Adrenal disorders are relatively rare in pregnancy, but a timely diagnosis and proper treatment are critical because these disorders can cause significant maternal and foetal morbidity and mortality. Making the diagnosis of adrenal disorders in pregnancy is challenging as symptoms associated with pregnancy are also seen in adrenal diseases. In addition, pregnancy is marked by several endocrine changes, including activation of the renin-angiotensin-aldosterone system and the hypothalamic-pituitary-adrenal axis.
Addison’s disease is very rare in pregnancy, and the exact prevalence is unknown. Diagnosis may be missed in the first trimester as many clinical symptoms can occur in normal pregnancies such as fatigue, hyperemesis and weight loss. Appropriately treated patients can expect to have uneventful pregnancies of normal duration and without foetal complications. However, adrenal insufficiency during pregnancy is associated with a high incidence of serious foetal and maternal complications, such as suboptimal birth weight, intrauterine growth retardation, preterm delivery and postpartum adrenal crises, if the disorder is not recognized and adequately treated.
Congenital adrenal hyperplasia (CAH) refers to a group of inherited autosomal recessive disorders of adrenal steroid biosynthesis, resulting in altered cortisol and aldosterone secretion. 21-hydroxylase deficiency (21-OHD) accounts for 95% of all affected patients. Traditionally, reduced fertility and pregnancy rates have been reported in women with classic CAH, whereas fertility is only mildly reduced in the non-classic form. However, without glucocorticoid treatment, an increased miscarriage rate has been reported. Progress in female genitalia reconstructive surgery, individualized hormonal therapies, psychosexual evaluation, and assisted reproductive technology have improved fertility and pregnancy outcomes in women with classic CAH. Successful gestational management in CAH patients requires the close coordination of care between endocrinologists and obstetricians.