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Cushing's syndrome in pregnancy

Author
Lynnette K Nieman, MD
Section Editors
André Lacroix, MD
Charles J Lockwood, MD, MHCM
Deputy Editor
Kathryn A Martin, MD

INTRODUCTION

Cushing's syndrome (CS) is rarely associated with pregnancy, as hypercortisolism typically results in anovulatory infertility.

This topic will review issues related to CS in pregnancy. The clinical manifestations, diagnosis, and treatment of CS in nonpregnant patients are discussed separately. (See "Epidemiology and clinical manifestations of Cushing's syndrome" and "Establishing the diagnosis of Cushing's syndrome" and "Overview of the treatment of Cushing's syndrome".)

OVERVIEW

Frequency — Cushing's syndrome (CS) is associated with a high prevalence (up to 75 percent) of ovulatory disturbances induced by cortisol excess [1-3]. As a result, women with untreated CS rarely become pregnant [2]. However, there are over 140 reported cases of CS in pregnancy, most of which were associated with important maternal and fetal complications.

Normal HPA axis changes — Normal pregnancy is associated with changes in the maternal hypothalamic-pituitary axis (including increased production of cortisol-binding globulin [CBG]); increased concentrations of serum, salivary, and urinary free cortisol; and lack of suppression of serum cortisol after dexamethasone. In addition, the placenta produces corticotropin (ACTH) and corticotropin-releasing hormone (CRH). Thus, the diagnosis of CS during pregnancy is more challenging than in the nonpregnant state.

Serum total and free cortisol concentrations and urinary cortisol excretion are increased in pregnant women, but the diurnal rhythm in serum cortisol is maintained and urinary 17-OH corticosteroid excretion is normal [4]. One study in normal women reported that plasma cortisol values in pregnancy increased from 14.9±3.4 ug/dL at 12 weeks (411±94 nmol/L) to 35.2±9.0 ug/dL (971±248 nmol/L) at 26 weeks' gestation and changed minimally thereafter. This same study found a two to threefold increase in urinary free cortisol (UFC) in the second and third trimesters [5].

              

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Literature review current through: Jun 2015. | This topic last updated: Apr 15, 2015.
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