Abstract 17650: Myocardial Fibrosis in Systemic Sclerosis, Progression and Response to Therapy Measured by Cardiovascular Magnetic Resonance

Introduction: Myocardial fibrosis is seen in half of Systemic Sclerosis (SSc) patients, and is associated with an increased risk of cardiac death. Extracellular volume fraction (ECV), an index of myocardial fibrosis measured by cardiovascular magnetic resonance imaging (CMR), has been shown to correlate with SSc disease severity measured by the modified Rodnan Skin Score (mRSS). Hypothesis: We hypothesized that ECV can quantify progression of myocardial fibrosis and response to therapy in SSc patients. Methods: We identified 30 pairs of CMR exams performed in 21 consecutive SSc patients who were evaluated for hematopoietic stem cell transplantation (HSCT). Scan pairs in which HSCT was performed between the first and second CMR were considered HSCT(+), while those without HSCT prior to second CMR were HSCT(-). Results: For all 21 SSc patients (age 43±11, 86% female), ECV was 28.9±2.8% and mRSS was 16.6±9.8 at baseline. Absolute change in ECV was +0.1±3.0% over 1.2±0.8 years in the entire cohort. ECV increa...