What is the treatment for an adrenal crisis in a patient with adrenal insufficiency?

Treatment of Adrenal Crisis

Immediately administer hydrocortisone 100 mg IV bolus the moment you suspect adrenal crisis—do not delay treatment for diagnostic confirmation, as this is the single most critical life-saving intervention. 1, 2, 3

Immediate Emergency Management (First Hour)

Glucocorticoid Administration

• Give hydrocortisone 100 mg IV bolus immediately upon clinical suspicion, as this high dose saturates 11β-hydroxysteroid dehydrogenase type 2 to provide the necessary mineralocorticoid effect without requiring separate fludrocortisone. 4, 1, 3
• Do not use dexamethasone as primary treatment for adrenal crisis—it lacks mineralocorticoid activity and is inadequate for primary adrenal insufficiency. 3, 5
• If IV access cannot be rapidly established, give hydrocortisone 100 mg IM as an acceptable alternative. 3

Fluid Resuscitation

• Initiate aggressive fluid resuscitation with 0.9% isotonic saline at 1 liter over the first hour to address severe volume depletion and hypotension. 4, 1, 2
• In pediatric patients, administer initial normal saline fluid bolus of 10-20 mL/kg (maximum 1,000 mL) for hypotension. 1, 3

Diagnostic Blood Draw

• Draw blood for serum cortisol, ACTH, electrolytes (sodium, potassium), creatinine, urea, and glucose before initiating treatment, but never delay therapy waiting for results. 4, 1, 2

Ongoing Management (First 24-48 Hours)

Continued Glucocorticoid Therapy

• Continue hydrocortisone 200 mg per 24 hours as continuous IV infusion, which is the preferred method as it maintains cortisol concentrations in the required range better than intermittent boluses. 1, 3, 6
• Alternative regimen: hydrocortisone 50 mg IV or IM every 6 hours if continuous infusion is not feasible. 4, 1, 2
• The high-dose hydrocortisone (≥50 mg per day) provides adequate mineralocorticoid activity, making separate fludrocortisone administration unnecessary during acute crisis. 3

Continued Fluid Management

• Maintain IV fluid resuscitation with 3-4 liters total of isotonic saline or 5% dextrose in isotonic saline over 24 hours, adjusting based on hemodynamic response. 1, 2, 3
• Monitor serum electrolytes frequently to guide fluid management and avoid complications such as fluid overload. 3

Supportive Care

• Consider ICU or high-dependency unit admission for severe cases with persistent hypotension or end-organ dysfunction. 1, 2
• Provide gastric stress ulcer prophylaxis during acute illness. 1, 2
• Consider low-dose heparin prophylaxis depending on severity of intercurrent illness. 3
• Monitor blood glucose frequently, especially in pediatric patients who are more vulnerable to hypoglycemia. 3

Treatment of Precipitating Causes

• Identify and treat precipitating conditions such as infections with appropriate antimicrobial therapy, as infections are the most common trigger for adrenal crisis. 2, 3, 7

Transition to Maintenance Therapy

• Taper parenteral glucocorticoids over 1-3 days to oral therapy once the precipitating illness permits and the patient can tolerate oral medications. 1, 2
• Resume oral hydrocortisone at double the usual dose for 48 hours after uncomplicated recovery, or up to a week following major surgery. 1
• Restart fludrocortisone when hydrocortisone dose falls below 50 mg per day, as lower doses no longer provide adequate mineralocorticoid effect. 3
• Transition to maintenance hydrocortisone 15-25 mg daily divided into 2-3 doses once fully recovered. 3

Critical Clinical Pitfalls to Avoid

• Never postpone treatment to obtain diagnostic confirmation when adrenal crisis is clinically suspected—mortality increases with delayed intervention. 1, 3
• Do not add separate mineralocorticoid (fludrocortisone) during acute crisis management, as the high-dose hydrocortisone provides adequate mineralocorticoid activity. 3
• Never start thyroid hormone replacement before adequate glucocorticoid replacement in patients with multiple hormone deficiencies, as this can trigger adrenal crisis. 3
• Do not attribute persistent fever solely to infection—it may be due to adrenal insufficiency itself, and steroid supplementation should not be reduced while the patient is febrile. 3
• Even mild gastrointestinal upset can precipitate crisis, as patients cannot absorb oral medication when they need it most. 4, 3

Prevention of Future Crises

• Teach "sick day rules": double or triple oral glucocorticoid doses during minor illness (fever, vomiting, diarrhea, or infection). 1, 2, 3
• Prescribe emergency injectable hydrocortisone 100 mg IM and train patients/families on self-injection technique for impending crisis. 1, 7, 8
• Ensure patients carry a steroid emergency card and wear medical alert jewelry to trigger stress-dose corticosteroids by emergency medical personnel. 2, 3, 8
• Arrange early endocrinology consultation for ongoing management and education on emergency injectables. 3
• Evaluate patients with recurrent crises for chronic under-replacement with fludrocortisone, poor compliance, and underlying psychiatric disorders. 3