Abstracts tagged "Miscellaneous Rheumatic and Inflammatory Diseases"

Abstract Number: PP04 • ACR Convergence 2025
MCTD and Aging – Resilience Required
Carol Kaminski
Background/Purpose: I was diagnosed with MCTD at age 25. My priorities were understanding a disease I had trouble pronouncing, and trying to learn to live with…
Abstract Number: 1148 • ACR Convergence 2025
Interstitial Lung Disease in MCTD: A Retrospective Cohort Study at a Large Tertiary Medical Center
Alana Haussmann¹ and Elizabeth Volkmann², ¹University of California Los Angeles, Los Angeles, CA, ²Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA, Los Angeles, CA
Background/Purpose: Mixed connective tissue disease (MCTD) is a rare autoimmune condition defined by the presence of anti-U1-ribonucleoprotein (RNP) antibodies and clinical features of at least…
Abstract Number: 2701 • ACR Convergence 2025
Ophthalmic Manifestations of Relapsing Polychondritis
Akash Gupta¹, Rennie Rhee², Kaitlin Quinn³, Naomi Amudala², Nirali Bhatt⁴, Carol McAlear², Marcela ferrada⁵, Peter Grayson⁶, Peter Merkel² and Shubhasree Banerjee², ¹Hospital of University of Pennsylvania, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ⁴Scheie Eye Institute, University of Pennsylvania, Philadelphia, PA, ⁵University of Maryland, Bethesda, MD, ⁶National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD
Background/Purpose: The prevalence of ocular involvement (OI) in patients with relapsing polychondritis (RP) has not been well defined. This study aimed to describe ocular manifestations,…
Abstract Number: 1121 • ACR Convergence 2025
Latent Class Analysis Identifies Distinct Phenotypes of Multisystem Immune-Related Adverse Events Predictive of Survival After Immune Checkpoint Inhibitors
Tao Ming Sim¹, May Shuen Tang¹, Qai Ven Yap², Alvin Wong¹, Yiqing Huang¹ and Frank Sen Hee Tay¹, ¹National University Hospital, Singapore, Singapore, ²National University of Singapore, Singapore
Background/Purpose: Use of immune checkpoint inhibitors (ICIs) can lead to a broad spectrum of immune-related adverse events (irAEs) affecting multiple organ systems. No clear pattern…
Abstract Number: 2666 • ACR Convergence 2025
Rare TNFAIP3 Hypomorphic Variants are a Massively Underestimated Driver of Human Autoinflammatory Disease
Danica Lee¹, Urekha Karri², Yiming Luo³, Kader Cetin Gedik⁴, Manuel Carpio Tumba⁵, Prabal Chhibbar⁶, Priyamvada Roy⁶, Guido Falduto⁷, Jishnu Das⁵ and Daniella Schwartz⁵, ¹University of Pittsburgh School of Medicine, Pittsburgh, PA, ²School of public health, Pittsburgh, PA, ³Columbia University, New York, NY, ⁴UPMC Children's Hospital of Pittsburgh/University of Pittsburgh, Pittsburgh, PA, ⁵University of Pittsburgh, Pittsburgh, PA, ⁶University of Pittsburgh School of Medicine, Pittsburgh, ⁷University of Pittsburgh Medical Center, Pittsburgh, PA
Background/Purpose: TNFAIP3 encodes the ubiquitin editing enzyme A20, which inhibits multiple proinflammatory signaling pathways. Heterozygous germline mutations in TNFAIP3 cause the autoinflammatory disease Haploinsufficiency of…
Abstract Number: 1099 • ACR Convergence 2025
Safety and Effectiveness of Hydroxychloroquine in the Treatment of Rheumatic Immune-Related Adverse Events
Grace Haeun Lee¹, Gregory Challener², Janeth Yinh³, Jeffrey Sparks⁴, Kerry L. Reynolds⁵, Donald P. Lawrence⁵, Meghan J. Mooradian⁵, Ryan J. Sullivan⁵, Hyon K. Choi⁶ and Minna Kohler⁷, ¹Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, MA, ²Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, ³Massachusetts General Hospital, Boston, MA, ⁴Brigham and Women's Hospital, Boston, MA, ⁵Massachusetts General Hospital, Boston, ⁶MASSACHUSETTS GENERAL HOSPITAL, Lexington, MA, ⁷Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Hydroxychloroquine (HCQ) has been recommended [1-3] as a steroid-sparing agent for immune checkpoint inhibitor-inflammatory arthritis (ICI-IA), preferred due to its low immunosuppressive effect. However,…
Abstract Number: 2663 • ACR Convergence 2025
PAXIS: A Randomized, Double-Blind, Placebo-Controlled, Dose Finding Phase 2 Study (Part 1) Followed by an Open-Label Period (Part 2) to Assess the Efficacy and Safety of Pacritinib in Patients with VEXAS Syndrome
David Beck¹, Mael Heiblig², Sinisa Savic³, Marcela ferrada⁴, Arsène Mekinian⁵, Onima Chowdhury⁶, Danielle Hammond⁷, Lachelle D. Weeks⁸, Carmelo Gurnari⁹, Yohei Kirino¹⁰, Sophie georgin-Lavialle¹¹, Sarah A. Buckley¹², Bryan G. harder¹², Sandra Goble¹² and Matthew Koster¹³, ¹Center for Human Genetics and Genomics, NYU Grossman School of Medicine. Division of Rheumatology, Department of Medicine, NYU Grossman School of Medicine. Department of Biochemistry and Molecular Pharmacology, NYU Grossman School of Medicine, New York, NY, USA, New York, NY, ²Lyon-Sud Hospital, Hospices Civils de Lyon, Paris and Université Claude Bernard, Lyon, France, ³University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom, ⁴University of Maryland, Bethesda, MD, ⁵Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DMU i³), Saint-Antoine University Hospital, ⁷⁵⁰¹² Paris, France, Paris, France, ⁶Oxford University Hospitals’ NHS Foundation Trust and Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom, ⁷The University of Texas MD Anderson Cancer Center, Houston, TX, ⁸Dana Farber Cancer Institute, Boston, MA, ⁹Department of Biomedicine and Prevention, University of Rome Tor Vergata and Translational Hematology and Oncology Research Department, Taussig Cancer Center, Cleveland Clinic, Clevland, OH, Rome, Italy, ¹⁰Yokohama City University Graduate School of Medicine, Yokohama, Japan, ¹¹Sorbonne university, Tenon hospital, DMU³ID, CEREMAIA, ERN RITA, Paris, France, ¹²Sobi Inc., Waltham, MA, ¹³Mayo Clinic, Rochester, MN
Background/Purpose: VEXAS syndrome (Vacuoles, E1 ubiquitin-activating enzyme, X-linked, Autoinflammatory, Somatic) is a systemic disorder characterized by an overlap of hematologic and inflammatory features. Treatment poses…
Abstract Number: 1089 • ACR Convergence 2025
Comparing the immunosuppressant burden in immune checkpoint inhibitor mediated inflammatory arthritis versus polymyalgia rheumatica: results from a prospective multicenter registry
Alice Tison¹, Deanna Jannat-Khah², Laura Cappelli³ and Anne R. Bass², ¹LBAI, UMR¹²²⁷, University of Brest, CHU Brest, Brest, France, Brest, France, ²Hospital For Special Surgery, New York, NY, ³Johns Hopkins School of Medicine, Baltimore, MD
Background/Purpose: Inflammatory arthritis (IA)- and polymyalgia rheumatica (PMR)-like syndromes occur in about 6% of patients receiving an immune checkpoint inhibitor (ICI) and can worsen quality…
Abstract Number: 2442 • ACR Convergence 2025
Reduction in Extrafollicular B Cell Responses in SLE Patients after CAR T Cell Therapy
Danae-Mona Nöthling¹, Kirill Anoshkin¹, Panagiotis Garantziotis¹, Laura Bucci¹, Tobias Rothe², Jule Taubmann³, Futoshi Iwata¹, Melanie Hagen¹, Andreas Wirsching¹, Simon Völkl⁴, Fabian Müller⁵, Aline Bozec¹, Andreas Mackensen⁶, Georg Schett⁷ and Ricardo Grieshaber-Bouyer⁸, ¹Department of Medicine ³ - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, ²Department of Medicine ³ - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlagen, Germany, ³Department of Medicine ³ - Rheumatology and Immunology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁴Department of Medicine ⁵ - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁵University Hospital of Erlangen, Erlangen, Germany, ⁶Department of Medicine ⁵ - Hematology and Oncology, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU) and Uniklinikum Erlangen, Erlangen, Germany, ⁷Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, ⁸University Hospital Erlangen, Erlangen, Germany
Background/Purpose: Systemic lupus erythematosus (SLE) is characterized by aberrant B cell activation and autoantibody production. CD19-targeted chimeric antigen receptor (CAR) T-cell therapy induces a short,…
Abstract Number: 1088 • ACR Convergence 2025
Unsupervised characterization of immune checkpoint inhibitor induced inflammatory arthritis using cluster and latent class analysis: result from a multicenter prospective registry
Alice Tison¹, Deanna Jannat-Khah², Laura Cappelli³ and Anne R. Bass², ¹LBAI, UMR¹²²⁷, University of Brest, CHU Brest, Brest, France, Brest, France, ²Hospital For Special Surgery, New York, NY, ³Johns Hopkins School of Medicine, Baltimore, MD
Background/Purpose: The broad use of immune checkpoint inhibitors (ICIs) in oncology has led to the emergence of ICI-induced inflammatory arthritis (ICI-IA). The aim of this…
Abstract Number: 2037 • ACR Convergence 2025
The Largest International Cohort Study of HA20 Reveals Novel Genetic Architecture and Age-Dependent Phenotypic Evolution
Manuel Carpio Tumba¹, Tingyan He², Jun Wang³, Elizabeth Kairis¹, Kyr Goyette¹, Akuti Kethri¹, Urekha Karri⁴, Atif Towheed¹, Jun Yang⁵, Qing Zhou⁶ and Daniella Schwartz¹, ¹University of Pittsburgh, Pittsburgh, PA, ²Shenzhen Children's Hospital, Shenzhen, China (People's Republic), ³Zhejiang University School of Medicine, Zhejiang, China (People's Republic), ⁴School of public health, Pittsburgh, PA, ⁵Rheumatology and Immunology Department of Shenzhen Children's Hospital, Shenzhen, ⁵¹⁸¹⁰⁰, China., Shenzhen, China (People's Republic), ⁶Zhejiang University School of Medicine, Hangzhou, China (People's Republic)
Background/Purpose: Haploinsufficiency of A20 (HA20) is a monogenic autoinflammatory disease caused by loss-of-function (LOF) mutations in TNFAIP3. HA20 was initially described as an early-onset form…
Abstract Number: 0777 • ACR Convergence 2025
Development of a Disease Activity Index for the Assessment of VEXAS Syndrome (VEXAS-DAI)
Kevin Byram¹, Herman Mann², Danielle Hammond³, Sinisa Savic⁴, Yohei Kirino⁵, Carmelo Gurnari⁶, Mael Heiblig⁷, Thibault Comont⁸, Arsène Mekinian⁹, Mrinal Patnaik¹⁰, Lachelle D. Weeks¹¹, Gary Ho¹², Onima Chowdhury¹³, Adam Al-Hakim¹⁴, Scott Goldberg¹⁵, Marcela ferrada¹⁶, Sophie georgin-Lavialle¹⁷, Peter Grayson¹⁸, Emma Groarke¹⁹, Bhavisha Patel²⁰, Megan Sullivan²¹, Sarah A. Buckley²², Bryan G. harder²², Sandra Goble²², Matthew Koster¹⁰ and David Beck²³, ¹Vanderbilt University Medical Center, Nashville, TN, ²Institute of Rheumatology, Praha ², Czech Republic, ³The University of Texas MD Anderson Cancer Center, Houston, TX, ⁴University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom, ⁵Yokohama City University Graduate School of Medicine, Yokohama, Japan, ⁶Department of Biomedicine and Prevention, University of Rome Tor Vergata and Translational Hematology and Oncology Research Department, Taussig Cancer Center, Cleveland Clinic, Clevland, OH, Rome, Italy, ⁷Lyon-Sud Hospital, Hospices Civils de Lyon, Paris and Université Claude Bernard, Lyon, France, ⁸Centre Hospitalier Universitaire Toulouse Institut Universitaire du Cancer Toulouse Oncopole, Toulouse, France, ⁹Department of Internal Medicine, Inflammation-Immunopathology-Biotherapy Department (DMU i³), Saint-Antoine University Hospital, ⁷⁵⁰¹² Paris, France, Paris, France, ¹⁰Mayo Clinic, Rochester, MN, ¹¹Dana Farber Cancer Institute, Boston, MA, ¹²New York University Grossman School of Medicine, VA New York Harbor Health Care System, Brooklyn, NY, ¹³Oxford University Hospitals’ NHS Foundation Trust and Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom, ¹⁴University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine,, Leeds, United Kingdom, ¹⁵New York University Grossman School of Medicine, Brooklyn, NY, ¹⁶University of Maryland, Bethesda, MD, ¹⁷Sorbonne university, Tenon hospital, DMU³ID, CEREMAIA, ERN RITA, Paris, France, ¹⁸National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD, ¹⁹National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, MD, ²⁰National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), Beltsville, MD, ²¹Mayo Clinic Arizona, Scottsdale, AZ, ²²Sobi Inc., Waltham, MA, ²³Center for Human Genetics and Genomics, NYU Grossman School of Medicine. Division of Rheumatology, Department of Medicine, NYU Grossman School of Medicine. Department of Biochemistry and Molecular Pharmacology, NYU Grossman School of Medicine, New York, NY, USA, New York, NY
Background/Purpose: VEXAS syndrome is a recently described severe disease characterized by a complex overlap of inflammatory and hematologic features. Due to the severity and refractory…
Abstract Number: 2034 • ACR Convergence 2025
Hidden Burden in Rheumatology: Cross-Sectional Study of Hearing Loss in Systemic Autoimmune Diseases
Ruddy Paola Montoya Rumpf¹, Liliam María Murcia Munévar¹, Jairo Cajamarca-Baron², Dominique Daniela González Casas¹, María Fernanda González Sánchez¹, Adriana Rojas-Villarraga³, Henry Leonardo Martínez Bejarano¹, Adriana Isaza¹, Alejandro Escobar¹, Maria Camila Restrepo Guarnizo¹, Nicolás Santiago Rodríguez¹ and Claudia Ibáñez-Antequera¹, ¹Fundacion universitaria de ciencias de la salud, bogota, Distrito Capital de Bogota, Colombia, ²Fundacion universitaria de ciencia de la Salud, Bogota, Distrito Capital de Bogota, Colombia, ³Fundacion Universitaria de Ciencias de la salud, Bogotá, Distrito Capital de Bogota, Colombia
Background/Purpose: Autoimmune inner ear disease is a progressive sensorineural hearing loss of autoimmune origin, often underdiagnosed due to the lack of specific criteria, and requires…
Abstract Number: 0494 • ACR Convergence 2025
LFD-200, an Antibody Drug Conjugate that Selectively Delivers a Glucocorticoid Payload to Immune Cells, Provides Sustained Anti-inflammatory Effects Without Systemic Toxicity in Non-human Primates
Matt McClure¹, Catherine Carriere¹, Kierstin Bell¹, Rex Williams², Geoff Kuesters², Emily Sansevere², Dave Nichols², Arthur Tzianabos² and Jay Rothstein¹, ¹Lifordi Immunotherapeutics, Lebanon, NH, ²Lifordi Immunotherapeutics, Burlington, MA
Background/Purpose: Glucocorticoids (GCs) are the most versatile and efficacious anti-inflammatory drugs rheumatologists have available for patients. Unfortunately, prolonged systemic GC exposure leads to unacceptable toxicities,…
Abstract Number: 2026 • ACR Convergence 2025
Clinical Features and Treatment Outcomes in Aseptic Abscess Syndrome: A Systematic Review of 104 Cases
Eleni Papachristodoulou¹, Loukas Kakoullis¹, Ratnesh Chopra², Chris Derk³ and Konstantinos Parperis⁴, ¹Mount Auburn Hospital, Harvard Medical School, Cambridge, MA, ²UMass Chan Medical School, Worcester, MA, ³University of Pennsylvania, Philadelphia, PA, ⁴University of Cyprus Medical School, Nicosia, Cyprus
Background/Purpose: Aseptic abscess syndrome (AAS) is an inflammatory condition of unknown etiology, characterized by the formation of sterile neutrophilic abscesses that frequently respond to immunosuppressive…

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