The “non-classical” form of APECED has been suggested to be characterized by variable autoimmune phenotypes, ranging from late-onset APECED to different combinations of autoimmune manifestations (APS-2), isolated organ-specific autoimmunity or autoantibodies, but no signs of autoimmune disease within individuals who harbor monoallelic AIRE mutations. The gene discussed is AIRE; the disease is autoimmune disease.