Polyendocrinopathy

Abstract

The occurrence of more than one endocrine disease in the same patient has intrigued first physicians and then endocrinologists, immunologists and geneticists. To paraphrase Oscar Wilde, to have one disease is permissible, to have two looks like immunological unruliness. As polyendocrinopathy is immunological in origin, the varieties of multiple endocrine adenomatosis will not be considered here. The overlap of organ-specific autoimmune diseases has been recognized for many years and eponyms such as Schmidt’s syndrome have been applied to particular combinations. The target glands affected include pancreatic islets, thyroid, adrenals and gonads and also importantly the parietal cells of the stomach. The particular association of thyroid, gastric, adrenal and pancreatic autoimmune phenomena with either overt disease or the presence of antibodies is common and has been designated type II autoimmune polyglandular syndrome (Winter and Maclaren 1987); it has a strong association with HLA DR3 or DR4. The form of diabetes is insulin dependent, type I, and indeed has been termed type IB (Bottazzo et al. 1974), the form with a female preponderance, later onset and persistence of islet cell antibodies.