Abstract Number: 029 • 2023 Pediatric Rheumatology Symposium
Cardiac Magnetic Resonance Imaging in Children with Systemic Lupus Erythematosus and Scleroderma Spectrum Disorders: A Single Center Experience
Background/Purpose: Cardiac disease is a major cause of morbidity and mortality in children with SLE and SSc. Various studies have demonstrated an association between duration…Abstract Number: 086 • 2023 Pediatric Rheumatology Symposium
Systemic Sclerosis Overlap Syndrome: A Case Series from a Single Large Pediatric Center
Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare but potentially life-threatening autoimmune condition with features including immune, fibrotic, and vascular manifestations affecting the skin and…Abstract Number: 098 • 2023 Pediatric Rheumatology Symposium
Characterization of Pulmonary Nodules in Juvenile-onset Systemic Sclerosis: A Single Center Case-Series
Background/Purpose: Given the high prevalence of intestinal lung disease (ILD) in systemic sclerosis (SSc) and the low sensitivity of pulmonary function testing for diagnosing ILD,…Abstract Number: 116 • 2023 Pediatric Rheumatology Symposium
Clinical Significance of Anti-Scl-70 Antibodies in Pediatric Lupus Patients: A Single Center Cohort
Background/Purpose: Production of autoantibodies is a hallmark of SLE, with ANAs as a required diagnostic feature and anti-dsDNA and anti-Smith antibodies being disease-specific. Anti-Scl-70 antibodies…Abstract Number: 123 • 2023 Pediatric Rheumatology Symposium
Development of Specific Classification Criteria for Juvenile System Sclerosis Patients: A Scoping Review
Background/Purpose: Juvenile systemic sclerosis (jSSc) is associated with one of the highest morbidity and mortality rate in pediatric rheumatology, yet care recommendations are based upon…Abstract Number: 1052 • ACR Convergence 2022
Short-term Safety of COVID-19 Vaccination in Systemic Sclerosis Patients: Report from a Global Patient-Reported E-survey
Background/Purpose: Dedicated data on COVID-19 vaccine-related adverse events (ADEs) in patients with systemic sclerosis (SSc) is limited. Therefore, we aimed to compare all patient-reported ADEs…Abstract Number: 1181 • ACR Convergence 2022
Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…Abstract Number: 1622 • ACR Convergence 2022
TNF Receptor 1 Drives Murine Pulmonary Arterial Hypertension and Is Characterized by Loss of Capillary Endothelial Cells and Pericytes, Smooth Muscle Cell Proliferation, and Alterations in Fibroblast Phenotype
Background/Purpose: We previously demonstrated that TNF-transgenic (TNF-Tg) mice have findings consistent with connective-tissue disease associated pulmonary arterial hypertension (CTD-PAH), and that this pathology is mediated…Abstract Number: 2171 • ACR Convergence 2022
Serum IFN Score Predicts Long Term Outcome in Limited Cutaneous Systemic Sclerosis
Background/Purpose: Type-I Interferon pathway activation has been associated with severity and progression of diffuse cutaneous SSc (dcSSc). The role of type-I Interferons (IFNs) in limited…Abstract Number: 1054 • ACR Convergence 2022
Distinct Differences in Complement Perturbation Between Scleroderma-related Interstitial Lung Disease with and Without Emphysema
Background/Purpose: Aberrant complement activation is associated with autoimmune diseases including systemic sclerosis (SSc). As a leading cause of death in SSc, interstitial lung disease (ILD)…Abstract Number: 1183 • ACR Convergence 2022
Functionally Distinct Fibroblast Populations in Systemic Sclerosis Skin Revealed by Explant Tissue Characterization and Single-cell RNAseq
Background/Purpose: Traditional studies of skin fibroblasts in SSc focus on the early "migratory" fibroblast population derived from explant culture of skin biopsies. Single-cell RNAseq has…Abstract Number: 1657 • ACR Convergence 2022
Extreme Phenotype Approach Identifies Rare Variants in Systemic Sclerosis and Dermatomyositis Patients with Severe Calcinosis
Background/Purpose: Calcinosis, deposition of insoluble calcium salts in skin and subcutaneous tissues, affects up to 40% of systemic sclerosis (SSc) patients, and up to 20%…Abstract Number: 2173 • ACR Convergence 2022
Rheumatology Telemedicine Consults: Impact of Demographic Variability on Patient Perception in Systemic Sclerosis versus Rheumatoid Arthritis Pts
Background/Purpose: In the era of emerging need to utilize remote technology-based health care services due to the COVID-19 pandemic, we wanted to evaluate patient satisfaction…Abstract Number: 1055 • ACR Convergence 2022
Associations of Potential Risk Factors with Severe Outcomes in Scleroderma Renal Crisis: Results from a Single Center Study
Background/Purpose: Scleroderma Renal Crisis (SRC) is a severe and life-threatening complication of Systemic Sclerosis (SSc). Given the rare nature of SSc, it is challenging to…Abstract Number: 1184 • ACR Convergence 2022
Intracellular Calcium Signals Regulate the Pro-fibrotic Phenotype of Scleroderma Fibroblasts via Calcium/calmodulin-dependent Kinase II
Background/Purpose: We performed an in-depth analysis of the involvement of histone reader bromodomain extra-terminal proteins (BETs) in scleroderma (SSc) fibrosis and showed that JQ1, a…
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