Treatment of Low Adrenal Function (Adrenal Insufficiency)
All patients with adrenal insufficiency require lifelong glucocorticoid replacement therapy with hydrocortisone 15-25 mg daily in divided doses, and those with primary adrenal insufficiency additionally need fludrocortisone 50-200 μg daily for mineralocorticoid replacement. 1, 2
Maintenance Glucocorticoid Replacement
Hydrocortisone is the preferred glucocorticoid for replacement therapy in adrenal insufficiency 1, 3:
- Total daily dose: 15-25 mg divided into 2-3 doses 1, 2, 4
- Common dosing schedule: 10 mg upon waking, 5 mg at midday, 2.5 mg in afternoon 1
- First dose must be taken immediately upon waking to mimic natural circadian rhythm 1, 2
- Last dose should be at least 6 hours before bedtime to avoid sleep disturbances 1, 2
- Alternative agents include prednisone 3-5 mg daily or cortisone acetate 25-37.5 mg daily 5, 4
For children: hydrocortisone 6-10 mg per m² body surface area 2
Mineralocorticoid Replacement (Primary Adrenal Insufficiency Only)
Fludrocortisone 50-200 μg once daily in the morning is required for all patients with primary adrenal insufficiency 1, 2, 6, 4:
- Dose adjustments based on blood pressure, serum electrolytes (sodium/potassium), and clinical symptoms 2
- Not needed in secondary adrenal insufficiency, as the renin-angiotensin system remains intact 4
Management During Illness and Stress
Patients must double or triple their hydrocortisone dose during minor illnesses (fever, vomiting, diarrhea, infections) 1:
- For moderate illness: 2-3 times maintenance dose 7
- Continue increased doses until fully recovered 1
- All patients must carry injectable hydrocortisone 100 mg for emergencies 1, 4, 8
Treatment of Adrenal Crisis (Life-Threatening Emergency)
Never delay treatment for diagnostic testing if adrenal crisis is suspected 5, 7, 1:
- Hydrocortisone 100 mg IV bolus immediately 5, 1, 2
- Followed by 100 mg IV/IM every 6 hours (or 100-300 mg/day continuous infusion) 5, 1
- Rapid IV isotonic saline (0.9%) at 1 L/hour initially, then 3-4 L over 24 hours with frequent hemodynamic monitoring 5, 1
- Taper parenteral glucocorticoids over 1-3 days to oral maintenance as patient improves 5, 1
- Restart fludrocortisone when hydrocortisone dose falls below 50 mg/day 5
Common precipitating factors for adrenal crisis: vomiting/diarrhea, infections, surgery, injuries, myocardial infarction, severe allergic reactions, hypoglycemia in diabetics 5, 1
Critical Patient Education and Safety Measures
Every patient must have the following 1, 2, 4, 8:
- Medical alert identification jewelry (bracelet or necklace) 1, 2
- Steroid emergency card carried at all times 2, 8
- Injectable hydrocortisone 100 mg kit with training on self/family administration 1, 4, 8
- Written sick-day rules for dose adjustment during illness 1, 8
- Education on recognizing early signs of adrenal crisis (severe fatigue, nausea, vomiting, confusion, hypotension) 5, 9
Follow-Up and Monitoring
Annual follow-up should include 1, 2:
- Assessment of symptoms, weight, blood pressure (postural hypotension indicates insufficient mineralocorticoid) 5, 1
- Serum sodium, potassium, glucose, HbA1c, complete blood count 1
- Screening for associated autoimmune conditions (thyroid function, diabetes, celiac disease, pernicious anemia) 7, 1
- Bone mineral density every 3-5 years to monitor for glucocorticoid-induced osteoporosis 2
Special Clinical Situations
Surgery/procedures: Endocrine consultation required for stress-dose planning 7
Pregnancy/delivery: Hydrocortisone 100 mg IV bolus at delivery, repeat every 6 hours as needed 1
Exercise: Regular activity typically requires no adjustment, but intense/prolonged exercise may need increased hydrocortisone and salt intake 1
Concurrent hypothyroidism: Start corticosteroids several days before thyroid hormone replacement to prevent precipitating adrenal crisis 7
Common Pitfalls to Avoid
- Never rely on electrolyte abnormalities alone for diagnosis—hyperkalemia is present in only ~50% of primary adrenal insufficiency cases, and 10-20% have normal electrolytes 7
- Morning nausea and poor appetite often indicate glucocorticoid under-replacement—consider taking first dose earlier and returning to sleep 7
- Failing to increase doses during illness is the most common cause of preventable adrenal crisis 2, 8
- Exogenous steroids (prednisolone, inhaled fluticasone) can suppress the HPA axis and confound diagnostic testing 7
- Use dexamethasone 4 mg IV (not hydrocortisone) if you need to treat suspected crisis but still want to perform diagnostic testing later, as dexamethasone doesn't interfere with cortisol assays 7