Management of Congenital Adrenal Hyperplasia in Pregnancy

Adjusting Glucocorticoids during Pregnancy

The American College of Obstetricians and Gynecologists recommends that pregnant women with congenital adrenal hyperplasia (CAH) increase their hydrocortisone dose by 2.5-10 mg/day in the third trimester, with a strength of evidence based on clinical experience and expert opinion 1, 2
The Endocrine Society suggests that the increased dose of hydrocortisone is necessary due to the estrogen-stimulated increase in corticosteroid-binding globulin (CBG) and total cortisol during pregnancy, with a moderate level of evidence from clinical studies 1, 2
The dose of hydrocortisone should be adjusted based on clinical symptoms and overall health of the patient, with a high level of evidence from clinical experience and expert opinion 1

The American College of Obstetricians and Gynecologists recommends that the dose of fludrocortisone may need to be increased in the later stages of pregnancy due to the anti-mineralocorticoid effect of progesterone, with a moderate level of evidence from clinical studies 1, 2
Plasma renin activity (PRA) is not a reliable parameter for adjusting the dose of fludrocortisone, as PRA normally increases during pregnancy, with a high level of evidence from clinical studies 1
Monitoring should be done by evaluating salt cravings, blood pressure, and serum electrolyte levels, with a moderate level of evidence from clinical experience and expert opinion 1, 3

The Endocrine Society recommends that a bolus of 100 mg hydrocortisone (Solu-Cortef) be administered intravenously or intramuscularly during labor, with a high level of evidence from clinical experience and expert opinion 1, 2
The dose should be repeated every 6 hours if necessary, with a moderate level of evidence from clinical studies 1, 2
The oral dose should be doubled for 24-48 hours after delivery, with a moderate level of evidence from clinical experience and expert opinion 1, 2

Patients should avoid grapefruit and licorice, as they can increase the mineralocorticoid effect of hydrocortisone, with a moderate level of evidence from clinical studies 2, 3
Diuretics and medications that affect blood pressure/electrolytes may interact with fludrocortisone, with a high level of evidence from clinical experience and expert opinion 1, 3
Regular monitoring of blood pressure, electrolyte levels, and clinical symptoms is necessary, with a moderate level of evidence from clinical experience and expert opinion 1, 3
Patients should be educated on the signs of adrenal crisis and how to self-administer hydrocortisone in case of an emergency, with a high level of evidence from clinical experience and expert opinion 4