The effect of long acting glucocorticoids on menstrual abnormalities in patients with virilizing congenital adrenal hyperplasia.

Amenorrhea or oligomenorrhea occurs in more than 25% of young women with virilizing congenital adrenal hyperplasia due to 21-hydroxylase deficiency who are treated with conventional glucocorticoid therapy. In four such patients, we studied the variation in plasma concentration of ACTH, 17-hydroxyprogesterone (17-OHP), estradiol (E2), estrone, testosterone (T), dehydroepiandrosterone and its sulfate, androstenedione, and progesterone before and at frequent intervals after the administration of the patients' usual dose of oral glucocorticoids. We found that plasma ACTH, 17-OHP, and T were usually increased above the normal range early in the morning before the first daily dose of medication; plasma dehydroepiandrosterone and its sulfate were decreased below the normal range for the patient's pubertal stage; and androstenedione, estrone, and E2 were within the normal range. Two of the four patients had low basal plasma LH and impaired LH response to LRF. Administration of a long acting glucocorticoid (dexamethasone) to three patients, and methylprednisolone to one, resulted in continuous suppression of T into the normal range in all patients, in spite of continued abnormally increased concentration of ACTH and 17-OHP in two patients. In three patients, E2 concentrations were increased when a long acting glucocorticoid was given. In all four patients, menses began or became more regular during treatment with the long acting glucocorticoid. We conclude that a long acting glucocorticoid is useful in the management of menstrual abnormalities in adolescent patients with congenital adrenal hyperplasia who have attained their adult height, and that monitoring the concentration of serum T in them is a valuable but not infallible procedure for assessing the effectiveness of therapy.