Abstracts tagged "Vasculitis"

Abstract Number: 2504 • ACR Convergence 2024
Polyarteritis Nodosa Associated with VEXAS Syndrome and Chronic Myelomonocytic Leukemia Compared with Primary Forms: A Case-Control Study
Paul Cioni¹, Yann Nguyen², Julien ROHMER³, Henry Dupuy⁴, Xavier Puéchal⁵, Estibaliz Lazaro⁶, Grégory Pugnet⁷, Cécile-Audrey Durel⁸, Edoardo Conticini⁹, François Lifermann¹⁰, Philip Bielefeld¹¹, pierre duffau⁴, Hartmut Mahrhofer¹², Alexis Régent⁵, Loïc Guillevin¹³, ludovic trefond¹⁴, Christian AGARD¹⁵, Alice Bérezné¹⁶, Valentin Lacombe¹⁷, Perrine SMETS¹⁸, Sophie Georgin-lavialle¹⁹, Arsene Mekinian²⁰ and Benjamin Terrier²¹, and the French Vasculitis Study Group and the French Vexas Group, ¹Cochin University Hospital, Paris, France, ²Université Paris Saclay, Clichy, Ile-de-France, France, ³APHP, Suresnes, France, ⁴Bordeaux University Hospital, Bordeaux, France, ⁵National Referral Center For Rare Systemic Autoimmune Diseases, Paris, France, ⁶Bordeaux University Hospital, Pessac, France, ⁷Toulouse Rangueil University Hospital, Toulouse, France, ⁸Edouard Herriot University Hospital, Lyon, France, ⁹University of Siena, Siena, Siena, Italy, ¹⁰Dax Hospital, Dax, France, Dax, France, ¹¹Dijon University Hospital, Dijon, France, ¹²Kirchheim Clinic, Kirchheim unter Teck, Germany, ¹³National Referral Center For Rare Systemic Autoimmune Diseases, Paris, Ile-de-France, France, ¹⁴Clermont Ferrand University Hospital, Clermont Ferrand, France, ¹⁵Nantes University Hospital, Nantes, France, ¹⁶Annecy Genevois Hospital, Annecy, France, ¹⁷Angers University Hospital, Angers, France, ¹⁸Clermont Ferrand University Hospital - National reference center for autoimmune disease, Internal Medicine, Clermont-Ferrand, France, ¹⁹Sorbonne Université, Department of internal medicine, Tenon Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ²⁰Saint Antoine University Hospital, Paris, France, ²¹Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France
Background/Purpose: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting medium-sized vessels. It is a life-threatening disease without appropriate treatment. In 2024, approximately one quarter…
Abstract Number: 2628 • ACR Convergence 2024
Clinical Features, Treatments, and Outcomes of a Large International Cohort of Childhood-onset Takayasu Arteritis: A PedVas Study
James Bistolarides¹, Vidya Sivaraman², Else Bosman³, Linda Wagner-Weiner⁴, Kimberly Morishita⁵, James Nocton¹, Melodee Liegl¹, Amy Pan¹ and David Cabral⁶, and PedVas Investigators Network, ¹Medical College of Wisconsin, Milwaukee, WI, ²Nationwide Children's Hospital/ The Ohio State University, Columbus, OH, ³UBC, Vancouver, BC, Canada, ⁴University of Chicago, Chicago, IL, ⁵University of British Columbia - Vancouver, Vancouver, BC, Canada, ⁶BC Children's Hospital and University of British Columbia, Vancouver, BC, Canada
Background/Purpose: Takayasu arteritis (TAK) is a rare systemic vasculitis. It occurs most commonly in patients aged < 40 years (y), and 32% of patients present…
Abstract Number: 0737 • ACR Convergence 2024
Rurality and Delayed Diagnosis of Giant Cell Arteritis – a Single Center Experience
Ruoning (Lemon) Ni¹, Bharat Kumar² and Aleksander Lenert³, ¹Cleveland Clinic, Cleveland, OH, ²University of Iowa Hospitals and Clinics, Iowa City, IA, ³University of Iowa, Iowa City, IA
Background/Purpose: Giant Cell Arteritis (GCA) poses diagnostic challenges, especially in rural areas, where limited access to healthcare can delay diagnosis and worsen outcomes, a concern…
Abstract Number: 0766 • ACR Convergence 2024
Neutrophil Transcriptomics in VEXAS Syndrome
Chloe Palmer¹, Gustaf Wigerblad¹, Tom Hill², Bhavisha Patel³, Emma Groarke⁴, Neal Young⁴, Stefania Dell'Orso⁵ and Peter Grayson⁶, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, MD, ³National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), Beltsville, MD, ⁴National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD, ⁵National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, ⁶National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD
Background/Purpose: Vacuoles, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is caused by somatic mutations of the ubiquitin-like modifier activating enzyme 1 (UBA1) gene and…
Abstract Number: 1597 • ACR Convergence 2024
Baseline Glucocorticoid-Related Toxicity in Newly-Diagnosed and Relapsing ANCA-Associated Vasculitis
Naomi Patel¹, Sarah Bray², Darcy Trimpe³, David Jayne⁴, Peter Merkel⁵ and John Stone⁶, ¹Massachusetts General Hospital, Boston, MA, ²Amgen Ltd, Cambridge, United Kingdom, ³Amgen Inc., Thousand Oaks, CA, ⁴University of Cambridge, Cambridge, United Kingdom, ⁵University of Pennsylvania, Philadelphia, PA, ⁶Massachusetts General Hospital , Harvard Medical School, Concord, MA
Background/Purpose: The ADVOCATE trial evaluated avacopan (a C5a receptor antagonist) as a replacement for a standard prednisone taper in the treatment of granulomatosis with polyangiitis…
Abstract Number: 1630 • ACR Convergence 2024
Disease Manifestations, Diagnostic Findings, and Mortality of 120 Consecutive Patients Suspected of Giant Cell Arteritis – Results from a Hospital GCA Fast-Track Clinic
Uffe Moller Dohn¹, Tanja Fromberg Gorlen², Rikke Westergaard¹, Viktoria Fana¹, Torsten Møller¹ and Lene Terslev³, ¹Rigshospitalet-Glostrup, Glostrup, Denmark, ²Rigshospitalet-Glostrup & COPECARE, Glostrup, Hovedstaden, Denmark, ³Rigshospitalet-Glostrup & COPECARE, Glostrup, Denmark
Background/Purpose: Giant cell arteritis (GCA) is a large-vessel vasculitis affecting the large vessels from aorta, its proximal branches, and the extra-cranial arteries. To avoid ischemic…
Abstract Number: 2180 • ACR Convergence 2024
The Association Between Age at Diagnosis and Patient-Reported Outcomes in Patients with Vasculitis
Jessica Bloom¹, Kaci Pickett-Nairne², Lori Silveira², David Cuthbertson³, Carol McAlear⁴, Peter Merkel⁴ and Karen James⁵, and the Vasculitis Clinical Research Consortium, ¹University of Colorado, Denver, CO, ²University of Colorado, Aurora, CO, ³University of South Florida, Tampa, FL, ⁴University of Pennsylvania, Philadelphia, PA, ⁵University of Utah, Salt Lake City, UT
Background/Purpose: Vasculitis is a chronic autoimmune condition that significantly impacts patients’ quality of life. It is unknown whether the degree of this impact varies based…
Abstract Number: 2505 • ACR Convergence 2024
C5 Signaling Pathway Genes as Key Drivers of the Pathogenesis of IgA Vasculitis?
Joao Carlos Batista-Liz¹, Vanesa Calvo-Rio², María Sebastián Mora-Gil³, Ligia Gabrie-Rodriguez⁴, Rafael Gálvez Sánchez⁴, Belén Sevilla-Pérez⁵, José Luis Callejas⁶, María Teresa Leonardo⁷, Ana Peñalba⁷, Javier Narvaez-García⁸, Luis Martín-Penagos⁹, Luis Caminal-Montero¹⁰, PAZ COLLADO¹¹, Patricia Quiroga Colina¹², Esther Vicente-rabaneda¹³, Esteban Rubio¹⁴, Manuel León Luque¹⁵, Juan María Blanco-Madrigal¹⁶, Eva Galindez-Agirregoikoa¹⁷, Santos Castañeda¹³, Ricardo Blanco-Alonso¹⁸, Veronica Pulito-Cueto¹ and Raquel Lopez-mejias¹, ¹IDIVAL, Santander, Spain, ²Valdecilla Hospital, Santander, Cantabria, Spain, ³Immunopathology Group, IDIVAL, Santander, Spain, ⁴Immunopathology Group, IDIVAL. Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁵Division of Pediatrics, Hospital Universitario San Cecilio, Granada, Spain, ⁶Systemic Autoimmune Diseases Unit, Hospital Clinico San Cecilio, Instituto de Investigación Biosanitaria de Granada ibs.GRANADA, Granada, Spain, ⁷Division of Pediatrics, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁸Hospital Universitario de Bellvitge, Barcelona, Spain, ⁹Immunopathology Group, IDIVAL. Division of Nephrology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ¹⁰Internal Medicine Department, Hospital Universitario Central de Asturias, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Oviedo, Spain, ¹¹Hospital Universitario Severo Ochoa, MADRID, Spain, ¹²Division of Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ¹³Hospital Universitario de La Princesa, Madrid, Spain, ¹⁴Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain, Sevilla, Spain, ¹⁵Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain., Sevilla, Spain, ¹⁶Division of Rheumatology, Hospital Universitario de Basurto, Bilbao, Spain., Bilbao, Spain, ¹⁷BASURTO UNIVERSITY HOSPITAL, BILBAO, Spain, ¹⁸Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: Immunoglobulin A Vasculitis (IgAV) is an inflammatory disease caused by the accumulation of immune complexes of IgA in the walls of small blood vessels…
Abstract Number: 2647 • ACR Convergence 2024
Clinical Characteristics , Treatment Outcomes and Predictors of Mortality in a DADA2 Cohort of 101 Patients
Aman Sharma¹, Niladri Bhowmick², shankar naidu³, Varun Dhir⁴, Prateek Bhatia³, Vishal Sharma³, sagar Bhattad⁵, Chengappa Kavadichanda⁶, Vikas Gupta⁷, Durga P Misra⁸, Sourabh Malviya⁹, DUDAM RAJKIRAN¹⁰, Banwari Sharma¹¹, John Mathew¹², Sathish Kumar¹³, Rajesh Bhojwani¹⁴, Aadhaar Dhooria¹⁵, Avinash Jain¹⁶, Pankaj Gupta¹⁷, Vikas Agarwal⁸, Rudrarpan Chatterjee¹⁸, Kusum Sharma¹⁹, Manphool Singhal¹⁹, Harjeet Singh¹⁹, Alpana Parmar²⁰, Padmanbha Shenoy²¹, Ritambhra Nada¹⁹, Ranjana Minz¹⁹, Archana Khan²², Sodality Sutnga²², Minu Singh¹⁹, Kaushik Bhojani²³, MAHABALESHWAR MAMADAPUR²⁴, Manesh Manoj²⁵, Puja Srivastava²⁶, Adarsh MB²⁷, Kavita Krishna²⁸, Ramesh Jois²⁹, Vir Singh Negi³⁰, Amita Aggarwal³¹, sanjay jain¹⁹, Raju Khubchandani³², Chip Chambers³³ and Pui Lee³⁴, ¹PGIMER, Chandigarh, India, Chandigarh, Chandigarh, India, ²Post Graduate Institute of Medical Education and Research , Chandigarh, Chandigarh, Chandigarh, India, ³PGIMER, Chandigarh, India, Chandigarh, India, ⁴PGIMER, CHD, INDIA, CHANDIGARH, India, ⁵2Aster CMI Hospital, Bengaluru, India, Bengaluru, ⁶Jawaharlal Institute of Postgraduate Medical Education and Research, pondicherry, Puducherry, India, ⁷Fortis Hospital, Ludhiana, India, Ludhiana, ⁸Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India, ⁹Medanta superspeciality hospital, Indore, India, ¹⁰Hyderabad Rheumatology Centre, Hyderabad, India, HYDERABAD, Andhra Pradesh, India, ¹¹Niramaya Healthcare, Jaipur, India, Jaipur, India, ¹²Christian Medical college , Vellore , India, Vellore, India, ¹³CMC, Vellore, Vellore, India, ¹⁴Santokba Durlabhji Memorial Hospital, Jaipur, India, Jaipur, India, ¹⁵Mahatma Gandhi Medical College and Hospital Jaipur, Jaipur, Rajasthan, India, ¹⁶Sawai Man Singh Hospital, Lucknow, Rajasthan, India, ¹⁷PGIMER, Chandigarh, India, Chandigarh, ¹⁸Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow., Lucknow, Uttar Pradesh, India, ¹⁹PGIMER, Chandigarh, Chandigarh, India, ²⁰Rheumatology Clinic, Surat, India, Surat, India, ²¹Center for arthritis and rheumatims excellence ( CARE, Cochin, Kerala, India, ²²SRCC Children’s Hospital, Mumbai, India, Mumbai, India, ²³Fortis Hospital, Mumbai, Mumbai, India, ²⁴JSS MEDICAL COLLEGE AND HOSPITAL,JSSAHER, Mysore, Karnataka, India, ²⁵AKG Memorial Cooperative Hospital Kannur, Nileshwar, Kerala, India, ²⁶STAR Rheumatology Clinics, Ahmedabad, India, Ahmedabad, India, ²⁷Government Medical College, Kozhikode, India, Kozhikode, India, ²⁸Bharati Vidyapeeth Medical College, Pune, Pune, India, ²⁹Manipal Hospital ,Rheumatology, Millers Road, Bengaluru, India, Bengaluru, India, ³⁰AIIMS, Bilaspur, Puducherry, Puducherry, India, ³¹Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, Lucknow, Uttar Pradesh, India, ³²SRCC Childrens Hospital, Mumbai, India, ³³Vanderbilt University Medical Center & DADA2 Foundation, Franklin, TN, ³⁴Boston Children's Hospital, Newton, MA
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is a rare and potentially life threatening monogenic disorder characterized by systemic vasculitis, bone marrow failure, and immunodeficiency.…
Abstract Number: 0742 • ACR Convergence 2024
Use of FDG-PET to Monitor Disease Activity in Patients with Giant Cell Arteritis on Tocilizumab
Kaitlin Quinn¹, mark ahlman² and Peter Grayson³, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²Radiology and Imaging, Medical College of Georgia, augusta, GA, ³National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD
Background/Purpose: The optimal way to monitor disease activity in patients with GCA on tocilizumab remains uncertain. Acute phase reactants are not a reliable indicator of…
Abstract Number: 0767 • ACR Convergence 2024
Comparing Large Vessel Uptake of 68Ga-HA-DOTATATE to 18F-FDG Using PET/CT Imaging in Patients with Active Giant Cell Arteritis
Alison Clifford¹, Jonathan Abele¹, Ryan Hung¹, Paolo Raggi¹, Frank Wuest¹, Jan Andersson¹, Elaine Yacyshyn², Eric Lenza¹, Glen Jickling¹ and Jan Willem Cohen Tervaert¹, ¹University of Alberta, Edmonton, Canada, ²University of Alberta, Edmonton, AB, Canada
Background/Purpose: 18F-fluoro-deoxyglucose (FDG) is the current standard among radiotracers for PET/CT imaging of large vessel vasculitis (LVV) in giant cell arteritis (GCA), but its performance…
Abstract Number: 1598 • ACR Convergence 2024
Projecting the Impact of Standard, Reduced-Dose, and Minimal-Dose Glucocorticoid Regimens in the Treatment of ANCA-Associated Vasculitis Using Simulation Modeling
Naomi Patel¹, Aaron Wu¹, Shruthi Srivatsan¹, Eli Miloslavsky², Peter Merkel³, John Stone⁴, Hyon K. Choi⁵, Emily Hyle¹ and Zachary Wallace², ¹Massachusetts General Hospital, Boston, MA, ²Massachusetts General Hospital, Newton, MA, ³University of Pennsylvania, Philadelphia, PA, ⁴Massachusetts General Hospital , Harvard Medical School, Concord, MA, ⁵Massachusetts General Hospital, Lexington, MA
Background/Purpose: Glucocorticoids (GCs) remain a cornerstone of treatment of ANCA-associated vasculitis (AAV) but predispose people to infectious, metabolic, and other toxicities. We projected the long-term…
Abstract Number: 1632 • ACR Convergence 2024
Multi-vessel Intimal Medial Thickness in Takayasu Arteritis: A Potential Marker for Disease Modification?
Augustine Jose¹, Molly Thabah², Chengappa Kavadichanda³, Jagan K L², Christina Mariaselvam⁴ and Vir Singh Negi⁵, ¹Jawaharlal Institute of Postgraduate Medical Education and Research, Gorimedu, Puducherry, India, ²Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, Puducherry, India, ³Jawaharlal Institute of Postgraduate Medical Education and Research, pondicherry, Puducherry, India, ⁴JIPMER, Puducherry, Puducherry, India, ⁵AIIMS, Bilaspur, Puducherry, Puducherry, India
Background/Purpose: Ultrasonography can measure vascular intimal-medial thickness (IMT) and delineate the degrees of stenosis in Takayasu arteritis (TAK). Further, higher IMT has been reported in…
Abstract Number: 2191 • ACR Convergence 2024
Optimizing Inpatient Kawasaki Disease Care
Audrea Chen¹, Beth Gamulka², Macarena Palomer³ and Rae Yeung⁴, ¹The Hospital for Sick Children, Burnaby, BC, Canada, ²The Hospital for Sick Children, Toronto, ³The Hospital for Sick Children, Toronto, Canada, ⁴The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
Background/Purpose: Kawasaki Disease (KD), an acute pediatric vasculitis, requires inpatient management to prevent coronary artery disease. The Hospital for Sick Children (SickKids) sees about 100…
Abstract Number: 2506 • ACR Convergence 2024
THP1-derived Macrophage Polarization in Takayasu Arteritis – Impact of Disease Status and Therapy
João Paulo dos Santos¹, Cristina Orikaza¹, Mariana Aguiar², Gerson Keppeke³ and Alexandre de Souza⁴, ¹Universidade Federal de Sao Paulo, São Paulo, SP, Brazil, ²Rheumatology Division, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil, ³Universidad Catolica del Norte, São Paulo, Coquimbo, Brazil, ⁴UNIFESP-EPM, São Paulo, SP, Brazil
Background/Purpose: Macrophages play an important role in the pathophysiology of Takayasu arteritis (TAK) as granulomatous inflammation is typically found in affected arteries. The expression of…

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