Adrenal Insufficiency Management Guidelines

Introduction to Adrenal Insufficiency

Patients with adrenal insufficiency require individualized glucocorticoid replacement therapy and must receive stress doses of hydrocortisone during periods of physiological stress, including the perioperative period, to prevent potentially life-threatening adrenal crisis 1, 2

Primary adrenal insufficiency results from diseases of the adrenal gland itself and involves deficiency of both cortisol and aldosterone 3, 4
Secondary adrenal insufficiency is caused by deficient ACTH secretion from the pituitary gland or deficient CRH from the hypothalamus, resulting in cortisol deficiency but preserved aldosterone production 3, 4
Glucocorticoid-induced (tertiary) adrenal insufficiency is the most common form, occurring in patients receiving supraphysiological doses of glucocorticoids for ≥1 month via any route of administration 1, 5

All glucocorticoid-dependent patients are at risk of adrenal crisis during periods of physiological stress, including surgery, acute illness, and trauma 2, 4
For major surgery in adults with adrenal insufficiency: Hydrocortisone 100 mg IV at induction, followed by continuous infusion of 200 mg/24h until oral intake is possible 4, 6
For body surface and intermediate surgery: Same initial dosing, then double the regular glucocorticoid dose for 48 hours postoperatively 6, 7
For minor procedures not requiring general anesthesia: Double the morning dose of hydrocortisone pre-operatively 8
For children with adrenal insufficiency undergoing major surgery: Hydrocortisone 2 mg/kg at induction followed by continuous IV infusion based on weight 8, 9

Women may require higher maintenance doses during later stages of pregnancy (from 20th week onwards) 4
For labor and vaginal delivery: Hydrocortisone 100 mg IV at onset of labor, followed by continuous infusion of 200 mg/24h 6, 7
For cesarean section: Follow major surgery protocol 6, 7
Adrenal crisis is a life-threatening emergency requiring immediate treatment 2
If in doubt about the need for glucocorticoids, they should be given as there are no long-term adverse consequences of short-term administration 4
Hydrocortisone 100 mg IV/IM should be administered immediately, followed by fluid resuscitation 4

After stress dosing, taper back to maintenance dose within 48 hours for uncomplicated cases, or up to a week for major/complicated surgery 4

Dexamethasone is not adequate as glucocorticoid treatment in patients with primary adrenal insufficiency as it lacks mineralocorticoid activity 4
Children with adrenal insufficiency are more vulnerable to problems with glycemic control than adults and require frequent blood glucose monitoring 4