【Abstract】 Systemic sclerosis (SS) is a common connective tissue disease, which can lead to fibrosis of multiorgans. Interstitial lung disease (ILD) is a significant contributor to mortality among systemic sclerosis patients. Recently, the etiology of systemic sclerosisrelated interstitial lung disease (SScILD) is still unknown, including a variety of signal pathways, cytokines, immune cells and epigenetic regulars. The treatments of this disease contain conventional immunosuppressive agents, biological agents, intravenous immunoglobulin, hematopoietic stem cell transplantation and lung transplant. This article reviews the recent advances regarding the pathogenesis and treatments of systemic sclerosisrelated interstitial lung disease.