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

Treatment of Nonclassic Congenital Adrenal Hyperplasia (21-Hydroxylase Deficiency) in Adult Women with Infertility or Hyperandrogenism

This protocol addresses the management of nonclassic congenital adrenal hyperplasia (NCCAH) due to 21-hydroxylase deficiency in adult women aged 18 years and older who present with patient-important hyperandrogenism or infertility.

Clinical scenario

Adult women (≥18 years) with nonclassic congenital adrenal hyperplasia (21-hydroxylase deficiency) who have patient-important hyperandrogenism or infertility — including those experiencing a delay in conception.

Treatment approach

Glucocorticoid treatment is recommended for this population, with the protocol specifying distinct therapeutic goals and a tailored approach for women with infertility or a delay in conception. Full regimen, specific targets, and clinical algorithm are in the complete protocol below.

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References

  1. In adult women with NCCAH who also have patient-important hyperandrogenism or infertility we suggest GC treatment.
  2. For women who experience a delay in conception, GC treatment should aim to achieve a follicular-phase progesterone level <0.6 ng/mL (2 nmol/L), a much tighter control than for women not attempting to conceive, often requiring a bedtime dose of prednisolone.
DOI: 10.1210/jc.2018-01865
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