Abstract Number: 818 • 2016 ACR/ARHP Annual Meeting
The Role of Endothelin 1 in Activation of Vascular Smooth Muscle Cells in Systemic Sclerosis; Increased Cell Proliferation and Resistance to Apoptosis Mediated By Endotehlin B Receptors
Background/Purpose: is overexpressed in SSc as illustrated by elevated circulating levels and increased tissue expression in involved organs. We previously reported vascular smooth muscle…Abstract Number: 819 • 2016 ACR/ARHP Annual Meeting
Impaired Adiponectin Signaling in SSc Contributes to Myofibroblast Differentiation and Organ Fibrosis
Background/Purpose: In systemic sclerosis (SSc) patients, skin fibrosis is accompanied by involution of dermal white adipose tissue (dWAT), a prominent source of adiponectin (APN). We…Abstract Number: 820 • 2016 ACR/ARHP Annual Meeting
EZH2 Modulates Angiogenesis and Fibrosis in Scleroderma
Background/Purpose: Scleroderma (SSc) is a complex disease that involves activation of the immune system, vascular complications, and tissue fibrosis. Although the pathogenesis of this disease…Abstract Number: 821 • 2016 ACR/ARHP Annual Meeting
IL-31 Is an Inflammatory Pro-Fibrotic Factor Elevated in a Subset of Scleroderma Patients with Severe Pruritus
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune rheumatic disease associated with fibroblast activation in the skin and visceral organs. In SSc, refractory pruritus is a…Abstract Number: 822 • 2016 ACR/ARHP Annual Meeting
Pulmonary Fibrosis in Mixed Connective Tissue Disease – Results from an Unselected Longitudinal Cohort
Background/Purpose: Mixed Connective Tissue Disease (MCTD) is a chronic, immune-mediated disorder defined by the combined presence of serum anti-ribonucleoprotein (RNP) antibodies and selected clinical features…Abstract Number: 823 • 2016 ACR/ARHP Annual Meeting
A Phase 2 Study of Pomalidomide (CC-4047) to Evaluate Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Effectiveness in Subjects with Systemic Sclerosis with Interstitial Lung Disease
Background/Purpose: Pomalidomide (POM) is an IMiD compound, structurally similar to thalidomide. POM binds to cereblon and facilitates Ikaros and Aiolos degradation, resulting in immunomodulation of…Abstract Number: 824 • 2016 ACR/ARHP Annual Meeting
Mycophenolate Versus Placebo for the Treatment of Systemic Sclerosis-Related Interstitial Lung Disease
Background/Purpose: Compared with placebo, treatment with cyclophosphamide (CYC) improved lung function and dyspnea in patients with systemic sclerosis-related interstitial lung disease (SSc-ILD).1 While treatment with…Abstract Number: 825 • 2016 ACR/ARHP Annual Meeting
KL-6 and Not CCL-18 Is a Predictor of Early Progression in Systemic Sclerosis Related Interstitial Lung Disease
Background/Purpose: Pneumoproteins are attractive biomarker candidates in systemic sclerosis (SSc) related interstitial lung disease (ILD) because they are easily obtainable and lung-specific. KL-6 and CCL-18…Abstract Number: 826 • 2016 ACR/ARHP Annual Meeting
Exercise Echocardiography Predicts Future Development of Pulmonary Hypertension in a High-Risk Cohort of Scleroderma Patients
Background/Purpose: Pulmonary hypertension (PH) is the leading cause of scleroderma related deaths and is often detected late in the disease course. Early identification of patients…Abstract Number: 827 • 2016 ACR/ARHP Annual Meeting
Severe Gastrointestinal Disease in Early Systemic Sclerosis Is Associated with an Increased Risk of Mortality
Background/Purpose: Studies of severe gastrointestinal (GI) disease in systemic sclerosis (SSc) are limited by small, selected samples composed largely of subjects with prevalent disease. We…Abstract Number: 828 • 2016 ACR/ARHP Annual Meeting
Survival and Health-Related Quality of Life in Incident Systemic Sclerosis Related Pulmonary Arterial Hypertension: A Multicentre Australian Cohort Study
Background/Purpose: Pulmonary arterial hypertension (PAH) is the leading cause of systemic sclerosis (SSc) related mortality. We sought to determine survival, predictors of mortality, and health…Abstract Number: 829 • 2016 ACR/ARHP Annual Meeting
High Level of Chemokine CCL2 Is Associated with Lung Fibrosis Progression and Reduced Survival in Two Independent Systemic Sclerosis Cohorts
Background/Purpose: Markers for early identification of progressive interstitial lung disease (ILD) in systemic sclerosis (SSc) are in demand. The proto-typical inflammatory chemokine CCL2 has been linked…Abstract Number: 830 • 2016 ACR/ARHP Annual Meeting
Cardiopulmonary Disease Development in Anti-RNA Polymerase III Positive Systemic Sclerosis; Comparative Analyses from an Unselected, Prospective Patient Cohort
Background/Purpose: Extensive skin disease and renal crisis are hallmarks of anti-RNA polymerase III (RNAP) positive systemic sclerosis (SSc), while data on lung and heart involvement…Abstract Number: 831 • 2016 ACR/ARHP Annual Meeting
Immunosuppression May Prevent Interstitial Lung Disease in Systemic Sclerosis
Background/Purpose: Interstitial lung disease (ILD) is a leading cause of premature mortality in systemic sclerosis (SSc). Immunosuppression is used for treatment of established disease. However,…Abstract Number: 832 • 2016 ACR/ARHP Annual Meeting
Defining Skin Ulcers in Systemic Sclerosis: A Systematic Literature Review of Skin Ulcer Definitions and a Preliminary Consensus-Based New SSc Skin Ulcer Definition
Background/Purpose: Skin ulcers in SSc are a major clinical challenge and there are various un-validated definitions of skin ulcers utilized in SSc-related clinical trials. We…
