What is the management approach for an adrenal crisis?

Adrenal Crisis Work-Up and Management

Immediate Emergency Management (Do Not Delay)

Administer hydrocortisone 100 mg IV bolus immediately upon clinical suspicion of adrenal crisis, followed by aggressive fluid resuscitation with 0.9% saline at 1 liter over the first hour—treatment must never be delayed for diagnostic confirmation as mortality increases with delayed intervention. 1

Initial Actions (First 15 Minutes)

• Draw blood samples for cortisol, ACTH, electrolytes (sodium, potassium), creatinine, urea, and glucose before treatment if possible, but never delay hydrocortisone administration waiting for results 1
• Give hydrocortisone 100 mg IV bolus immediately—this high dose saturates 11β-hydroxysteroid dehydrogenase type 2 to provide both glucocorticoid and mineralocorticoid effects, eliminating the need for separate fludrocortisone during acute crisis 1
• Initiate rapid IV fluid resuscitation with 0.9% isotonic saline at 1 L/hour in the first hour to address severe dehydration and hypotension 2, 1

Ongoing Management (First 24-48 Hours)

• Continue hydrocortisone 100-300 mg per day either as continuous IV infusion or as frequent IV/IM boluses every 6 hours 2
• Maintain IV fluid administration with 3-4 liters total of isotonic saline or 5% dextrose in isotonic saline over 24 hours, with frequent hemodynamic monitoring and serum electrolyte measurements to avoid fluid overload 2
• Monitor closely for response to treatment and adjust fluid rate based on blood pressure, urine output, and electrolyte levels 2

Supportive Care Based on Severity

• Consider ICU or high-dependency unit admission for severe cases with persistent hypotension or end-organ dysfunction 2, 1
• Provide gastric stress ulcer prophylaxis and consider low-dose heparin depending on severity of intercurrent illness 2
• Treat precipitating conditions such as infections with appropriate antimicrobial therapy 2

Clinical Recognition and Diagnostic Clues

Key Clinical Presentations to Recognize

• Hypotension (often severe) and shock are the most common life-threatening manifestations 1
• Gastrointestinal symptoms including severe nausea, vomiting, and abdominal pain (which can mimic an acute abdomen) 1
• Dehydration with malaise, fatigue, muscle pain/cramps 1
• Neurological manifestations ranging from confusion and impaired cognitive function to loss of consciousness and coma in severe cases 1
• Orthostatic hypotension occurs before supine hypotension develops and represents an early warning sign—monitor both sitting/standing and supine blood pressure 1

Laboratory Findings to Look For

• Hyponatremia is present in approximately 90% of newly presenting cases 1
• Hyperkalemia is found in only 50% of patients—its absence does not exclude adrenal crisis 1
• Hypoglycemia is common in children but less frequent in adults 1
• Increased creatinine and BUN due to prerenal renal failure from volume depletion 1
• Metabolic acidosis due to impaired renal function and aldosterone deficiency 1
• Serum cortisol <250 nmol/L with markedly elevated plasma ACTH is diagnostic of primary adrenal insufficiency 1

Critical Pitfall to Avoid

• Do not rely on hyperkalemia to confirm diagnosis—it is present in only 50% of cases, while hyponatremia occurs in 90% but its absence should not prevent treatment if clinical suspicion is high 1
• Do not wait for supine hypotension—orthostatic changes occur first and represent a critical early warning sign 1

Identifying Precipitating Causes

Most Common Triggers (In Order of Frequency)

• Gastrointestinal illness with vomiting/diarrhea is the most common trigger—even mild upset stomach may precipitate crisis as patients cannot absorb oral medication when they need it most 1, 3
• Infections of any type are major precipitating causes due to lack of increased cortisol during infection, which enhances pro-inflammatory cytokine release 3
• Surgical procedures without adequate steroid coverage 1
• Physical injuries or trauma 1
• Failure to increase glucocorticoid doses during intercurrent illness despite patient education 1
• Chronic under-replacement with fludrocortisone combined with low salt consumption 1

Etiologic Work-Up After Stabilization

• Test for 21-hydroxylase autoantibodies (21OH-Ab) which are positive in autoimmune Addison disease, accounting for approximately 85% of cases in Western Europe 1
• Obtain CT scan of the adrenals if 21OH-Ab is negative, to evaluate for hemorrhage, tumor, tuberculosis, or other infiltrative processes 1
• Test very long chain fatty acids (VLCFA) if adrenoleukodystrophy is suspected 1
• Investigate poor compliance and underlying psychiatric disorders in patients with recurrent crises 2, 1

Transition to Maintenance Therapy

Tapering Protocol

• Taper parenteral glucocorticoids over 1-3 days to oral therapy once the precipitating illness permits and the patient can tolerate oral medications 2
• Restart fludrocortisone 50-200 µg daily as a single morning dose when the hydrocortisone dose falls to <50 mg/day 2, 4
• Transition to maintenance hydrocortisone 15-25 mg daily divided into 2-3 doses (e.g., 10 mg + 5 mg + 2.5 mg morning, midday, afternoon) 1, 4

Prevention of Future Crises

Patient Education and Emergency Preparedness

• Ensure patient has emergency supplies of injectable hydrocortisone for self-administration during severe illness 1, 3
• Provide medical alert identification jewelry and steroid emergency card to trigger appropriate treatment by emergency personnel 1, 3
• Educate on stress dosing rules: double or triple oral glucocorticoid dose during minor illness with fever; use parenteral hydrocortisone during severe illness or inability to take oral medications 1, 4
• Reinforce that even mild illness (especially gastrointestinal) requires dose adjustment, as patients cannot absorb oral medication when they need it most 1

Follow-Up Monitoring

• Annual screening should include serum sodium, potassium, glucose, HbA1c, complete blood count, and thyroid function (TSH, FT4, TPO-Ab) every 12 months 2, 4
• Screen for vitamin B12 deficiency due to autoimmune gastritis annually 2, 4
• Assess for signs of under-replacement: weight loss, fatigue, postural hypotension, salt craving 4
• Investigate recurrent crises by evaluating for chronic mineralocorticoid under-replacement, low salt consumption, poor compliance, and psychiatric disorders 2, 1