IL18 and hyperinsulinemic hypoglycemia, familial, 4: Future targeted therapy to aid remission of autoinflammation in XIAP and NLCR4-related disease, which is driven by high IL-18 levels, may come in the form of tadekinig alfa, which mimics endogenous neutralizing IL-18 binding protein; whilst its use in XIAP deficiency has been reported in a single patient, further evidence on efficacy may come from a randomized trial (NCT03113760), which has completed recruitment and is ending soon (44, 155).