Systemic sclerosis (SSc) is a rare autoimmune disease characterized by widespread microvasculopathy, inflammation, and fibrosis of the skin and internal organs. The involvement of the
gastrointestinal tract is associated with a wide variety of symptoms and affects circa 90% of patients during the course of the disease. The gastrointestinal microbiota contains trillions of
microbial cells and has been found to contribute to both local and systemic homeostasis. In both health and disease, a dynamic interrelationship between gut microbiome activity and the host immune system has been identified. Gastrointestinal dysbiosis has been described as having an important role in obesity, diabetes mellitus, liver disease, cardiovascular and neuropsychiatric disorders, neoplasia, as well as autoimmunity. Recent scientific data indicates a notable role of dysbiosis in the pathogenesis of SSc-related digestive involvement together with various other clinical manifestations. The present review aims to summarize the recent findings regarding digestive dysbiosis as well as the relationship between gastrointestinal microbiota and certain features of SSc.