AIRE and Autoimmunity: The likelihood of developing IFN-I autoimmunity follows a spectrum from ~100% in AIRE deficiency, to various, but lower, frequencies in other genetic disorders that affect the development and function of the thymus, and as also reflected by dominant AIRE mutations Patients with systemic autoimmune disorders probably need additional environmental triggers, such as infections, drugs or vaccines, to develop IFN-I autoimmunity.