Pneumatosis intestinalis (PI) is an uncommon condition characterized by the presence of gas within the wall of the gastrointestinal tract. PI is a physical or imaging finding that is the result of an underlying pathological process or a disease. This finding may present in diverse conditions, such as obstructive pulmonary disease and intestinal disease, including obstruction, inflammation, or ischemia. PI has also been reported in different autoimmune conditions, especially in systemic sclerosis and rarely with systemic lupus erythematosus. In this report we present PI occurring in three patients with different autoimmune conditions and review the literature.