Immune tolerance breakdown in inborn errors of immunity: Paving the way to novel therapeutic approaches

Up to 25% of the patients with inborn errors of immunity (IEI) also exhibit immunodysregulatory features. The association of immune dysregulation and immunodeficiency may be explained by different mechanisms. The understanding of mechanisms underlying immune dysregulation in IEI has paved the way for the development of targeted treatments. In this review article, we will summarize the mechanisms of immune tolerance breakdown and the targeted therapeutic approaches to immune dysregulation in IEI. •Immunodysregulatory features may be observed in about 25% of patients with Inborn errors of immunity.•The association of immune dysregulation and immunodeficiency may be explained by different mechanisms.•The identification of the pathogenetic mechanisms has led to the development of precision treatments.