Management of Adrenal Insufficiency
Chronic Maintenance Therapy
For chronic adrenal insufficiency, prescribe hydrocortisone 15-25 mg daily in divided doses (typically 10 mg morning, 5 mg noon, with optional 5 mg evening) to mimic physiologic cortisol secretion 1, 2, 3. This represents the standard of care across all major endocrine societies 2.
Primary vs Secondary Adrenal Insufficiency
Primary adrenal insufficiency (Addison's disease) requires both glucocorticoid AND mineralocorticoid replacement with fludrocortisone 0.05-0.3 mg daily, as the adrenal gland itself is destroyed and cannot produce aldosterone 1, 4, 3.
Secondary adrenal insufficiency (pituitary/hypothalamic dysfunction) requires only glucocorticoid replacement, as aldosterone production remains intact 5, 3.
Glucocorticoid-induced adrenal insufficiency (most common type encountered) results from chronic exogenous steroid use and may recover after cessation, unlike primary insufficiency which requires lifelong treatment 6, 5, 3.
Alternative Glucocorticoid Options
Prednisone 3-5 mg daily can be used as an alternative to hydrocortisone, though hydrocortisone is preferred due to superior physiologic mimicry 2, 3.
Dexamethasone is NOT recommended for routine maintenance therapy; the FDA label explicitly states "hydrocortisone or cortisone is the drug of choice" for chronic adrenal insufficiency 7.
Stress Dose Management During Illness
All patients must double their usual hydrocortisone dose immediately at the onset of any minor-to-moderate illness (fever, infection, gastroenteritis) and continue for 48 hours to one week until symptoms resolve 5, 2. This is non-negotiable—do not wait to see if symptoms improve 2.
Escalation to Emergency Treatment
If the patient cannot take oral medication due to vomiting, has severe weakness, confusion, or hypotension, administer hydrocortisone 100 mg IV/IM immediately 1, 2. This represents adrenal crisis, which carries a mortality risk with 2.19-fold increase for men and 2.86-fold increase for women 5.
Follow the initial 100 mg bolus with continuous infusion of 200 mg/24 hours OR 50 mg IV/IM every 6 hours until the patient stabilizes and can resume oral intake 6, 1.
Provide aggressive fluid resuscitation concurrently, as hypotension and shock are life-threatening features of adrenal crisis 1.
Perioperative Management
Major Surgery in Adults
Administer hydrocortisone 100 mg IV at induction, followed by continuous infusion of 200 mg/24 hours (or 50 mg IV/IM every 6 hours) until oral intake resumes 6, 1. Once the patient can take oral medications, double the usual oral dose for 48 hours, then taper to maintenance over 1-3 days 6, 1.
Major Surgery in Children
Give hydrocortisone 2 mg/kg IV at induction, then 2 mg/kg every 4 hours postoperatively (or weight-based continuous infusion) 6, 1. When enteral intake is established, double the normal dose for 48 hours before returning to standard dosing 6.
No child with adrenal insufficiency should be fasted for more than 6 hours; start hourly blood glucose checks if fasting exceeds 4 hours 6.
Children with both adrenal insufficiency and diabetes insipidus on DDAVP are at high risk for water intoxication without adequate cortisol replacement—strict fluid balance is mandatory 6.
Minor Procedures
For minor surgery or procedures under general anesthesia, give a single dose of hydrocortisone 2 mg/kg (children) or 100 mg (adults) at induction, then double the usual oral dose postoperatively 6, 1.
Labor and Delivery
Initiate hydrocortisone 100 mg IV at onset of active labor (contractions every 5 minutes or cervical dilation >4 cm), followed by 200 mg/24 hours continuous infusion or 50 mg IM every 6 hours, with rapid taper over 1-3 days after uncomplicated delivery 6.
Essential Patient Education and Safety Measures
Every patient with adrenal insufficiency must receive four critical safety interventions 5, 1, 2:
Emergency hydrocortisone injection kit (100 mg for IM/IV use) kept at home with a trained family member who can administer it 5, 2.
Medical alert bracelet or steroid emergency card identifying their diagnosis and need for hydrocortisone 5, 1, 2.
Written sick-day protocol with explicit instructions to double their dose during any illness and when to use the emergency injection 5, 2.
Education on adrenal crisis symptoms: severe weakness, confusion, vomiting, severe abdominal pain, or hypotension requiring immediate emergency injection and 911 call 1, 2.
Monitoring for Over- and Under-Replacement
Under-replacement signs: lethargy, nausea, weight loss, postural hypotension, persistent fatigue 2.
Over-replacement signs: weight gain, insomnia, peripheral edema, cushingoid features (moon facies, central obesity, striae) 2.
The incidence of adrenal crisis remains 6-8 cases per 100 patients per year even under established replacement therapy, emphasizing the importance of patient education 5, 8.
Critical Pitfalls to Avoid
Never confuse maintenance dosing with stress dosing: hydrocortisone 10 mg morning + 5 mg noon is a standard maintenance regimen, NOT a stress dose 2. During stress, this baseline must be doubled 2.
Never delay stress dosing: patients should start doubling their dose immediately when illness begins, not wait to see if symptoms improve 2.
Never abruptly stop doubled doses: taper back to maintenance over 1-2 days once symptoms fully resolve to avoid rebound insufficiency 2.
Never use dexamethasone for chronic maintenance: it lacks the mineralocorticoid activity that hydrocortisone provides at physiologic doses and is explicitly not recommended by FDA labeling for routine replacement 2, 7.
Patients taking prednisolone ≥5 mg daily (adults) or hydrocortisone-equivalent ≥10-15 mg/m² daily (children) for ≥1 month via ANY route (oral, inhaled, topical, intranasal, intra-articular) are at risk for hypothalamic-pituitary-adrenal axis suppression and require perioperative stress dosing 6.