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, 3
• Elevated creatinine and BUN from prerenal renal failure due to volume depletion 1, 2
• Hypoglycemia (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, 3. Similarly, even mild gastrointestinal upset can precipitate crisis because patients cannot absorb oral medications when they need them most 2.

Common Precipitating Factors

• Gastrointestinal illness with vomiting/diarrhea is the most common trigger 2, 4, 5, 6, 7
• Infections of any type (respiratory, urinary, etc.) 1, 2, 4, 6
• Surgical procedures without adequate steroid coverage 2, 4
• Physical trauma or injuries 1, 2, 4
• Failure to increase glucocorticoid doses during intercurrent illness despite patient education 1, 5, 7
• Chronic under-replacement with fludrocortisone combined with low salt consumption 1
• Medications that accelerate cortisol clearance (e.g., rifampin, phenytoin, barbiturates) without dose adjustment 1
• Starting thyroid hormone replacement before adequate glucocorticoid replacement in patients with multiple hormone deficiencies 1
• Poor compliance with maintenance therapy, sometimes related to underlying psychiatric disorders 1

Transition to Maintenance Therapy

• 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
• Transition to oral hydrocortisone 15-25 mg/day divided into 2-3 doses for long-term maintenance 3

Prevention Strategies

Patient Education and Self-Management

Despite extensive patient education, current strategies do not prevent many adrenal crisis events, highlighting the need for improved approaches. 5, 6

• Teach "sick day rules": double or triple oral glucocorticoid dose during minor illness (fever, infection, gastroenteritis) 1, 3, 4, 7
• Instruct on parenteral hydrocortisone use during severe illness, persistent vomiting, or inability to take oral medications 1, 3, 4, 6
• Emphasize seeking medical help early before reaching a state where self-care is impossible 1
• Address barriers to self-injection: many patients and physicians are reluctant to increase doses or switch to parenteral administration, contributing to preventable crises 5, 7

Emergency Preparedness

• Provide emergency injectable hydrocortisone kit for home use 3, 4, 6
• Ensure medical identification: medical alert jewelry and emergency steroid card 3, 4, 6, 8
• Educate family members and caregivers on crisis recognition and emergency injection administration 8

Stress Dosing Guidelines

• Minor illness/stress: double the usual oral dose 3, 4
• Moderate illness with fever: triple the usual oral dose 3, 4
• Severe illness, persistent vomiting, or major surgery: use parenteral hydrocortisone 100 mg IV/IM 3, 4, 6

Follow-Up After Crisis

• Identify and treat the precipitating cause (infection, trauma, medication non-compliance) 1, 4
• Review patient understanding of stress dosing and self-management strategies 1, 4
• Verify emergency supplies are available and not expired 4
• Investigate recurrent crises: consider chronic mineralocorticoid under-replacement, low salt intake, poor compliance, or psychiatric disorders 1

Long-Term Monitoring

Annual Follow-Up Assessment

• Clinical evaluation: appetite, weight stability, blood pressure (check for postural hypotension suggesting mineralocorticoid insufficiency), skin pigmentation 1
• Laboratory monitoring: serum sodium, potassium, thyroid function (TSH, FT4, TPO antibodies), glucose/HbA1c, complete blood count, vitamin B12 1
• Screen for associated autoimmune conditions: hypothyroidism, diabetes mellitus, pernicious anemia, celiac disease (tissue transglutaminase antibodies) 1
• Review quality of life: self-esteem, family relationships, professional duties, and treatment satisfaction 1

Etiologic Workup for New Diagnoses

• Test for 21-hydroxylase autoantibodies (positive in ~85% of autoimmune Addison disease cases in Western Europe) 2
• CT scan of adrenals if autoantibodies are negative, to evaluate for hemorrhage, tumor, tuberculosis, or infiltrative processes 2
• Very long chain fatty acids (VLCFA) if adrenoleukodystrophy is suspected 2

Critical Pitfalls to Avoid

• Never delay treatment for diagnostic procedures or laboratory results when adrenal crisis is suspected 1, 2, 3, 4
• Do not assume absence of hyperkalemia excludes crisis—it is only present in 50% of cases 2, 3
• Recognize that even mild gastrointestinal symptoms can rapidly progress to crisis due to inability to absorb oral medications 2
• Avoid starting thyroid hormone replacement before ensuring adequate glucocorticoid replacement in patients with multiple pituitary hormone deficiencies 1
• Do not use high-dose corticosteroids for traumatic brain injury without other clear indications, as this increases mortality 9
• Be aware of increased infection susceptibility in patients on chronic corticosteroid therapy 9

Mortality and Morbidity Context

Adrenal crisis carries an estimated mortality rate of 0.5 per 100 patient-years despite being a treatable condition for nearly 70 years 6, 7. The incidence of adrenal crises ranges from 5-10 events per 100 patient-years, contributing significantly to the excess mortality experienced by patients with adrenal insufficiency 5, 6, 7. Prompt recognition and immediate treatment with IV hydrocortisone and fluids are highly effective and can prevent unnecessary deaths. 6, 7