Autoimmune diseases are a group of heterogeneous disorders equally characterized by the same pathogenetic mechanism: an immunological reaction against self antigens promoted by antibodies, immuno-complex formation, and self-reactive T lymphocytes. Autoimmune diseases may be separated into organ-restricted diseases and systemic ones. The damage of single organs produced by antibodies focused against specific cellular antigens characterizes the first group of diseases, whereas the latter are produced by a systemic inflammatory process initiated by inappropriate and excess immune activation that leads to immuno-complex formation and deposition onto sensitive tissues. Since connective and vascular tissue are principally damaged in these disorders, systemic autoimmune diseases are more commonly known as "connective tissue diseases" (CTD) and include: systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, Sjogren syndrome, and others. Although they are considered as different from a pathogenetic point of view, they overlap in many aspects, such as general symptoms as fever and fatigue, chronical ongoing, steroid therapy. As patients suffering from CTD are predominantly young women between the ages of 20 and 40 years, which is the period of the highest childbearing potential, particular interest must be regarded to the impact that these diseases and their therapies have on pregnancy and, conversely, the effect of pregnancy on these disorders, which may have long-lasting implications for mothers and neonates. Adverse fetal outcomes, maternal disease flares, and drug potential teratogenic risk are the main reasons why women suffering from CTD and who are pregnant or intend to become pregnant are considered a high-risk population. These patients require integrated, interdisciplinary care, addressing every aspect of rheumatology, obstetrics, and neonatology to reduce maternal, fetal, and neonatal complications.