Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 2660 • ACR Convergence 2024
Developing Predictive Models for the Diagnosis of VEXAS Syndrome
Daniel Montes¹, Andrew C. Hanson², Hannah Langenfeld¹, Cynthia Crowson¹, Mrinal Patnaik¹, Ronald Go¹, Alexander Hines¹, Kambiz Kalantari¹, Yael Kusne³, Terra Lasho², Abhishek Mangaonkar¹, Horatiu Olteanu¹, Kaaren Reichard¹, Megan Sullivan⁴, David Viswanatha¹, Kenneth Warrington¹ and Matthew Koster¹, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic, Rochester, ³Mayo Clinic, Phoenix, AZ, ⁴Mayo Clinic Arizona, Scottsdale, AZ
Background/Purpose: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a recently identified autoinflammatory disorder with a heterogenous presentation. Patients may experience delays in diagnosis…
Abstract Number: 0072 • ACR Convergence 2024
The Association Between Activation of Inflammasome and HLA-B27 in Dendritic Cells of Patients with Axial Spondyloarthritis
Jina Yeo¹, Min-Gang Kim², Hye Eun Lee³, Yeonsoo Yang², Mi Ryoung Seo¹, Hyo-Jin Choi¹, Eun Young Lee⁴, Dae Ho Lee³ and Han Joo Baek⁵, ¹Division of Rheumatology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, Republic of Korea, ²Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Republic of Korea, ³Division of Endocrinology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Incheon, Republic of Korea, ⁴3Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea, ⁵Division of Rheumatology, Department of Internal Medicine, Gil Medical Center, Gachon University College of Medicine, Inchon, South Africa
Background/Purpose: Axial spondyloarthritis (axSpA) exhibits a predominant involvement of the innate immune system, marked by abnormal functions of innate immune cell such as dendritic cells…
Abstract Number: 0414 • ACR Convergence 2024
Relationship Between Culture Negative Infectious Osteomyelitis and Chronic Nonbacterial Osteomyelitis
Andrew Grim¹, Yongdong (Dan) Zhao², Christine Wang¹, Erin Janssen³, Jessica Turnier⁴, Anastasia Hyrhorczuk¹ and Nadine Saad¹, ¹University of Michigan, Ann Arbor, MI, ²University of Washington, Redmond, WA, ³CS Mott Children's Hospital, Ann Arbor, MI, ⁴University of Michigan, Saline, MI
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is a rare autoinflammatory bone disorder. Some patients with CNO may present acutely and be misdiagnosed as infectious osteomyelitis (IO).…
Abstract Number: 1112 • ACR Convergence 2024
Pleuroparenchymal Fibroelastosis Associated with Connective Tissue Diseases (PPFE-CTD)
Maximiliano Diaz Menindez¹, Alejandro Diaz Arumir Vergara², Vivian Shing², carolyn mead harvey³, Ana Zamora Martinez² and Vivek Nagaraja⁴, ¹Mayo Clinic School of Graduate Medical Education, Phoenix, AZ, ²Mayo Clinic, Phoenix, ³Mayo Clinic Florida, Jacksonville, FL, ⁴Mayo Clinic Arizona, Scottsdale, AZ
Background/Purpose: Pleuroparenchymal Fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia characterized by fibrosis of the visceral pleura and subpleural parenchyma, predominantly affecting the upper lobes.…
Abstract Number: 1788 • ACR Convergence 2024
Memory B Cell Activation and Dysregulation in Systemic Lupus Erythematosus
Shady Younis¹, Salvinaz Moutusy², Shaghayegh Jahanbani², Xiaohao Wu², Marlayna Harris², Mahesh Pandit³, Laura van Dam⁴, orr Sharpe⁵, Paul Utz¹ and William Robinson⁶, ¹Stanford University, Stanford, CA, ²Stanford University School of Medicine, Stanford, ³Stanford University School of Medicine, Stanofrd, ⁴Stanford, Palo Alto, CA, ⁵Department of Immunology and Rheumatology, Stanford University, Stanford, CA, ⁶Division of Immunology and Rheumatology, Stanford University, and VA Palo Alto Health Care System, Stanford, CA
Background/Purpose: B cell dysregulation and production of autoantibodies against autoantigens are hallmarks of Systemic lupus erythematosus (SLE). In healthy adults (HC), B cells with autoreactive…
Abstract Number: 2054 • ACR Convergence 2024
Phenotypical Differences in VEXAS Syndrome, Where Are We a Year Later? Results from a National Cohort Focused on Rheumatological Patients
Paula García Escudero¹, Marta López I Gómez², Berta Magallanes López³, Diego Dios Santos⁴, Francisco Javier Toyos Sáenz de Miera⁵, Alicia García Dorta⁶, José Ángel Hernández Beriain⁷, Íñigo Rúa-Figueroa⁸, cristiana sieiro santos⁹, Elvira Díez Álvarez¹⁰, Marta Ibáñez Martínez¹¹, Beatriz Frade-Sosa¹², Judit Font Urgelles¹³, Meritxell Sallés Lizarzaburu¹⁴, Elena Riera Alonso¹⁵, Ernesto Trallero Araguás¹⁶, Eugenia Enríquez Merayo¹⁷, Maria Rodriguez-Laguna¹⁸, irene monjo¹⁹, Carolina Merino²⁰, Paloma Vela-Casasempere²¹, Ignacio Vázquez Gómez²², Jose Alberto Miranda Filloy²³, Rafael Benito Melero-Gonzalez²⁴, Clara Garcia Belando²⁵, Giuliano Boselli²⁶, Alina Lucica Boteanu²⁷, Dolly Viviana Fiallo Suárez²⁸, Cristina Corrales Selaya²⁹, ALBERTO MARIANO RUIZ ROMAN³⁰ and Jaime Calvo-Alén³¹, ¹Hospital Universitario Araba, Vitoria, ²Hospital Universitario Alava, Vitoria, Pais Vasco, Spain, ³Hospital de la Santa Creu i Sant Pau, Barcelona, ⁴C. H. U. A Coruña, C. H. U. A Coruña, ⁵H. U. Virgen Macarena, H. U. Virgen Macarena, ⁶Rheumatologist, La Laguna, Spain, ⁷Rheumatology Department. Hospital Universitario Insular de Gran Canaria, Las Palmas de Gran C, Spain, ⁸Department of Rheumatology, Hospital Universitario Doctor Negrín, Las Palmas de Gran Canaria, Las Palmas GC, Spain, ⁹Rheumatology Department, Complejo Asistencial Universitario de León, León, Spain, Leon, Spain, ¹⁰Complejo Asistencial Universitario de León, León, Spain, ¹¹Hospital Clínico Universitario de Salamanca, Salamanca, Spain, ¹²Hospital Clinic de Barcelona, Barcelona, Spain, ¹³Hospital Universitari Germans Trias i Pujol, Badalona, Spain, ¹⁴Althaia Xarxa Assistencial Universitària Manresa, Manresa, Catalonia, Spain, ¹⁵Mutua de Terrasa, Barcelona, ¹⁶Hospital Vall D'Hebron, Barcelona, Spain, ¹⁷Hospital 12 de Octubre, Madrid, ¹⁸Resident in Rheumatology, Madrid, Spain, ¹⁹University Hospital La Paz, Madrid, Spain, ²⁰Hospital Universitario Puerta de Hierro Majadahonda., Majadahonda (Madrid), Spain, ²¹Hospital General Universitario Alicante, Alicante, Spain, ²²H. U. Dr. Peset, Valencia, ²³C. H. U. Lucus Augusti, Lugo, Spain, ²⁴CHU Ourense, O Carballino, Spain, ²⁵H. C. U. Virgen de la Arrixaca, Murcia, ²⁶hospital miguel servet, zaragoza, Aragon, Spain, ²⁷H.U. Ramón y Cajal, Madrid, Spain, ²⁸H. U. de Gran Canaria. Dr. Negrin, H. U. de Gran Canaria, ²⁹Rheumatology, Marques de Valdecilla University Hospital. IDIVAL, Santander, Cantabria, Spain, ³⁰Hospital Universitario Juan Ramón Jiménez, Huelva, Spain, ³¹Department of Rheumatology, Hospital Araba, Vitoria, Pais Vasco, Spain
Background/Purpose: VEXAS syndrome is an autoinflammatory disorder caused by a mutation in the UBA1 gene, which leads to a heterogeneous clinical presentation and progressive bone…
Abstract Number: PP09 • ACR Convergence 2024
Finding the Balance: Regaining My Strength While Living with Sjögren’s & POTS
Robert Fearon, Sjögren's Foundation, Reston, VA
Background/Purpose: Like many suffering from autoimmune diseases, my diagnosis was not straightforward and took nearly 13 years to get answers. I first started experiencing an…
Abstract Number: 0081 • ACR Convergence 2024
The Axial Spondyloarthritis Risk Variant CARD9 S12N Enhances Neutrophil-mediated Th Responses
Hillary Struthers¹, Kofi Asare-Konadu², Emily Vance¹ and Ruth Napier³, ¹Oregon Health & Science University, Portland, OR, ²Department of Molecular Microbiology and Immunology, Oregon Health & Science University, Portland, OR, ³Department of Molecular Microbiology and Immunology and Division of Arthritis and Rheumatic Disease, Oregon Health & Science University, VA Portland Health Care System, Portland, OR
Background/Purpose: Axial spondyloarthritis (axSpA) is a type of inflammatory arthritis that primarily affects the entheses of the spine and/or sacroiliac joints. The cause of axSpA…
Abstract Number: 0570 • ACR Convergence 2024
Prevalence of Comorbidities and Racial Disparities in Anterior Uveitis Risk Among Ankylosing Spondylitis Patients
Halah Alfatlawi¹, abdulmajeed alharbi², eun seo kwak², rawnag el sheikh² and nezam altorok², ¹the university of toledo, Toledo, OH, ²the university of toledo, Toledo
Background/Purpose: Ankylosing spondylitis (AS) is a chronic inflammatory condition primarily affecting the axial spine, commonly associated with extra-articular manifestations such as anterior uveitis (AU). Racial…
Abstract Number: 1120 • ACR Convergence 2024
Long-Term Echocardiographic Findings in Idiopathic Recurrent Pericarditis Patients Treated with Anakinra
Zeynep Toker Dincer¹, Damla Raimoglu², Erkin Yilmaz³, Ayten Dag¹, Sejla Karup⁴, Melike Melikoglu¹ and Serdal Ugurlu⁵, ¹Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Cardiology, Istanbul, Turkey, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Istanbul, Turkey, ⁴Cerrahpasa Faculty of Medicine, Istanbul, Turkey, ⁵Istanbul University-Cerrahpasa, Istanbul, Turkey
Background/Purpose: Idiopathic recurrent pericarditis (IRP) is characterized by recurrent pericarditis attacks, and autoinflammatory pattern has been highlighted due to similarities in both clinical features and…
Abstract Number: 1886 • ACR Convergence 2024
Heterogeneity of VEXAS Syndrome: A Multicenter Case-series of 299 Cases from the French VEXAS Study Group (FRENVEX)
Sophie Georgin-lavialle¹, Benjamin Terrier², Vincent Jachiet³, Rim Bourguiba⁴, jerome HAdjaj⁵, olivier Kosmider⁶ and Arsene Mekinian⁷, and French VEXAS study group, ¹Sorbonne Université, Department of internal medicine, Tenon Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ²Service de Médecine interne, Hôpital Cochin, AP-HP, Paris, Ile-de-France, France, ³Service de médecine interne, AP-HP, Hôpital Saint Antoine, Sorbonne Universite, Paris, France., Paris, France, ⁴Hopital des Forces de sécurité de l'interieur, La Marsa, Tunisia, ⁵Saint antoine APHP, Paris, France, ⁶Hopital Cochin, Paris, France, ⁷Saint Antoine University Hospital, Paris, France
Background/Purpose: VEXAS syndrome is a monogenic somatic autoinflammatory syndrome associated with UBA1 mutations. Objective. To describe clinical characteristics, laboratory findings, main treatments and outcomes of…
Abstract Number: 2152 • ACR Convergence 2024
Factors Associated with Health-related Quality of Life in Adults with SAPHO and Chronic Nonbacterial Osteomyelitis – a Cross Sectional Analysis from the SAPHO-CNO Study
Aleksander Lenert¹, Helena Abodeely¹, Karmela Kim Chan², Sandy Hong³, Arundathi Jayatilleke⁴, Courtney Kremer⁵, Emma Leisinger¹, Petar Lenert⁶, Melissa Oliver⁷, T. Shawn Sato⁸, Melanie Smith², Thomason Jenna⁹, Yongdong (Dan) Zhao¹⁰, Jonathan Templin¹, Robyn Domsic¹¹, Mary Vaughan-Sarrazin¹, Daniel Solomon¹² and Polly Ferguson¹³, ¹University of Iowa, Iowa City, IA, ²Hospital For Special Surgery, New York, NY, ³University of Iowa Stead Family Children's Hospital, Iowa City, IA, ⁴Temple University, Philadelphia, PA, ⁵University of Iowa Stead Family Children's Hospital, Solon, Iowa, IA, ⁶University of Iowa Hospitals and Clinics, Iowa City, IA, ⁷Indiana University, Indianapolis, IN, ⁸University of Iowa, Iowa City, ⁹University of Washington, Division of Rheumatology, Seattle, WA, ¹⁰University of Washington, Redmond, WA, ¹¹Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ¹²Brigham and Women's Hospital, Newton, MA, ¹³University of Iowa Carver College of Medicine, Iowa City, IA
Background/Purpose: Health-related quality of life (HRQOL) is decreased in individuals with chronic rheumatic diseases (CRD) yet remains undefined in adults with SAPHO syndrome and chronic…
Abstract Number: PP10 • ACR Convergence 2024
Walking Away from Arthritis Pain: Exercise Helped Me Manage Sarcoidosis and Osteoarthritis Physically and Emotionally
Bridget Kelly, Hospital for Special Surgery, Rockville Centre, NY
Background/Purpose: In 2004, we moved to a new home with 2 small children, when I started experiencing terrible joint pain. For 2 years I was…
Abstract Number: 0082 • ACR Convergence 2024
Comparative Immunology of Entheseal Anchorage Sites Between Spine, Hip and Knee Demonstrates up to 70-fold Greater IL-23 Induction from Axial Enthesis Bone: A New Angle on the Failure of IL-23 Blockade in Ankylosing Spondylitis
Mark Harland¹, Paula David², Chi Wong¹, Jake Weddell¹, Abhay Rao³, Almas Khan³, Jake Timothy³, Peter Loughenbury³, Robert Dunsmuir³, Vishal Borse³, Campbell MacEachern³, Antonius Pieter Van der Heuvel⁴, Warner Chen⁴, Tom Macleod¹ and Dennis McGonagle⁵, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine (LIRMM), University of Leeds, Leeds, UK, Leeds, United Kingdom, ²Department of Medicine B, Sheba Medical Center, Tel Hashomer, Israel, Ramat Gan, Israel, ³Leeds Teaching Hospitals NHS Trust, Leeds, UK, Leeds, United Kingdom, ⁴Janssen Research & Development, LLC, a Johnson & Johnson Company, Spring House, PA, USA, Spring House, PA, ⁵National Institute for Health Research (NIHR) Leeds Biomedical Research Centre (BRC), Leeds Teaching Hospitals, Leeds, UK, Leeds, England, United Kingdom
Background/Purpose: Both peripheral and axial spondyloarthropathy (SpA), psoriasis and inflammatory bowel disease are strongly linked to IL-23 pathway immunogenetics and immunology. Unexpectedly, IL-23 inhibition lacked…
Abstract Number: 0632 • ACR Convergence 2024
Cutaneous Lupus Erythema and Scale Have Similar Six-month Trends Without Significant Impact from Race/ethnicity or Disease Subtype
Tyler Cepica¹, Lillian Xie², Daniella Faden², Caroline Stone³, Victoria Werth⁴ and Benjamin Chong⁵, ¹University of Texas Southwestern Medical School, Benbrook, TX, ²University of Pennsylvania, Philadelphia, PA, ³University of Pennsylvania, Phiiladelphia, PA, ⁴Hospital of the University of Pennsylvania, Philadelphia, PA, ⁵The University of Texas Southwestern Medical Center, Department of Dermatology, Dallas, TX
Background/Purpose: The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a validated outcome measure designed to assess cutaneous lupus erythematosus (CLE) activity (CLASI-A)…

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