Accumulating studies have shown that long non-coding RNA (lncRNA) plays major roles in endothelial injury, inflammation, cancer, and autoimmune diseases. Systemic sclerosis (SSc) is an autoimmune disease with an unclear pathogenesis. lncRNA may be involved in the occurrence and development of SSc by regulating immune disorders, microvascular disease, and fibrosis, and may be a biomarker of SSc to predict occurrence, activity and progression of the disease. Therefore, lncRNA may be a novel therapeutic target of SSc.