Cushing's syndrome in pregnancy
- Lynnette K Nieman, MD
Lynnette K Nieman, MD
- Section Editor — Adrenal Disease
- Senior Investigator
- Bethesda, MD
- Section Editors
- André Lacroix, MD
André Lacroix, MD
- Section Editor — Adrenal Disease
- Professor of Medicine
- University of Montreal, Quebec, Canada
- Charles J Lockwood, MD, MHCM
Charles J Lockwood, MD, MHCM
- Section Editor — Obstetrics
- Senior Vice President, USF Health
- Dean, Morsani College of Medicine
- Professor, Obstetrics and Gynecology
- University of South Florida
Cushing's syndrome (CS) is rarely associated with pregnancy as hypercortisolism typically results in anovulatory infertility. However, there are over 200 reported cases of CS in pregnancy.
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".)
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 . However, there are over 220 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-adrenal (HPA) axis, including increased production of cortisol-binding globulin (CBG); increased concentrations of serum, salivary, and urinary free cortisol (UFC); and lack of suppression of serum cortisol after dexamethasone. In addition, the placenta produces corticotropin (ACTH) and corticotropin-releasing hormone (CRH). Thus, the biochemical 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 bedtime nadir of serum cortisol is maintained and urinary 17-hydroxycorticosteroid excretion is normal . One study in normal women reported that plasma cortisol values in pregnancy increased from 14.9±3.4 mcg/dL at 12 weeks (411±94 nmol/L) to 35.2±9.0 mcg/dL (971±248 nmol/L) at 26 weeks gestation and changed minimally thereafter . This same study found a two to threefold increase in UFC in the second and third trimesters.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- Normal HPA axis changes
- Causes of Cushing's in pregnancy
- CLINICAL FEATURES
- Biochemical findings
- - Imaging
- Maternal and fetal complications
- Additional evaluation to determine cause
- Suggested approach
- - ACTH independent
- - ACTH dependent
- - Limitations of treatment
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS