What is the management and treatment of an adrenal crisis?

Adrenal Crisis: Comprehensive Management Guide

Immediate Emergency Management

Adrenal crisis is a life-threatening emergency requiring immediate treatment with IV hydrocortisone 100 mg bolus and aggressive fluid resuscitation—treatment must never be delayed for diagnostic procedures. 1, 2, 3

Initial Resuscitation Protocol

• Administer hydrocortisone 100 mg IV bolus immediately upon clinical suspicion, without waiting for laboratory confirmation 1, 2, 3
• Begin aggressive fluid resuscitation with 0.9% saline 1 L over the first hour, followed by 3-4 L over 24-48 hours with frequent hemodynamic monitoring 1, 3, 4
• Continue hydrocortisone 100-300 mg/day either as continuous IV infusion or divided IV/IM boluses every 6 hours 1, 3, 4
• Draw blood for serum cortisol, ACTH, electrolytes (Na, K), creatinine, urea, glucose, and tests for precipitating causes (infection workup) before treatment, but do not delay therapy waiting for results 1, 2, 4

The rationale for the 100 mg bolus is critical: this high dose saturates 11β-hydroxysteroid dehydrogenase type 2, providing the necessary mineralocorticoid effect in addition to glucocorticoid replacement 1. This is why mineralocorticoid replacement is not needed acutely during crisis management.

Supportive Care Measures

• Consider ICU or high-dependency unit admission depending on severity of presentation 1, 4
• Initiate gastric stress ulcer prophylaxis 1, 4
• Provide low-dose heparin for DVT prophylaxis 1, 4
• Start empiric antibiotic treatment if infection is suspected as the precipitating cause 1, 4
• Monitor hemodynamics and electrolytes frequently to avoid fluid overload 1, 4

Clinical Recognition

Cardinal Presenting Features

• Hypotension and shock are the most critical manifestations requiring immediate intervention 2, 3, 4
• Severe dehydration with associated prerenal renal failure 1, 2, 4
• Gastrointestinal symptoms: severe nausea, vomiting, abdominal pain (sometimes mimicking acute abdomen with peritoneal irritation) 1, 2, 4
• Constitutional symptoms: profound malaise, fatigue, muscle pain or cramps 1, 2, 4
• Neurological manifestations: impaired cognitive function, confusion, loss of consciousness, or coma in severe cases 1, 2, 4

Key Laboratory Abnormalities

• Hyponatremia (present in ~90% of cases) 2
• Hyperkalemia (present in ~50% of cases—absence does not exclude crisis) 2
• Elevated creatinine and BUN from prerenal renal failure due to volume depletion 1, 2, 4
• Hypoglycemia (more common in children, less frequent in adults) 1, 2
• Mild hypercalcemia (10-20% of cases) 2
• Metabolic acidosis from impaired renal function and aldosterone deficiency 2

Critical pitfall: The absence of hyperkalemia should not prevent treatment if clinical suspicion is high, as it is only present in half of cases 2. Similarly, even mild gastrointestinal upset can precipitate crisis because patients cannot absorb oral medications when they need them most 2.

Common Precipitating Factors

Understanding triggers is essential for both acute management and prevention:

• Gastrointestinal illness with vomiting/diarrhea is the most common precipitant 2, 4, 5
• Infections of any type (respiratory, urinary, systemic) 1, 2, 4, 6
• Surgical procedures without adequate steroid coverage 2, 4
• Physical trauma or injuries 1, 2, 4
• Myocardial infarction 2, 4
• Severe allergic reactions 2, 4
• Failure to increase glucocorticoid doses during intercurrent illness despite patient education 1
• Chronic under-replacement with fludrocortisone combined with low salt consumption 1
• Medications that accelerate cortisol clearance (e.g., rifampin, phenytoin, phenobarbital) without dose adjustment 1
• Starting thyroid hormone replacement before adequate glucocorticoid replacement in patients with multiple hormone deficiencies 1

Transition to Maintenance Therapy

Tapering Protocol

• Taper parenteral glucocorticoids over 1-3 days to oral maintenance therapy once the patient is stabilized and the precipitating illness permits 1, 3, 4
• Restart fludrocortisone when hydrocortisone dose falls below 50 mg/day 1
• Continue slower-rate isotonic saline infusion for 24-48 hours after initial resuscitation 1

Long-term Maintenance

• Hydrocortisone 15-25 mg/day divided into 2-3 doses is the preferred glucocorticoid for maintenance 3
• Fludrocortisone for mineralocorticoid replacement in primary adrenal insufficiency 1

Prevention Strategies

Patient education alone does not prevent many adrenal crisis events, highlighting the need for comprehensive preventive measures. 7, 6

Essential Patient Education Components

• Teach patients to double or triple their glucocorticoid dose during minor illness (fever, infection, significant stress) 3, 4
• Instruct on parenteral hydrocortisone self-administration during severe illness when oral intake is not possible 3, 4, 6
• Emphasize seeking medical help promptly before reaching a state where self-care is impossible 1
• Reinforce that even mild gastrointestinal upset requires dose adjustment because oral absorption is compromised 2

Emergency Preparedness

• Every patient must carry medical alert identification (jewelry, emergency card) 3, 4
• Provide emergency supplies including injectable hydrocortisone for home use 3, 4, 6
• Ensure patients and family members are trained in emergency injection technique 6, 8
• Consider hydrocortisone pen devices to lower barriers to self-injection 6

Stress Dosing Guidelines for Specific Situations

• Minor illness/stress: Double the usual daily dose 3, 4
• Moderate illness with vomiting: Use parenteral hydrocortisone 100 mg IM 3, 4
• Major surgery: Hydrocortisone 100 mg IV at induction, then 200 mg/24h continuous infusion or divided doses 6

Follow-up After Crisis

Immediate Post-Crisis Evaluation

• Identify and treat the precipitating cause (infection, trauma, medication non-compliance) 1, 4
• Review patient's understanding of stress dosing and self-management to prevent recurrence 1, 4
• Investigate potential contributing factors: poor compliance, psychiatric disorders, inadequate mineralocorticoid replacement, low salt intake 1

Long-term Monitoring

• Annual follow-up with assessment of health status, well-being, weight, blood pressure, and electrolytes 1, 4
• Monitor for associated autoimmune conditions: thyroid function (TSH, FT4, TPO-Ab) every 12 months 1
• Screen annually for diabetes (glucose, HbA1c), anemia (CBC), B12 deficiency 1
• Evaluate for celiac disease in patients with frequent diarrhea (tissue transglutaminase antibodies, total IgA) 1

Critical Pitfalls to Avoid

• Never delay treatment for diagnostic confirmation—clinical suspicion alone warrants immediate therapy 1, 2, 3
• Do not assume absence of hyperkalemia excludes crisis—it's only present in 50% of cases 2
• Avoid starting thyroid replacement before glucocorticoid replacement in patients with multiple deficiencies 1
• Do not underestimate mild gastrointestinal symptoms—they can rapidly progress to full crisis 2
• Recognize that patient education alone is insufficient—emergency supplies and action plans are essential 7, 6
• Remember that high-dose corticosteroids should NOT be used for traumatic brain injury due to increased mortality 9

Diagnostic Workup for Underlying Etiology

Once the patient is stabilized, determine the cause of adrenal insufficiency:

• Test for 21-hydroxylase autoantibodies (21OH-Ab), positive in ~85% of autoimmune Addison disease cases in Western Europe 2
• CT scan of adrenals if 21OH-Ab negative to evaluate for hemorrhage, tumor, tuberculosis, or infiltrative processes 2
• Very long chain fatty acids (VLCFA) if adrenoleukodystrophy is suspected 2
• Markedly elevated plasma ACTH with low cortisol confirms primary adrenal insufficiency 2