Cyproterone AcetateVersusHydrocortisone Treatment in Late-Onset Adrenal Hyperplasia

Abstract

Thirty late-onset adrenal hyperplasia patients consulting for isolated hirsutism were randomly divided into two groups; group 1 (n = 16) was treated with hydrocortisone in order to suppress androgen adrenal secretion, and group 2 (n = 14) received cyproterone acetate (CPA) antiandrogen therapy to inhibit peripheral androgen activity. The clinical and hormonal effects of each type of treatment were evaluated. Before treatment, the clinical and hormonal profiles of the two patient groups did not differ significantly. Excellent clinical evolution in terms of the regression of hirsutism was observed in the CPA-treated patients (54% decrease in the clinical score in 1 yr), in contrast with the slight decrease in hirsutism (26%) after hydrocortisone treatment. In hydrocortisone-treated patients, plasma androgen decreased to normal levels: testosterone from 3.05 +/- 1.45 to 1.46 +/- 0.42 nmol/L and delta 4-androstenedione from 13.6 +/- 4.1 to 6.33 +/- 1.47 nmol/L. Conversely, in CPA-treated patients, only a slight decrease in testosterone from 2.98 +/- 1.98 to 2.29 +/- 0.64 nmol/L and in delta 4-androstenedione from 12.9 +/- 5.9 to 9.86 +/- 2.23 nmol/L was observed. This slight decrease in plasma androgens contrasts with the rapid clinical improvement after CPA. These results emphasize the importance of peripheral receptivity to androgens in the clinical expression of hyperandrogenism. Moreover, they indicate that peripheral antiandrogen therapy may be more appropriate in late-onset adrenal hyperplasia patients than conventional adrenal inhibition using cortisone therapy.

Treatment options for polycystic ovary syndrome

“…A trial of CPA versus hydrocortisone in patients with late-onset congenital adrenal hyperplasia showed a greater decrease in hirsutism scores with 1 year of CPA compared with hydrocortisone (54% vs 26%). These results occurred despite a greater reduction of androgens with glucocorticoids, highlighting the importance of peripheral receptivity to androgens 70. Overdosing can occur, leading to adrenal atrophy, weight gain, and decreased bone mineral density.…”

Management of adolescents with congenital adrenal hyperplasia

“…1,35,41 Some female patients might choose to continue glucocorticoid therapy if oral contraceptive pills, anti-androgens, or both are not tolerated or desired, or pronounced hyperandrogenism is present. 4,35 Additionally, glucocorticoid therapy might be indicated at specific times during adulthood for women with non-classic disease, for example as an effective treatment for infertility and recurrent miscarriages.…”

Endocrine evaluation of hirsutism

“…They suppress adrenal androgen production and control hirsutism, while maintaining ovulatory cycles. Trials comparing glucocorticoids to antiandrogens and OCPs in women with the nonclassic form of 21-hydroxylase deficiency (NCCAH) found glucocorticoids to be more effective in suppressing adrenal androgens, but less effective in controlling hirsutism (Spritzer et al, 1990). …”

“…The use of glucocorticoids in women with NCCAH is suggested for those who do not respond to or cannot tolerate OCPs or antiandrogens, or those who seek ovulation induction (Spritzer et al, 1990). …”