[X chromosome inactivation and systemic autoimmune diseases]

Women tend to develop more effective immunity against infections than men, but they are at higher risk for systemic autoimmune diseases. In both humans and mice, the number of X chromosomes is associated with an increased risk of autoimmune diseases, including rheumatic diseases such as systemic lupus erythematosus, Sjögren's syndrome, and systemic sclerosis. In this review, we present recent advances regarding the mechanisms associated with X-chromosome inactivation in the sex-specific predisposition to autoimmunity, emphasizing recent findings suggesting that dysregulation of gene expression linked to the inactive X chromosome may contribute to women's increased susceptibility to these diseases.