title: Autoimmune Addison's disease.
abstract: Primary adrenocortical insufficiency, or Addison's disease (AD), results from an adrenal cortex hypofunction/dysfunction with a deficient production of glucocorticoids, mineralocorticoids and androgens, and with high levels of both ACTH and plasma renin activity. The prevalence of AD is 110-144 cases per million population in the developed countries. Autoimmune AD is the most frequent etiological form in adult patients, accounting for about 80% of cases, followed by post-tuberculosis AD in 10-15%, the remaining 5% being cases are due to vascular, neoplastic or rare genetic forms. Congenital adrenal hyperplasia is the most frequent form of AD in children and accounts for 72% of cases, whereas autoimmune AD is seen in around 10-15% of cases. The markers of autoimmune AD are adrenal cortex (ACA) or 21-hydroxylase autoantibodies (21-OHAbs) and they are present at diagnosis in more than 90% of cases. In autoimmune AD, the adrenal cortex is infiltrated by lymphocytes and plasma cells and the glands are sclerotic and reduced in volume. Autoimmune AD occurs mainly in middle-aged females, alone or associated with other (clinical, subclinical or potential) autoimmune diseases, giving rise to various forms of autoimmune polyglandular syndrome (type 1, 2 or 4). Replacement therapy with gluco-and mineralocorticoids is life-saving for patients with chronic adrenal insufficiency.
authors.forename: Corrado
authors.initials: C
authors.lastname: Betterle
authors.forename: Luca
authors.initials: L
authors.lastname: Morlin
mesh_terms: D000224:Addison Disease / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D000328:Adult; D000818:Animals; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D020249:Hormone Replacement Therapy / Q000379:methods; D006801:Humans; D008901:Mineralocorticoids / Q000627:therapeutic use; D008954:Models, Biological
chemical_list: D005938:Glucocorticoids; D008901:Mineralocorticoids