Congenital adrenal hyperplasia due to 21-hydroxylase deficiency
ICD-10 E25.0 · ICD-11 5A71.01

Nonclassic CAH in Adult Women with Infertility or Hyperandrogenism After Glucocorticoid Treatment Has Not Achieved Conception Targets

Clinical Scenario

This protocol is for adult women (18 years and older) with nonclassic congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase deficiency who have patient-important hyperandrogenism or infertility.

Escalation Trigger — Prior Treatment Did Not Achieve Goals

The previous line of treatment used glucocorticoid therapy, with tighter control targeting a follicular-phase progesterone level below 0.6 ng/mL (2 nmol/L) for women trying to conceive. This protocol applies when that progesterone target has not been reached and the clinical goals of the prior line remain unmet.

Next-Step Approach (Partial Overview)

For women where hirsutism is the primary concern, the protocol introduces an oral hormonal therapy as an additional measure. The full agent selection, criteria, and clinical algorithm are detailed in the complete protocol.

Clinical Goal

The primary target of this treatment line is remission of hirsutism.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1210/jc.2018-01865

In adult women with NCCAH who also have patient-important hyperandrogenism or infertility we suggest GC treatment.

As in other androgenic disorders, an oral contraceptive with or without anti-androgens is likely the best approach for treating hirsutism in women with NCCAH.

View source ↗