AIRE and autoimmune disease: APECED is a rare monogenic autoimmune disease (OMIM#, 240300) caused by loss-of-function AIRE mutations that impair central immune tolerance and result in the peripheral escape of self-reactive T lymphocytes, which infiltrate various endocrine (e.g., parathyroids, adrenals, gonads, thyroid, pancreas) and non-endocrine (e.g., enamel, stomach, small intestine, lungs, liver, salivary glands, kidneys, spleen, skin) organs and cause autoimmune tissue destruction.