Adrenal Disorders in Pregnancy

The hypothalamic-pituitary-adrenal (HPA) axis and the renin-angiotensin system (RAS) are up-regulated during normal pregnancy. Pregnancy represents a state of relative hypercortisolism, resulting from the interaction of the maternal HPA axis and the fetal-placental unit. Consistent with a physiologic role, the RAS maintains normal sodium balance and volume homeostasis. In normal gestation, hypercortisolism and relative hyperaldosteronism are not usually clinically apparent. In contrast, adrenal disorders that do occur during pregnancy contribute to significant maternal and fetal morbidity. This article reviews the natural history, causes, diagnosis, and treatment of adrenal causes of Cushing’s syndrome, adrenocortical hypofunction, primary hyperaldosteronism, and the management of adrenal pheochromocytoma in pregnancy. Cushing’s syndrome in pregnancy The clinical presentation of Cushing’s syndrome in pregnancy is similar to that in the general population, except for the preservation of menses before conception. The cause, however, differs between the pregnant and nonpregnant state because adrenal causes of Cushing’s syndrome account for over 60% of 122 previous reports in pregnancy, in contrast with 15% in nonpregnant women [1,2]. Solitary adrenal adenomas are the most common cause, whereas adrenal carcinoma accounts for approximately 10% of cases [3,4]. Pheochromocytoma causing ectopic Cushing’s syndrome was reported on two occasions. Its rarity may be explained by the anovulation that usually accompanies severe hypercortisolism [3,5]. Primary pigmented