For example, this update includes the findings that bi-allelic mutations in ZNF341 [19, 20], IL6ST (encoding gp130, a common component of the receptors for IL-6, IL-11, IL-27, LIF, OSM, CNTF) [21, 22], or IL6R [23, 24] all cause conditions that resemble autosomal dominant hyper-IgE syndrome due to dominant negative mutations in STAT3 [15]. Here, IL6ST is linked to Autosomal dominant hyper-IgE syndrome.