What is the best treatment approach for a patient experiencing an adrenal crisis, including management of hypotension and electrolyte imbalances?

Adrenal Crisis Management

Immediate Emergency Treatment

Administer hydrocortisone 100 mg IV bolus immediately upon clinical suspicion of adrenal crisis, without waiting for diagnostic confirmation, followed by aggressive fluid resuscitation with 1 liter of 0.9% isotonic saline over the first hour. 1, 2

Critical First Steps (Do Not Delay)

• Give hydrocortisone 100 mg IV bolus as soon as adrenal crisis is suspected—treatment must never be delayed for diagnostic procedures 3, 1, 2
• Draw blood for cortisol, ACTH, electrolytes, creatinine, and glucose before administering hydrocortisone if possible, but do not delay treatment waiting for results 1
• Start 0.9% isotonic saline infusion at 1000 mL over the first hour to address profound volume depletion 1, 2

Why Hydrocortisone is the Only Appropriate Choice

• Hydrocortisone is structurally identical to cortisol and provides both glucocorticoid and mineralocorticoid activity at high doses 2
• The 100 mg IV dose saturates 11β-hydroxysteroid dehydrogenase type 2, providing the necessary mineralocorticoid effect without requiring separate fludrocortisone 1
• Dexamethasone is specifically contraindicated in primary adrenal insufficiency because it has no mineralocorticoid activity 3, 2, 4
• Methylprednisolone may only be used if hydrocortisone is unavailable, but is not preferred due to lack of mineralocorticoid activity 2

Ongoing Management (First 24-48 Hours)

Hydrocortisone Continuation

• Continue hydrocortisone 200 mg per 24 hours as continuous IV infusion (preferred for enhanced safety) 3, 1, 2
• Alternative: hydrocortisone 50 mg IV or IM every 6 hours if continuous infusion is impractical 3, 1, 2
• Maintain this dosing until the patient can tolerate oral medications and the precipitating illness has resolved 1

Fluid Management

• Administer 3-4 liters total of isotonic saline or 5% dextrose in isotonic saline over 24 hours 1, 2
• Monitor hemodynamics frequently to avoid fluid overload 1
• Adjust fluid rate based on blood pressure response, urine output, and clinical status 1

Critical Monitoring Parameters

• Check serum electrolytes frequently (every 4-6 hours initially) to guide fluid management 1
• Monitor blood glucose closely, especially in pediatric patients who are more vulnerable to hypoglycemia 3, 1
• Measure blood pressure in both supine and standing positions to detect orthostatic changes 1
• Monitor for signs of fluid overload, particularly in elderly patients or those with cardiac disease 1

Management of Hypotension

Hemodynamic Support

• Hypotension typically responds to hydrocortisone and fluid resuscitation alone without requiring vasopressors 1
• If hypotension persists despite adequate hydrocortisone and fluids, consider ICU admission for closer monitoring 1
• In pediatric patients with vasopressor-resistant hypotension, stress-dose hydrocortisone may be effective without requiring high doses of other corticosteroids 1

Pediatric-Specific Fluid Resuscitation

• Administer initial normal saline fluid bolus of 10-20 mL/kg (maximum 1000 mL) in children with hypotension 1
• Minimize fasting period and prioritize pediatric patients on routine surgical lists 3

Electrolyte Management

Expected Abnormalities

• Hyponatremia occurs in approximately 90% of newly presenting adrenal crisis cases 1
• Hyperkalemia is present in approximately 50% of patients 1
• Hypoglycemia is common in children but less frequent in adults 1
• Metabolic acidosis may occur due to impaired renal function and aldosterone deficiency 1

Treatment Approach

• Do not add separate mineralocorticoid (fludrocortisone) during acute crisis management—high-dose hydrocortisone provides adequate mineralocorticoid activity 1, 2
• Electrolyte abnormalities typically correct with hydrocortisone and fluid resuscitation 1
• Avoid aggressive correction of hyponatremia to prevent osmotic demyelination syndrome 1
• Monitor potassium closely if patient is on digitalis glycosides due to enhanced arrhythmia risk 5

Transition to Maintenance Therapy

Tapering Protocol

• Taper parenteral hydrocortisone over 1-3 days once the patient is clinically stable and can tolerate oral intake 1, 2
• Resume oral hydrocortisone at double the usual maintenance dose for 48 hours after uncomplicated recovery 1, 2
• For major surgery or complicated illness, maintain stress dosing for up to one week before tapering 3
• Return to standard maintenance dose (15-25 mg hydrocortisone daily in 2-3 divided doses) once fully recovered 2

When to Restart Fludrocortisone

• Restart fludrocortisone when hydrocortisone dose falls below 50 mg per day, as lower doses no longer provide adequate mineralocorticoid effect 1
• Typical fludrocortisone maintenance dose is 0.05-0.2 mg daily 5

Critical Pitfalls to Avoid

Common Errors That Increase Mortality

• Never delay hydrocortisone administration to obtain confirmatory cortisol testing—mortality increases with delayed intervention 1, 2
• Never assume normal or even elevated plasma cortisol levels exclude relative adrenal insufficiency in physiologically stressed patients 1
• Never use dexamethasone alone in patients with primary adrenal insufficiency 3, 2
• Never withhold stress-dose steroids if there is any doubt about HPA axis function 2
• 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 1

Medication-Related Pitfalls

• The absence of hyperkalemia does not exclude adrenal crisis—it is present in only 50% of cases 1
• Hyponatremia is very common (90% of cases), but its absence should not prevent treatment if clinical suspicion is high 1
• Starting thyroid hormone replacement before adequate glucocorticoid replacement in patients with multiple hormone deficiencies can trigger adrenal crisis 1, 2

Special Clinical Situations

Perioperative Management

• Give hydrocortisone 100 mg IV at induction of anesthesia in all patients with adrenal insufficiency 3
• Continue hydrocortisone 200 mg per 24 hours as continuous infusion until patient can take double their usual oral dose 3
• For major or complicated surgery, maintain stress dosing for up to one week 3

Obstetric Patients

• Administer hydrocortisone 100 mg at onset of labor 1
• Follow with either 200 mg per 24 hours IV infusion or 50 mg IM every 6 hours until after delivery 1

Patients Unable to Take Oral Medications

• Continue hydrocortisone 200 mg per 24 hours as continuous IV infusion while the patient remains unable to tolerate oral medications 1
• Resume oral hydrocortisone only when the patient can reliably tolerate oral intake and the precipitating illness has resolved 1

Prevention of Future Crises

Patient Education Essentials

• Instruct patients to double or triple oral glucocorticoid doses during minor illness (fever, infection, gastroenteritis) 1, 6
• Teach patients to use parenteral hydrocortisone during severe illness or inability to take oral medications 1, 2
• Provide emergency injectable hydrocortisone kit for home use 6, 7
• Ensure patients carry medical alert identification indicating steroid dependence 1, 2

High-Risk Situations Requiring Increased Dosing

• Gastrointestinal illness with vomiting/diarrhea is the most common trigger for adrenal crisis 1
• Any infection can precipitate crisis 1, 6
• Surgical procedures require stress-dose coverage 1
• Physical trauma or myocardial infarction can trigger crisis 1

Medications That Increase Crisis Risk

• Drugs that accelerate cortisol clearance (barbiturates, phenytoin, rifampin) require glucocorticoid dose adjustment 5
• Immune checkpoint inhibitors can cause hypophysitis with rapid onset of adrenal insufficiency 1
• Rapid tapering of corticosteroids used for other immune-related adverse events can precipitate crisis 1

Mortality Context

• Adrenal crisis carries significant mortality risk, with overall mortality in patients with adrenal insufficiency elevated with a risk ratio of 2.19 for men and 2.86 for women 2, 8
• Estimated mortality rate from adrenal crisis is 0.5 per 100 patient years 6
• Prompt recognition and immediate treatment with hydrocortisone and fluids is highly effective and life-saving 6, 9