What is the appropriate workup and immediate management for a patient in adrenal crisis?

Adrenal Crisis Workup and Immediate Management

Immediately administer hydrocortisone 100 mg IV bolus and start 0.9% saline 1 L over the first hour without waiting for diagnostic confirmation—treatment delay increases mortality. 1, 2

Immediate Recognition and Clinical Diagnosis

Clinical presentation that should trigger immediate treatment includes:

• Hypotension (often <90/60 mmHg) with or without shock 1, 2
• Orthostatic hypotension (occurs before supine hypotension develops—check both sitting/standing and supine BP) 2
• Severe dehydration with volume depletion 1, 2
• Gastrointestinal symptoms: nausea, vomiting (often severe), abdominal pain (may mimic acute abdomen) 1, 2
• Altered mental status: malaise, confusion, obtundation, or coma 1, 2
• Fever (may be due to adrenal insufficiency itself, not just infection) 2
• Muscle pain or cramps 1, 2

Immediate Laboratory Workup (Do Not Delay Treatment)

Draw blood samples before giving hydrocortisone if possible, but never postpone treatment waiting for results: 1, 2

Essential immediate labs:

• Serum cortisol and ACTH (cortisol <250 nmol/L with elevated ACTH confirms primary adrenal insufficiency) 1, 2
• Electrolytes: sodium, potassium 1, 2
• Creatinine and BUN (assess prerenal azotemia) 1, 2
• Glucose (especially critical in children) 1, 2
• Blood cultures and other infection workup (infections are the most common precipitant) 1, 3

Expected laboratory findings:

• Hyponatremia (present in ~90% of cases) 2
• Hyperkalemia (present in ~50% of cases—absence does not exclude crisis) 2
• Elevated creatinine/BUN from prerenal renal failure 1, 2
• Hypoglycemia (more common in children, less frequent in adults) 2
• Mild hypercalcemia (10-20% of cases) 2
• Metabolic acidosis 2

Emergency Treatment Protocol (First Hour)

Glucocorticoid replacement:

• Hydrocortisone 100 mg IV bolus immediately (this dose saturates 11β-hydroxysteroid dehydrogenase type 2, providing both glucocorticoid and mineralocorticoid effects) 1, 4, 2
• If IV access cannot be rapidly established: give hydrocortisone 100 mg IM as backup 2

Fluid resuscitation:

• 0.9% isotonic saline 1 L IV over the first hour (rapid volume expansion is critical) 1, 4, 2

Ongoing Management (First 24-48 Hours)

Continue hydrocortisone:

• Hydrocortisone 200 mg/24 hours as continuous IV infusion (preferred method) 1, 4, 2
• Alternative: hydrocortisone 50 mg IV or IM every 6 hours 1, 4, 2

Continue fluid resuscitation:

• Total 3-4 L of 0.9% saline or 5% dextrose in saline over 24-48 hours 1, 4, 2
• Monitor hemodynamics frequently to avoid fluid overload 4, 2
• Monitor serum electrolytes frequently to guide fluid management 1, 4, 2

Do NOT add fludrocortisone during acute crisis—high-dose hydrocortisone provides adequate mineralocorticoid activity 4, 2

Supportive Care and Precipitant Management

Critical care considerations:

• Admit to ICU or high-dependency unit for severe cases with persistent hypotension 1, 4, 2
• Gastric stress ulcer prophylaxis 1, 4, 2
• Low-dose heparin for DVT prophylaxis 1, 4, 2
• Treat precipitating infections with appropriate antimicrobial therapy 1, 4, 2
• Frequent blood glucose monitoring (especially in children who are more vulnerable to hypoglycemia) 1, 2

Etiologic Workup (After Stabilization)

Determine underlying cause of adrenal insufficiency:

• 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 (evaluate for hemorrhage, tumor, tuberculosis, infiltrative processes) 2
• Very long chain fatty acids (VLCFA) if adrenoleukodystrophy suspected 2

Transition to Maintenance Therapy

Taper parenteral glucocorticoids over 1-3 days once precipitating illness permits and patient can tolerate oral medications: 1, 4, 2

• Resume oral hydrocortisone at double the usual dose for 48 hours 4, 2, 5
• Then taper to normal maintenance dose (15-25 mg daily in 2-3 divided doses) 4, 2
• Restart fludrocortisone only when hydrocortisone dose falls below 50 mg/day (lower doses no longer provide adequate mineralocorticoid effect) 2

Critical Pitfalls to Avoid

• Never delay treatment for diagnostic confirmation—mortality increases with delayed intervention 1, 2
• Do not assume absence of hyperkalemia excludes crisis—it is present in only 50% of cases 2
• Do not attribute persistent fever solely to infection—it may be due to adrenal insufficiency itself 2
• Do not start thyroid hormone replacement before adequate glucocorticoid replacement in patients with multiple hormone deficiencies—this can trigger crisis 4, 2
• Do not reduce steroid supplementation while patient remains febrile 2

Common Precipitating Factors to Investigate

• Gastrointestinal illness with vomiting/diarrhea (most common trigger—patients cannot absorb oral medications when they need them most) 1, 2, 3
• Infections (any type) 1, 2, 3
• Surgical procedures without adequate steroid coverage 1, 2
• Physical trauma or injuries 1, 2
• Medication non-compliance or treatment failure 1, 2
• Medications accelerating cortisol clearance 2
• Myocardial infarction, severe allergic reactions 1, 2

Prevention of Future Crises

Patient education is paramount to preventing recurrent crises and unnecessary deaths: 4, 2

• Teach patients to double or triple oral glucocorticoid doses during minor illness 4, 2
• Provide emergency injectable hydrocortisone for severe illness or inability to take oral medications 4, 2
• Ensure patients wear medical alert jewelry and carry emergency steroid cards 4, 2
• Evaluate for chronic under-replacement with fludrocortisone, low salt intake, poor compliance, or psychiatric disorders in patients with recurrent crises 2