Maintenance glucocorticoid regimens for adults with classic congenital adrenal hyperplasia

Agent(s)	Typical dosing regimen (oral)
Preferred therapy
Hydrocortisone*	15 to 30 mg/day in 3 divided doses
Combined regimens^¶
Hydrocortisone and prednisolone	Hydrocortisone 10 to 25 mg/day in 2 or 3 divided doses Prednisolone 1 to 2 mg once daily at bedtime
Hydrocortisone and methylprednisolone	Hydrocortisone 10 to 25 mg/day in 2 or 3 divided doses Methylprednisolone 1 to 2 mg once daily at bedtime
Longer-acting agents
Prednisolone^Δ	4 to 6 mg/day in 2 divided doses
Methylprednisolone^Δ	4 to 6 mg/day in 2 divided doses
Prednisone (not preferred)^◊	5 to 7.5 mg/day in 2 divided doses
Dexamethasone (not preferred)^◊	0.25 to 1 mg once daily at bedtime

This table summarizes our suggested approach to glucocorticoid therapy in adults with classic CAH. In such individuals, the goals of therapy are both to provide glucocorticoid replacement and to attenuate the production of adrenal-derived steroids. Therefore, in individuals with larger body size or more severe CAH, the total daily glucocorticoid requirement may be higher than the typical dosing range. Most adults also require mineralocorticoid therapy with fludrocortisone. For additional details, refer to UpToDate topics on the treatment of classic CAH due to 21-hydroxylase deficiency in adults.

CAH: congenital adrenal hyperplasia; ACTH: corticotropin; TARTs: testicular adrenal rest tumors.

* With hydrocortisone therapy, the largest dose is taken in the morning upon waking, and progressively smaller doses are taken late morning/midday and late afternoon/early evening (eg, 15, 5, and 2.5 mg). The last dose should be taken no later than 4 to 6 hours before bedtime.

¶ Combination therapy is useful when adequate adrenal steroid suppression is not achieved with hydrocortisone alone. It entails typical cortisol replacement doses of hydrocortisone during the day and a very small dose of a longer-acting glucocorticoid at bedtime. The lowest dose of the longer-acting glucocorticoid that achieves adequate adrenal steroid suppression should be used. Combined regimens are very effective for suppressing ACTH and minimizing total glucocorticoid exposure. Prednisolone liquid (1 to 3 mg/mL) allows for finer and more precise dose adjustments than methylprednisolone tablets.

Δ For individuals with classic CAH who have difficulty with hydrocortisone dosing requirements, treatment with a longer-acting agent may improve treatment adherence. Of the longer-acting agents, prednisolone or methylprednisolone is preferred. These glucocorticoids are typically administered twice a day with a larger dose in the morning to replace the cortisol deficiency (eg, 3 to 5 mg) and a small dose at bedtime to attenuate the pre-dawn ACTH rise (eg, 1 to 2.5 mg).

◊ Prednisone or dexamethasone may be used if necessary due to cost or availability issues with preferred agents. Prednisone is generally not preferred, because it requires hepatic activation, which could lead to more variability in dosing requirements. Dexamethasone is generally not preferred due to adverse effects of long-term use and difficulty achieving treatment goals. Use should be reserved for specific indications (eg, TARTs) and should be of limited duration. Refer to UpToDate topic for discussion.

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Maintenance glucocorticoid regimens for adults with classic congenital adrenal hyperplasia