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.
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 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 . 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 .
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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
- SUMMARY AND RECOMMENDATIONS