Adrenal autoimmunity: Results and developments

Autoimmune Addison's disease (autoimmune adrenalitis) often occurs in autoi mmune polyendocrinopathy syndromes APS1 (APECED) and APS2. Although the gen etic 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 iden tified, whereas in APS2, the major genetic factor remains to be found in th e human major histocompatibility complex haplotype (HLA) region. In additio n to the genetic factors, the recent findings in genetics and immunity lead ing to the pathogenesis of adrenal autoimmunity in polyendocrinopathy syndr omes are discussed.