Targeted therapies based on the underlying mechanism of disease control hyperinflammation and immune dysregulation, such as abatacept in CTLA-4 and LRBA deficiency, tocilizumab in STAT3-GOF, and sirolimus in activated phosphoinositide 3-kinase d syndrome (APDS) [12]. This evidence concerns the gene CTLA4 and activated PI3K-delta syndrome.