Treatment of ACTH-Dependent Relative Adrenal Insufficiency

Initial Management

The recommended treatment for ACTH-dependent relative adrenal insufficiency is hydrocortisone replacement therapy at physiologic doses, with dose adjustments based on clinical response, starting with 20-30 mg in the morning and 10-20 mg in the afternoon for significant symptoms 1
Administer the first dose immediately after waking, and the last dose not less than 6 hours before bedtime to mimic the natural cortisol rhythm, using the lowest dose compatible with health and a sense of well-being, typically 15-25 mg daily total 2
For moderate symptoms, consider starting at 2-3 times maintenance dose and tapering to maintenance over 5-10 days as symptoms improve 1
For severe symptoms or adrenal crisis, immediate hospitalization with IV hydrocortisone 100 mg bolus followed by 100-300 mg/day as continuous infusion or boluses every 6 hours is required 3

Titrate the hydrocortisone dose based on symptom response, up to a maximum of 30 mg daily for outpatient management 1
Monitor for clinical improvement of symptoms including fatigue, nausea, abdominal pain, and hypotension 3
Assess weight, blood pressure, and serum electrolytes at least annually 2
If essential hypertension develops while on treatment, reduce the dose but do not stop the medication 2

During minor illness, double the usual oral dose for 24-48 hours, then taper back to normal dose 3
For severe illness or surgery, administer 100 mg hydrocortisone IM/IV immediately, followed by 100 mg every 6-8 hours until recovered 3
For dental procedures, take an extra morning dose 1 hour prior to the procedure 4
For major surgery, 100 mg hydrocortisone IM just before anesthesia, continue 100 mg IM every 6 hours until able to eat and drink, then double oral dose for 48+ hours before tapering to normal dose 4

All patients need education on stress dosing for sick days and when to seek medical attention for impending adrenal crisis 5
Provide emergency steroid injectable supplies and training on self-administration 2
Recommend medical alert bracelet for adrenal insufficiency 5, 1
Advise patients to take salt and salty foods ad libitum and avoid licorice and grapefruit juice 2

Always start corticosteroids first when planning hormone replacement therapy for multiple deficiencies, as other hormones can accelerate cortisol clearance and precipitate adrenal crisis 5
ACTH stimulation can give false-negative results early in the course of adrenal insufficiency; in cases of clinical uncertainty, opt for replacement and test for ongoing need at 3 months 5
If prednisone is used instead of hydrocortisone, consider lower doses due to reports of reduced survival on higher doses 5

Schedule follow-up in 2-4 weeks to reassess symptoms and adjust medication 1
Monitor for development of iatrogenic Cushing's syndrome with excessive replacement 1
Consider testing for recovery of the hypothalamic-pituitary-adrenal axis after 3 months of maintenance therapy, especially if the condition was precipitated by exogenous steroid use 5