Adrenal autoimmunity: results and developments.

Autoimmune Addison's disease (autoimmune adrenalitis) often occurs in autoimmune polyendocrinopathy syndromes APS1 (APECED) and APS2. Although the genetic background and etiology of the two syndromes is remarkably different, they both result in a similar autoimmune destruction of the adrenal cortex. Recently, the defective gene in APS1, AIRE (autoimmune regulator) was identified, whereas in APS2, the major genetic factor remains to be found in the human major histocompatibility complex haplotype (HLA) region. In addition to the genetic factors, the recent findings in genetics and immunity leading to the pathogenesis of adrenal autoimmunity in polyendocrinopathy syndromes are discussed.