Polyglandular autoimmune syndromes.
Dysfunction of multiple endocrine glands may develop as the result of hypopituitarism, various infiltrative disorders, or an organ-specific autoimmune mechanism. When dysfunction of two or more endocrine glands occurs in association with circulating organ-specific antibodies directed against the involved glands, the term polyglandular autoimmune syndrome is applied. Characteristics of polyglandular autoimmunity include specific patterns of disease association and frequently a family history of similar involvement. The principal endocrine components of these syndromes are adrenal insufficiency, autoimmune thyroid disease, insulin-dependent diabetes mellitus, and premature gonadal failure. In addition, primary hypoparathyroidism is a key feature of one form of polyglandular autoimmunity that occurs in children. Several nonendocrine organ-specific autoimmune disorders are also associated with polyglandular autoimmunity, of which pernicious anemia is the most frequent. The underlying abnormality responsible for polyglandular autoimmunity is most likely a defect in T suppressor cell function, but there is evidence that aberrant expression of HLA DR antigens also plays an important role in the pathogenesis of these disorders.
D000224:Addison Disease / Q000150:complications; D000293:Adolescent; D000752:Anemia, Pernicious / Q000150:complications; D001327:Autoimmune Diseases / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000209:etiology; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000150:complications; D004700:Endocrine System Diseases / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000209:etiology; D005260:Female; D005757:Gastritis, Atrophic / Q000150:complications; D006680:HLA Antigens / Q000235:genetics; D006801:Humans; D007111:Immunity, Cellular; D008297:Male; D013577:Syndrome; D013959:Thyroid Diseases / Q000150:complications
D006680:HLA Antigens