Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0006 • ACR Convergence 2024
NX-5948, a Clinical-Stage BTK Degrader, Achieves Deep Suppression of BCR, TLR, and FcR Signaling in Immune Cells and Demonstrates Efficacy in Preclinical Models of Arthritis and Other Inflammatory Diseases
Mark Noviski¹, Jun Ma¹, Nivetha Brathaban¹, Aishwarya Kumar¹, Dhwani Haria¹, Jenny McKinnell¹, Robert Cass¹, Frederick Cohen¹, Davorka Messmer², Gwenn Hansen¹ and Ryan Rountree¹, ¹Nurix Therapeutics, San Francisco, CA, ²Nurix Therapeutics, San Diego, CA
Background/Purpose: Bruton’s tyrosine kinase (BTK) mediates signaling downstream of the B cell receptor (BCR), toll-like receptors (TLRs), and Fc receptors (FcRs). This makes BTK an…
Abstract Number: 0408 • ACR Convergence 2024
Analysis of Protein Biomarkers for the Prediction of IL-1 Inhibitor Treatment Response in the CARRA First-line Options for Systemic Juvenile Idiopathic Arthritis Treatment (FROST) Study
Michael Matt¹, Mariana Correia Marques², Sabrina Fuehner³, Lexi Auld¹, George Tomlinson⁴, Michael Ombrello⁵, Yukiko Kimura⁶, Christoph Kessel⁷ and grant schulert¹, and the CARRA FROST Investigators, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²National Institutes of Health, Bethesda, MD, ³Universitaetsklinikum Muenster, Muenster, Germany, ⁴University of Toronto, Toronto, ON, Canada, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁶Hackensack Meridian School of Medicine, New York, NY, ⁷University Hospital Muenster, Muenster, Germany
Background/Purpose: IL-1 inhibitors are both widely used and highly effective as first-line therapy for children with systemic juvenile idiopathic arthritis (sJIA); however, the mechanisms underlying…
Abstract Number: 1062 • ACR Convergence 2024
Assessing Musculoskeletal Symptom Burden in a Cohort of Patients with Hidradenitis Suppurativa Using the IDEOM MSK-Q
Gretchen Ball¹, Hassan Hamade², Sarah Romanelli², Zachary Levy², Lourdes Perez Chada³, Joseph Merola⁴ and Alice Gottlieb⁵, ¹Albert Einstein College of Medicine, Ridgefield, CT, ²Icahn School of Medicine at Mount Sinai, New York, NY, ³Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, ⁴UT Southwestern Medical Center, Newton, MA, ⁵Icahn School of Medicine at Mount Sinai, Department of Dermatology, Seattle, WA
Background/Purpose: Hidradenitis suppurativa (HS) has been associated with inflammatory joint diseases such as psoriatic arthritis and ankylosing spondylitis. Despite this correlation, dermatologists do not routinely…
Abstract Number: 1778 • ACR Convergence 2024
A Novel Variant in IRAK2 Results in Immune Dysregulation in Systemic Juvenile Idiopathic Arthritis (sJIA)
Mariana Correia Marques¹, Brooke Boyd², Alana Platukus³, Elizabeth Schmitz², Hiroto Nakano², Faiza Naz⁴, Anthony Cruz¹, Stefania Dell'Orso⁴, Zuoming deng⁴ and Michael Ombrello⁵, ¹National Institutes of Health, Bethesda, MD, ²Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ³1Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD
Background/Purpose: Lung disease (LD) is poorly understood complication of Still’s disease with high fatality. HLA-DRB1*15 is a strong risk factor for the development of this…
Abstract Number: 2046 • ACR Convergence 2024
Duration of Disease and Long-term Outcomes in Patients with Difficult-to-treat Recurrent Pericarditis: A Chronic Condition Treated with NSAIDs, Colchicine, Corticosteroids, and Anti-IL-1 Agents
Antonio Brucato¹, Elisa Ceriani², Francesco Agozzino³, Silvia Berra⁴, Antonio Gidaro², Silvia Macchi⁵, Letizia Vena⁵, Paolo Bindi⁵, Alberto Pavarani⁶, Francesco Moda⁷, Ludovico Luca Sicignano⁸, Celeste Murace⁸, Elena Verrecchia⁸, Caterina Chiara Decarlini⁹, Silvia Maestroni¹⁰, Gabriella Marinaro⁴, Lucia Trotta³, Massimo Pancrazi³, Lisa Serati³, Enrica Negro³, Claudia Gabiati³, Elisa Calabrò³, Angela Mauro¹¹, Luisa Carrozzo³, Emanuele Bizzi³ and Massimo Imazio¹², ¹Department of Biomedical and Clinical Sciences, University of Milano, Department of Internal Medicine, Ospedale Fatebeneratelli, Milano, Milano, Lombardia, Italy, ²Department of Internal Medicine, Ospedale Luigi Sacco, Milano, Milan, Italy, ³Department of Internal Medicine, Ospedale Fatebeneratelli, Milano, Milan, Italy, ⁴Department of Internal Medicine, Ospedale Fatebeneratelli, Milano, Milano, Italy, ⁵University of Milano, Milan, Italy, ⁶University of Milano, Milan, ⁷Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy, ⁸Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Rome, Italy, ⁹Cardiology Division, Cardiovascular Department, San L. Mandic Hospital, Merate, Lecco, Italy, ¹⁰Internal Medicine Department, Papa Giovanni XXIII Hospital, Bergamo, Bergamo, Italy, ¹¹Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy, ¹²University of Udine, and Cardiothoracic Department, University Hospital Santa Maria della Misericordia, Udine, Milan, Italy
Background/Purpose: Recurrent pericarditis (RP) challenges pts and clinicians in terms of long-term management. Objective of this study was to evaluate the remission rate (drug-free for…
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…

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