Cushing's disease
ICD-10 E24.0 · ICD-11 5A70.0

Treatment of Cushing's Disease in Pregnancy When Transsphenoidal Surgery Has Not Achieved Remission

This protocol addresses the management of Cushing's disease specifically in pregnant women or women desiring pregnancy, in whom transsphenoidal pituitary surgery — the established first-line definitive treatment — did not produce remission.

Clinical Scenario

Women who are pregnant, or who wish to become pregnant, with active Cushing's disease represent a distinct sub-population requiring tailored management. No medications for Cushing's disease are currently approved for use in pregnancy.

Previous Treatment — Remission Not Achieved

Prior therapy: Transsphenoidal pituitary surgery.
Target not met: Remission, defined as postoperative serum cortisol below 55 nmol/L (2 μg/dL), was not achieved — triggering escalation to this next line of care.

Next-Line Approach (Partial Overview)

After surgical non-remission, oral medical therapy is among the management options considered to control cortisol in this setting. The approach targets a pregnancy-adjusted cortisol goal, accounting for the physiologically elevated cortisol levels that occur during pregnancy. The full structured protocol details the specific agents, selection criteria, monitoring requirements, and the precise cortisol target.

References

DOI: 10.1016/S2213-8587(21)00235-7

  1. In pregnant women or those desiring pregnancy, consider cabergoline or metyrapone, although no CD medications are approved for use in pregnancy.
  2. Although none of these drugs is specifically approved for use in pregnancy, metyrapone may be considered with precautions in selected women who are pregnant.
  3. In such cases, given the higher normal cortisol levels during pregnancy, a higher cut-off target for cortisol, such as 1.5 × ULN, is used.
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