Treatment of Adrenal Insufficiency
The treatment of adrenal insufficiency requires glucocorticoid replacement (hydrocortisone 15-25 mg daily in divided doses) and mineralocorticoid replacement (fludrocortisone 50-200 μg daily) for patients with primary adrenal insufficiency, along with patient education on stress dosing to prevent adrenal crisis. 1
Maintenance Therapy
Glucocorticoid Replacement
- Hydrocortisone is the preferred glucocorticoid for replacement therapy, typically administered in a total daily dose of 15-25 mg divided into 2-3 doses 1
- Common dosing schedules include three daily doses: 10 mg in the morning, 5 mg at midday, and 2.5 mg in the afternoon 1
- The first dose should be taken immediately upon waking, and the last dose should be taken at least 6 hours before bedtime to avoid sleep disturbances 1
- Cortisone acetate can be used as an alternative at 18.75-31.25 mg daily in divided doses 1
Mineralocorticoid Replacement
- Fludrocortisone (50-200 μg once daily) is required for patients with primary adrenal insufficiency 1, 2
- Higher doses (up to 500 μg daily) may be needed in children, younger adults, or during the last trimester of pregnancy 1
- Patients should be advised to consume salt and salty foods without restriction 1
Management During Stress and Illness
Adrenal Crisis Management
- Adrenal crisis requires immediate treatment without delay for diagnostic procedures 3
- Treatment includes:
- Hydrocortisone 100 mg IV bolus followed by 100-300 mg/day as continuous infusion or divided IV/IM doses every 6 hours 3, 1
- Rapid IV administration of isotonic saline (0.9%) at an initial rate of 1 L/hour, followed by 3-4 L over 24 hours with frequent hemodynamic monitoring 3
- Treatment of precipitating conditions 3
- Parenteral glucocorticoids should be tapered over 1-3 days to oral maintenance therapy as the patient's condition improves 3
Stress Dosing for Illness
- During minor illnesses with fever, the usual glucocorticoid dose should be doubled or tripled 1
- For vomiting or severe illness, parenteral hydrocortisone is required 4
Perioperative Management
- Major surgery requires 100 mg hydrocortisone IM before anesthesia, followed by 100 mg IM every 6 hours until able to take oral medications 3
- Minor surgery requires 100 mg hydrocortisone IM before anesthesia, then double oral dose for 24 hours 3
- Dental procedures require an extra morning dose 1 hour prior to surgery and double oral dose for 24 hours afterward 3
Prevention of Adrenal Crisis
- All patients should wear medical alert identification jewelry 1
- Patients need comprehensive education on:
- Common precipitating factors for adrenal crisis include:
Follow-up and Monitoring
- Annual follow-up should include:
- Signs of inadequate replacement include:
- Signs of excessive replacement include weight gain, hypertension, and edema 1
Special Considerations
Physical Activity
- Regular, accustomed physical activity generally doesn't require dose adjustment 3
- For intense or prolonged exercise, an increase in hydrocortisone and salt intake may be necessary 3
- For events like marathons, an extra 5 mg of hydrocortisone can be taken before the race 3
Pregnancy
- During delivery, a bolus parenteral dose of 100 mg of hydrocortisone should be given and repeated if necessary every 6 hours 3
- The oral dose should be doubled for 24-48 hours postpartum 3
Common Pitfalls and Caveats
- Under-replacement with mineralocorticoids is common and can predispose patients to recurrent adrenal crises 1
- Medications that can affect glucocorticoid metabolism (requiring dose adjustments) include:
- Despite state-of-the-art steroid replacement therapy, patients with adrenal insufficiency still experience reduced quality of life and increased mortality 4, 5
- Adrenal crisis affects approximately 8 of 100 persons with adrenal insufficiency per year and requires immediate treatment 6
- The sustained administration of excessive amounts of steroid can shorten patients' lives by several years 6