FAMILIAL POLY-ENDOCRINOPATHY

Abstract

ABSTRACT Two family histories are presented. In the first (family W.) two siblings, aged 12 and 16, had idiopathic Addison's disease. The younger patient also had a goitre, possibly representing Hashimoto's thyroiditis. In the second family Graves' disease was present in three generations in direct succession. The oldest patient in addition had Addison's disease, diabetes mellitus and total alopecia. Organ-specific antibodies were looked for quite extensively in the serum of the patients and their relatives, with several positive findings, but without a consistent pattern. A review is given of the literature on familial poly-endocrinopathies and the probable auto-immune character of these dieases. The present cases constitute strong evidence for a genetic transmission of these diseases, and for a common basic aetiology. If this common basis is an auto-immuno aberration, it seems most reasonable, in view of the polyglandular involvement, to assume that the immune-system proper is at fault, rather than a defect in the individual organs.