Hirsutism in Nonclassic Congenital Adrenal Hyperplasia (21-Hydroxylase Deficiency) When Antiandrogen Therapy Has Not Improved the Ferriman–Gallwey Score

This protocol addresses women with hirsutism in the setting of nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency (NCCAH) who have not achieved adequate improvement despite a prior antiandrogen-based regimen.

Clinical scenario Women with hirsutism and underlying nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. The management approach in this population follows the same principles as for hirsutism in other hyperandrogenic conditions.

Prior treatment — escalation trigger The previous step involved adding an antiandrogen (spironolactone, finasteride, or cyproterone acetate) to an oral contraceptive. Escalation to this protocol is indicated when that regimen produces a suboptimal response — specifically, a failure to achieve a meaningful reduction in the Ferriman–Gallwey hirsutism score.

Next-step approach (partial overview) For women who have a suboptimal response to oral contraceptives and/or antiandrogens, or cannot tolerate them, the structured protocol involves a glucocorticoid-based strategy. The complete regimen — including agent selection, dosing guidance, and monitoring — is outlined in the full protocol.

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References

Our approach to treating hirsutism in women with NCCAH is the same as for women with PCOS.

We only suggest glucocorticoids for the management of hirsutism in women who have a suboptimal response to OCs and/or antiandrogens, or cannot tolerate them.

For hirsutism, we use prednisone 4 to 6 mg daily or dexamethasone 0.25 mg/d.

DOI: 10.1210/jc.2018-00241

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