Treatment of Classic CAH (21-Hydroxylase Deficiency) in Adults (Age ≥18)
Classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency requires active management into and throughout adulthood. Adult patients (aged 18 years and older) have distinct hormonal treatment targets and long-term considerations that differ from those in childhood or adolescence.
Clinical Scenario
Classic CAH in the adult population involves ongoing adrenal insufficiency and androgen excess that must be managed together. Treatment choices in adults balance adequate hormone replacement against the real risks of over-treatment and its adverse effects.
Treatment Goals
The primary aim is to bring androstenedione and testosterone to at or near normal levels. In women who are trying to conceive, an additional, more stringent hormonal target applies. Importantly, the protocol recommends against fully suppressing endogenous adrenal steroid secretion — avoiding over-treatment is an explicit goal.
Treatment Approach — Partial Overview
Management in adults centres on daily glucocorticoid replacement, with the choice between a short-acting and a longer-acting agent an explicit consideration, along with mineralocorticoid supplementation where clinically indicated.
Agent selection, the full dosing structure, monitoring parameters, and special-situation guidance are detailed in the complete protocol →
References
DOI: 10.1210/jc.2018-01865
- In adults with classic CAH, we recommend using daily HC and/or long-acting GCs plus mineralocorticoids (MCs), as clinically indicated.
- At or near completion of growth, long-acting GCs may be administered (Table 3), although HC remains the preferred treatment.
- Most nonhypertensive adults with classic CAH benefit from continued fludrocortisone treatment.
- For women, androstenedione and testosterone are good parameters of disease control (202), but additional tests should be considered in the context of menstrual irregularity and signs of androgen excess.
- In adults with congenital adrenal hyperplasia, we recommend that clinicians do not completely suppress endogenous adrenal steroid secretion to prevent adverse effects of over treatment.
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