Sitat:
After the appearance of antibodies to the adrenal cortex and/or to 21-hydroxylase (21-OHA), the first evidence of adrenal insufficiency is usually an increase in PRA after patients have been recumbent for more than 0.5 h. The raised renin level is due to a failing zona glomerulosa with salt loss, with low-normal or low plasma aldosterone concentrations (27). Zona fasciculata dysfunction can become evident months to years later, first by raised afternoon serum ACTH levels, then by decreasing serum cortisol responses to (ACTH) stimulation, and finally by decreasing basal serum cortisol concentrations and the appearance of symptoms (Fig. 5). Thus, these autoantibodies are useful markers for the prediction of the development of Addison’s disease, particularly so for children (28). There are two types of antibodies detected by microscopic immunofluorescence: AAs reacting with only the adrenal cortex and steroidal cell antibodies (SCAs) that react with all steroid hormone-producing cells. One autoantigen is involved in reactions of AA is the protein of P450 21-OH enzyme, with epitopes in the central segment of the enzyme and the C-terminal portion (29, 30). Component antigens for SCA have been recognized to be other P450 enzymes, 17-{alpha}-hydroxylase (17-OH) (31) and SCC enzyme (32). Almost all patients with both Addison’s disease and gonadal failure have positive 17-OH and SCC antibodies and APS-1.
