Evaluation of digital ischemia in scleroderma with multiparametric magnetic resonance imaging

Systemic sclerosis (SSc) or scleroderma is a chronic multisystem autoimmune disorder. The underlying pathophysiology includes innate/adaptive immune system abnormalities, endothelial injury with small vessel vasculopathy, and fibroblast activation with subsequent fibrosis. Digital ischemia/digital loss is a feared complication of SSc. Prospective studies report that an estimated 50% of patients with SSc experience digital ulcerations over 5 years of follow-up, and 20% experience finger amputation secondary to ischemia. The currently available clinical tools for the assessment and monitoring of the underlying pathophysiology (vasculopathy, tissue ischemia and fibrosis) are only semi-quantitative. The dearth of quantitative tools to evaluate potential treatments has resulted in patients with SSc enduring significant morbidity.We propose to use multiparametric magnetic resonance imaging (MRI) in patients with SSc for a comprehensive quantitative assessment of vasculopathy, tissue hypoxia, and fibrosis in the hands, which contributes the most to patient disability in SSc. The provided dataset includes MRI of hands of healthy participants (n=5 studies) that contained the following acquisitions: scout images, T1 mapping for fibrosis, T2*BOLD images for hypoxia and time-of-flight MR angiography studies to evaluate vasculopathy. The overall goal of this work is to provide new noninvasive imaging biomarkers to inform prevention and treatment interventions and to monitor effectiveness of therapy for digital ischemia.