Paris, France, ²Institut Mutualiste Montsouris, Paris, France, ³National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ⁴Université Paris Cité, Hôtel-Dieu, Paris, France
Background/Purpose: Long-term follow-up analysis of the three MAINRITSAN trials confirmed the efficacy of rituximab (RTX) in preventing relapses in ANCA-associated vasculitis (AAV), especially RTX administration…
Abstract Number: 1555 • ACR Convergence 2022
Comparison Between Clinical Features, Acute Phase Reactants, Imaging Between Takayasu and LV-GCA Patients at Diagnosis and During Follow-up in Italian Patients in Monocentric Study
Luigi Boiardi¹, PIERLUIGI MACCHIONI², Francesco Muratore¹, GIULIA KLINOWSKI³, ELENA GALLI¹, FEDERICA MACALUSO³, MASSIMILIANO CASALI¹, GIULIA BESUTTI¹ and Carlo Salvarani⁴, ¹Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia, Italy, ²IRCCS-S.Maria Nuova, Reggio Emilia, Italy, ³IRCCS REGGIO EMILIA, Reggio Emilia, Italy, ⁴Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Università di Modena e Reggio Emilia, Reggio Emilia, Italy
Background/Purpose: Few studies compared the demographic, clinical, laboratory and imaging features, and outcomes of Takayasu arteritis (TAK) and large-vessel giant cell arteritis (LV-GCA). Aim of…

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