Abstract Number: 1573 • ACR Convergence 2024
Early Scleroderma with Non-Raynaud’s Symptoms Prior to Raynaud’s Onset Is Associated with Rapid Progression to Diffuse Skin Disease and Joint Contractures
Background/Purpose: Raynaud’s phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc). RP alerts clinicians to the possibility of an autoimmune rheumatic disease,…Abstract Number: 1750 • ACR Convergence 2024
Safety and Preliminary Efficacy of CD19 CAR-T Cell Treatment in Rheumatic Disease – Data from the First Part of the Phase I/II CASTLE Basket Study
Background/Purpose: Systemic autoimmune diseases are based on an aberrant activation of B cells. Autologous CD19 chimeric antigen receptor (CAR) T cells allow deep depletion of…Abstract Number: 1834 • ACR Convergence 2024
Altered Mechanotransduction via Myosin II Contributes to Collagen and IL-6 Production in Systemic Sclerosis Skin
Background/Purpose: Scleroderma (SSc), an autoimmune disease, features progressive fibrosis, leading to significant morbidity and mortality. Current therapies manage symptoms but lack efficacy in directly targeting…Abstract Number: 2455 • ACR Convergence 2024
Improvement Across Multi-organ Domains and Patient Reported Outcomes in Refractory Juvenile-Onset Systemic Sclerosis (jSSc) up to 4 Years After Autologous Stem Cell Transplantation (ASCT)
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is a systemic autoimmune disease characterized by vasculopathy and multiorgan fibrosis leading to significant morbidity and early mortality. Autologous stem…Abstract Number: 2643 • ACR Convergence 2024
Integrated Bulk and Single Cell RNA Sequencing Defines Key Pathways Regulating Myofibroblast Differentiation Across ANA Subgroups in Diffuse Systemic Sclerosis
Background/Purpose: Myofibroblasts are key cells in the pathogenesis of systemic sclerosis (SSc). TGFβ is a key growth factor driving myofibroblast formation in SSc. The main…Abstract Number: 0687 • ACR Convergence 2024
Patterns of Disease Progression in Early Systemic Sclerosis Patients with Interstitial Lung Disease
Background/Purpose: Understanding the trajectory of disease progression in SSc-ILD is crucial for effective patient management and prognostication. Trajectory modeling offers a novel approach to deciphering…Abstract Number: 0786 • ACR Convergence 2024
First Analyses of Transcriptomic Changes in the Skin of SSc Patients upon CD19-targeting CAR T Cell Therapy
Background/Purpose: CD19-targeting CAR T cells showed remarkable improvements of modified Rodnan skin score in SSc patients within 6 months after treatment and stable reduction afterwards…Abstract Number: 0973 • ACR Convergence 2024
Mechano-transduction via MRTF-A Pathway Is Required for Cytokine Release by Scleroderma Macrophages
Background/Purpose: In keeping with hallmark clinical evidence of stiffening and fibrotic thickening of the skin, we and others have previously demonstrated a role for the…Abstract Number: 1575 • ACR Convergence 2024
Anti-U1RNP Antibodies Are Associated with a Distinct Clinical Phenotype and a Worse Survival in Patients with Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is a rare systemic disease within the connective tissue disease (CTD) spectrum. It is characterized by microcirculatory abnormalities, skin and internal…Abstract Number: 1814 • ACR Convergence 2024
Understanding Monocyte Derived Macrophages in the Skin of SSc Patients Through Single Cell Analysis of Blister Fluid Immune Cell Populations
Background/Purpose: Cells of the monocyte-macrophages (Mφ) are key players in the pathogenesis of systemic sclerosis (SSc), contributing to inflammation and fibrosis. To advance beyond the…Abstract Number: 1867 • ACR Convergence 2024
RNA Polymerase III Specific CD8+ T Cells at the Interface Between Scleroderma and Cancer
Background/Purpose: Increasing evidence suggests an immunologic link between cancer and autoimmunity. Systemic sclerosis (SSc), offers a unique opportunity to study the evolution of naturally occurring…Abstract Number: 2456 • ACR Convergence 2024
High Levels of Circulating IFNα Are Associated with Increased Mortality in Patients with Diffuse Cutaneous Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is a connective tissue disease characterized by skin and organ fibrosis, autoimmunity, and vasculopathy. Although its pathophysiology remains unclear, recent data…Abstract Number: 2646 • ACR Convergence 2024
Rare Variants in the IL1RAP Gene Implicate the IL-1 Signaling Pathway in the Pathogenesis of Systemic Sclerosis in African and European Ancestries
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by systemic inflammation and fibrosis. Interleukin-1 receptor accessory protein (IL1RAP) is a co-receptor for the Interleukin-1…Abstract Number: 0688 • ACR Convergence 2024
Mycophenolate Mofetil Treatment in Limited Cutaneous Systemic Sclerosis Reduces the Risk of Vascular Complication Leading to Treatment Escalation: Emulation of a Target Trial Using Time-dependent Propensity Score-matching
Background/Purpose: The prescription of Mycophenolate Mofetil (MMF) represents the primary treatment for interstitial lung disease (ILD) associated with systemic sclerosis (SSc) and is an option…Abstract Number: 0853 • ACR Convergence 2024
Lung Ultrasound in Rheumatoid Arthritis and Systemic Sclerosis: Accuracy of B-line and Pleural Assessment
Background/Purpose: Interstitial lung disease (ILD) is a frequent and severe complication of rheumatoid arthritis (RA) and systemic sclerosis (SSc). Although computerized tomography (CT) is considered…
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