Systemic sclerosis (SSc) is a rare, complex autoimmune disease characterized by vasculopathy and fibrosis of the skin and internal organs. Clinical manifestations of SSc are the result of the interplay between innate and adaptive immune systems, microvascular abnormalities with fibroproliferative vasculopathy of microcirculation, and fibroblast dysfunction with excessive collagen and matrix components accumulation in skin, blood vessels, and internal organs [1,2]. Currently, available treatments are mainly symptom-oriented [3], with heterogeneous clinical phenotypes largely dependent upon the involvement of lung, kidney, heart and gastrointestinal tract (GIT) [4].