Grant: Doctoral Research Award: Canada Graduate Scholarships

Systemic sclerosis (SSc) is a rare immune disorder that affects mostly young and middle-aged women, resulting in reduced quality of life and early death. SSc is characterized by the thickening and tightening of the skin, connective tissue, and internal organs. The thickening and tightening of tissues is referred to as scarring or fibrosis, caused by excessive collagen production. Patients living with SSc can also develop lung fibrosis, which leads to breathing difficulties and death within 10 years of diagnosis for 40% of patients. How SSc occurs is not well understood, and currently, no treatments are available for patients with SSc, especially those that develop lung fibrosis. The main cells involved in fibrosis in the body are fibrocytes, fibroblasts, and myofibroblasts. In SSc patients, it is understood that excess production of the growth factor, transforming growth factor-beta (TGF-beta) may lead to uncontrolled release of collagen leading to fibrosis. The goal of this study is to use fibroblasts in a model of the skin and lung, to assess differences in the cells from SSc patients with and without lung fibrosis compared to controls. We will focus on the TGF-beta signaling pathway and determine if repurposing of drugs that can change the activation of TGF-beta receptor could provide new treatments for skin and lung fibrosis, to improve the lives and outcomes of SSc patients.