What is the management of hypotension in patients with adrenal insufficiency?

Management of Hypotension in Adrenal Insufficiency

Immediately administer hydrocortisone 100 mg IV bolus and initiate aggressive fluid resuscitation with 0.9% isotonic saline at 1 liter over the first hour when hypotension occurs in patients with known or suspected adrenal insufficiency. 1

Immediate Emergency Management

First-Line Interventions (Within Minutes)

• Give hydrocortisone 100 mg IV bolus immediately upon clinical suspicion—this dose saturates 11β-hydroxysteroid dehydrogenase type 2 to provide necessary mineralocorticoid effect, eliminating the need for separate fludrocortisone during acute crisis 1

• Start aggressive fluid resuscitation with 0.9% isotonic saline at 1 liter over the first hour, as dehydration and volume depletion are key pathophysiologic features of adrenal crisis 1

• Draw blood for cortisol, ACTH, electrolytes, creatinine, urea, and glucose before treatment begins, but never delay therapy waiting for results—treatment takes absolute priority over diagnostic confirmation 1, 2

Pathophysiology Driving Hypotension

The hypotension in adrenal insufficiency results from multiple mechanisms that must be addressed simultaneously:

• Mineralocorticoid deficiency (in primary adrenal insufficiency) causes sodium loss in urine leading to volume depletion 1
• Glucocorticoid deficiency impairs vasomotor tone and alpha-adrenergic receptor responsiveness 1
• Orthostatic hypotension develops before supine hypotension—this is an early warning sign that should trigger immediate intervention 1

Ongoing Management (First 24-48 Hours)

Continued Glucocorticoid Administration

• Continue hydrocortisone 200 mg/24 hours as continuous IV infusion (or alternatively hydrocortisone 50 mg IV/IM every 6 hours) 3, 1
• This high-dose regimen provides adequate mineralocorticoid activity, so do not add separate fludrocortisone during acute crisis 1

Fluid Management

• Maintain slower isotonic saline infusion for the following 24-48 hours with frequent hemodynamic monitoring to avoid fluid overload 1
• Administer 3-4 liters total of isotonic saline or 5% dextrose in isotonic saline over 24 hours, adjusting based on hemodynamic response 1
• Monitor serum electrolytes frequently to guide fluid management—expect hyponatremia in approximately 90% of cases and hyperkalemia in 50% 1

Special Considerations for Vasopressor-Resistant Hypotension

• In critically ill patients, hypotension refractory to fluids and requiring vasopressors is the most common presentation of adrenal insufficiency in the ICU 4
• Pediatric patients with vasopressor-resistant hypotension may respond to hydrocortisone alone without requiring high doses of other corticosteroids 1
• For children, administer hydrocortisone 2 mg/kg IV/IM immediately, followed by continuous IV infusion based on weight 3
• Initial normal saline fluid bolus of 10-20 ml/kg (maximum 1,000 ml) should be given to hypotensive children 1

Transition to Maintenance Therapy

Tapering Strategy

• Continue IV hydrocortisone while the patient is nil by mouth, then transition to oral glucocorticoids at double the usual maintenance dose for 48 hours once enteral route is available 3
• Taper parenteral glucocorticoids over 1-3 days to oral therapy once the precipitating illness permits and the patient can tolerate oral medications 1
• For uncomplicated recovery, double the usual oral hydrocortisone replacement dose for 48 hours; for major or complicated surgery, continue doubled oral doses for up to one week before tapering to maintenance 5

Adding Mineralocorticoid Replacement

• For patients with primary adrenal insufficiency, add fludrocortisone once enteral feeding is established 3
• Typical maintenance dose is fludrocortisone 0.05-0.3 mg daily 2

Critical Pitfalls to Avoid

• Never postpone treatment to obtain diagnostic confirmation when adrenal crisis is clinically suspected—mortality increases with delayed intervention 1, 6
• Do not wait for supine hypotension to develop; orthostatic changes occur first and represent a critical early warning sign 1
• Do not use dexamethasone alone in patients with primary adrenal insufficiency, as it lacks mineralocorticoid activity 3, 5
• The absence of hyperkalemia does not exclude adrenal crisis, as it is present in only half of cases 1
• Even mild gastrointestinal illness can precipitate crisis, as patients cannot absorb oral medication when they need it most 1

Supportive Care and Monitoring

• Consider ICU or high-dependency unit admission for severe cases with persistent hypotension or end-organ dysfunction 1
• Provide prophylaxis for gastric stress ulcers and consider low-dose heparin depending on severity of intercurrent illness 1
• Treat any precipitating conditions such as infections with appropriate antimicrobial therapy—infections are the major precipitating cause of adrenal crisis 1, 6
• Monitor both sitting (or standing) and supine blood pressure for early detection of orthostatic hypotension 1

Long-Term Prevention

The incidence of adrenal crisis is 5-10 per 100 patient-years with a mortality rate of 0.5 per 100 patient-years 6:

• Every patient should carry an emergency card and be provided with an emergency kit for parenteral hydrocortisone self-administration 6, 7
• Patients should be instructed to use parenteral hydrocortisone during severe illness or inability to take oral medications 5
• Medical alert jewelry is recommended to trigger stress-dose corticosteroids by emergency medical personnel 5
• Active and repeated patient education on managing adrenal insufficiency, including advice on how to increase medication during intercurrent illness, is required to prevent crisis 7